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Title: A System of Practical Medicine By American Authors, Vol. II - General Diseases (Continued) and Diseases of the Digestive System
Author: Various
Language: English
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A SYSTEM OF PRACTICAL MEDICINE BY AMERICAN AUTHORS.


EDITED BY WILLIAM PEPPER, M.D., LL.D., PROVOST AND PROFESSOR OF THE
THEORY AND PRACTICE OF MEDICINE AND OF CLINICAL MEDICINE IN THE
UNIVERSITY OF PENNSYLVANIA.

ASSISTED BY LOUIS STARR, M.D., CLINICAL PROFESSOR OF DISEASES OF
CHILDREN IN THE HOSPITAL OF THE UNIVERSITY OF PENNSYLVANIA.


VOLUME II. GENERAL DISEASES (CONTINUED) AND DISEASES OF THE DIGESTIVE
SYSTEM.



PHILADELPHIA: LEA BROTHERS & CO.

1885.



Entered according to Act of Congress, in the year 1885, by

LEA BROTHERS & CO.,

in the Office of the Librarian of Congress at Washington. All rights
reserved.

WESTCOTT & THOMSON, _Stereotypers and Electrotypers, Philada._

WILLIAM J. DORNAN, _Printer, Philada._



CONTENTS OF VOLUME II.


GENERAL DISEASES (CONTINUED).
                                                                  PAGE
RHEUMATISM. By R. PALMER HOWARD, M.D.  . . . . . . . . . . . . .    19

GOUT. By W. H. DRAPER, M.D.  . . . . . . . . . . . . . . . . . .   108

RACHITIS. By ABRAHAM JACOBI, M.D.  . . . . . . . . . . . . . . .   137

SCURVY. By PHILIP S. WALES, M.D. . . . . . . . . . . . . . . . .   167

PURPURA. By I. EDMONDSON ATKINSON, M.D.  . . . . . . . . . . . .   186

DIABETES MELLITUS. By JAMES TYSON, A.M., M.D.  . . . . . . . . .   195

SCROFULA. By JOHN S. LYNCH, M.D. . . . . . . . . . . . . . . . .   231

HEREDITARY SYPHILIS. By J. WILLIAM WHITE, M.D. . . . . . . . . .   254


DISEASES OF THE DIGESTIVE SYSTEM.

DISEASES OF THE MOUTH AND TONGUE. By J. SOLIS COHEN, M.D.  . . .   321

DISEASES OF THE TONSILS. By J. SOLIS COHEN, M.D. . . . . . . . .   379

DISEASES OF THE PHARYNX. By J. SOLIS COHEN, M.D. . . . . . . . .   390

DISEASES OF THE OESOPHAGUS. By J. SOLIS COHEN, M.D.  . . . . . .   409

FUNCTIONAL AND INFLAMMATORY DISEASES OF THE STOMACH. By SAMUEL
  G. ARMOR, M.D., LL.D.  . . . . . . . . . . . . . . . . . . . .   436

SIMPLE ULCER OF THE STOMACH. By W. H. WELCH, M.D.  . . . . . . .   480

CANCER OF THE STOMACH. By W. H. WELCH, M.D.  . . . . . . . . . .   530

HEMORRHAGE FROM THE STOMACH. By W. H. WELCH, M.D.  . . . . . . .   580

DILATATION OF THE STOMACH. By W. H. WELCH, M.D.  . . . . . . . .   586

MINOR ORGANIC AFFECTIONS OF THE STOMACH (Cirrhosis; Hypertrophic
  Stenosis of Pylorus; Atrophy; Anomalies in the Form and the
  Position of the Stomach; Rupture; Gastromalacia). By W. H.
  WELCH, M.D.  . . . . . . . . . . . . . . . . . . . . . . . . .   611

INTESTINAL INDIGESTION. By W. W. JOHNSTON, M.D.  . . . . . . . .   620

CONSTIPATION. By W. W. JOHNSTON, M.D.  . . . . . . . . . . . . .   638

ENTERALGIA (INTESTINAL COLIC). By W. W. JOHNSTON, M.D. . . . . .   658

ACUTE INTESTINAL CATARRH (DUODENITIS, JEJUNITIS, ILEITIS,
  COLITIS, PROCTITIS). By W. W. JOHNSTON, M.D. . . . . . . . . .   667

CHRONIC INTESTINAL CATARRH. By W. W. JOHNSTON, M.D.  . . . . . .   699

CHOLERA MORBUS. By W. W. JOHNSTON, M.D.  . . . . . . . . . . . .   719

INTESTINAL AFFECTIONS OF CHILDREN IN HOT WEATHER. By J. LEWIS
  SMITH, M.D.  . . . . . . . . . . . . . . . . . . . . . . . . .   726

PSEUDO-MEMBRANOUS ENTERITIS. By PHILIP S. WALES, M.D.  . . . . .   763

DYSENTERY. By JAMES T. WHITTAKER, A.M., M.D. . . . . . . . . . .   777

TYPHLITIS, PERITYPHLITIS, AND PARATYPHLITIS. By JAMES T.
  WHITTAKER, A.M., M.D.  . . . . . . . . . . . . . . . . . . . .   814

INTESTINAL ULCER. By JAMES T. WHITTAKER, A.M., M.D.  . . . . . .   823

HEMORRHAGE OF THE BOWELS. By JAMES T. WHITTAKER, A.M., M.D.  . .   830

INTESTINAL OBSTRUCTION. By HUNTER MCGUIRE, M.D.  . . . . . . . .   835

CANCER AND LARDACEOUS DEGENERATION OF THE INTESTINES. By I.
  EDMONSON ATKINSON, M.D.  . . . . . . . . . . . . . . . . . . .   868

DISEASES OF THE RECTUM AND ANUS. By THOMAS G. MORTON, M.D., and
  HENRY M. WETHERILL, JR., M.D., PH.G. . . . . . . . . . . . . .   877

INTESTINAL WORMS. By JOSEPH LEIDY, M.D., LL.D. . . . . . . . . .   930

DISEASES OF THE LIVER. By ROBERTS BARTHOLOW, A.M., M.D., LL.D. .   965

DISEASES OF THE PANCREAS. By LOUIS STARR, M.D. . . . . . . . . .  1112

PERITONITIS. By ALONZO CLARK, M.D., LL.D.  . . . . . . . . . . .  1132

DISEASES OF THE ABDOMINAL GLANDS (TABES MESENTERICA). By SAMUEL
  C. BUSEY, M.D. . . . . . . . . . . . . . . . . . . . . . . . .  1182


INDEX. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .  1195



CONTRIBUTORS TO VOLUME II.


ARMOR, SAMUEL G., M.D., LL.D.,
  Brooklyn.

ATKINSON, I. EDMONDSON, M.D.,
  Professor of Pathology and Clinical Medicine and Clinical Professor
  of Dermatology in the University of Maryland, Baltimore.

BARTHOLOW, ROBERTS, A.M., M.D., LL.D.,
  Professor of Materia Medica, General Therapeutics, and Hygiene in
  the Jefferson Medical College, Philadelphia.

BUSEY, SAMUEL C., M.D.,
  An Attending Physician and Chairman of the Board of Hospital
  Administration of the Children's Hospital, Washington, D.C.

CLARK, ALONZO, M.D., LL.D.,
  Late Professor of Pathology and Practical Medicine in the College of
  Physicians and Surgeons, New York.

COHEN, J. SOLIS, M.D.,
  Professor in Diseases of the Throat and Chest in the Philadelphia
  Polyclinic; Physician to the German Hospital, Philadelphia.

DRAPER, W. H., M.D.,
  Attending Physician to the New York and Roosevelt Hospitals, New
  York.

HOWARD, R. PALMER, M.D.,
  Professor of Theory and Practice of Medicine in McGill University,
  Montreal; Consulting Physician to Montreal General Hospital, Canada.

JACOBI, ABRAHAM, M.D.,
  Clinical Professor of Diseases of Children in the College of
  Physicians and Surgeons, New York, etc.

JOHNSTON, W. W., M.D.,
  Professor of Theory and Practice of Medicine in the Columbian
  University, Washington.

LEIDY, JOSEPH, M.D., LL.D.,
  Professor of Anatomy in the University of Pennsylvania,
  Philadelphia.

LYNCH, JOHN S., M.D.,
  Professor of Principles and Practice of Medicine in the College of
  Physicians and Surgeons, Baltimore.

MORTON, THOMAS G., M.D.,
  Surgeon to the Pennsylvania Hospital, Philadelphia.

MCGUIRE, HUNTER, M.D.,
  Richmond, Va.

SMITH, J. LEWIS, M.D.,
  Clinical Professor of Diseases of Children in the Bellevue Hospital
  Medical College, New York.

STARR, LOUIS, M.D.,
  Clinical Professor of Diseases of Children in the Hospital of the
  University of Pennsylvania, Philadelphia.

TYSON, JAMES, A.M., M.D.,
  Professor of General Pathology and Morbid Anatomy in the University
  of Pennsylvania, Philadelphia.

WALES, PHILIP S., M.D.,
  Washington.

WELCH, WILLIAM H., M.D.,
  Professor of Pathology in Johns Hopkins University, Baltimore.

WETHERILL, HENRY M., JR., M.D.,
  Assistant Physician to the Pennsylvania Hospital for the Insane,
  Philadelphia.

WHITE, J. WILLIAM, M.D.,
  Surgeon to the Philadelphia Hospital; Assistant Surgeon to the
  University Hospital; Demonstrator of Surgery and Lecturer on
  Venereal Diseases and Operative Surgery in the University of
  Pennsylvania, Philadelphia.

WHITTAKER, JAMES T., M.D.,
  Professor of Theory and Practice of Medicine in the Medical College
  of Ohio, Cincinnati.



ILLUSTRATIONS TO VOLUME II.

FIGURE                                                            PAGE
 1. POSITION OF PUNCTURES IN DIABETIC AREA OF MEDULLA OBLONGATA
    NECESSARY TO PRODUCE GLYCOSURIA  . . . . . . . . . . . . . .   195

 2. THE LAST CERVICAL AND FIRST THORACIC GANGLIA, WITH CIRCLE OF
    VIEUSSENS, IN THE RABBIT, LEFT SIDE  . . . . . . . . . . . .   196

 3. DIAGRAM SHOWING COURSE OF THE VASO-MOTOR NERVES OF THE
    LIVER, ACCORDING TO CYON AND ALADOFF . . . . . . . . . . . .   197

 4. DIAGRAM SHOWING ANOTHER COURSE WHICH THE VASO-MOTOR NERVES
    OF THE LIVER MAY TAKE  . . . . . . . . . . . . . . . . . . .   197

 5. JOHNSON'S PICRO-SACCHARIMETER  . . . . . . . . . . . . . . .   214

 6. PEMPHIGUS BULLA FROM A NEW-BORN SYPHILITIC CHILD . . . . . .   276

 7. SECTION OF RETE MUCOSUM AND PAPILLÆ FROM SAME CASE OF
    PEMPHIGUS AS FIG. 6  . . . . . . . . . . . . . . . . . . . .   276

 8. SECTION OF AN OLD GUMMA OF THE LIVER . . . . . . . . . . . .   284

 9. SYPHILITIC DACTYLITIS, FROM BUMSTEAD . . . . . . . . . . . .   292

10. THE SAME AS FIG. 9 . . . . . . . . . . . . . . . . . . . . .   292

11. SERRATIONS OF NORMAL INCISOR TEETH . . . . . . . . . . . . .   297

12. NOTCHING OF SYPHILITIC INCISOR TEETH . . . . . . . . . . . .   297

13. OÏDIUM ALBICANS FROM THE MOUTH IN A CASE OF THRUSH . . . . .   331

14. CHRONIC INTUMESCENCE OF THE TONGUE (HARRIS)  . . . . . . . .   351

15. HYPERTROPHY OF TONGUE (HARRIS), BEFORE OPERATION AND AFTER .   352

16. GLOSSITIS (LISTON) . . . . . . . . . . . . . . . . . . . . .   361

17. INCISION FOR A CUSPID TOOTH (WHITE)  . . . . . . . . . . . .   378

18. INCISION FOR A MOLAR TOOTH (WHITE) . . . . . . . . . . . . .   378

19. FUSIFORM DILATATION OF OESOPHAGUS (LUSCHKA)  . . . . . . . .   433

20. and 21. FAUCHER'S TUBE FOR WASHING OUT THE STOMACH . . . . .   605

22. ROSENTHAL'S METHOD OF WASHING OUT THE STOMACH  . . . . . . .   606

23. ANTERIOR VIEW OF A STRANGLUATED INTESTINE AND STRICTURE  . .   842

24. POSTERIOR VIEW OF A STRANGULATED INTESTINE AND STRICTURE . .   842

25. APPEARANCE OF THE NATURAL RELATIONS OF THE DIVERTICULUM TO
    THE INTESTINE  . . . . . . . . . . . . . . . . . . . . . . .   843

26. SIMPLE INVAGINATION OF THE ILEUM . . . . . . . . . . . . . .   844

27. SIMPLE INVAGINATION, WITH OCCLUSION OF BOWEL, FROM
    INFLAMMATORY CHANGES . . . . . . . . . . . . . . . . . . . .   844



{17}

GENERAL DISEASES (_CONTINUED_).

FROM DERANGEMENTS OF THE NORMAL PROCESSES OF NUTRITION.


RHEUMATISM.    | PURPURA.
               |
GOUT.          | DIABETES MELLITUS.
               |
RACHITIS.      | SCROFULA.
               |
SCURVY.        | HEREDITARY SYPHILIS.



{19}

RHEUMATISM.

BY R. P. HOWARD, M.D.


Acute Articular Rheumatism.

SYNONYMS AND DEFINITION.--Acute Rheumatism, Acute Rheumatic
Polyarthritis, Rheumarthritis, Rheumatic Fever, Polyarthritis
Synovialis Acuta (Heuter).

Acute articular rheumatism is a general non-contagious, febrile
affection, attended with multiple inflammations, pre-eminently of the
large joints and very frequently of the heart, but also of many other
organs; these inflammations observing no order in their invasion,
succession, or localization, but when affecting the articulations
tending to be temporary, erratic, and non-suppurating; when involving
the internal organs proving more abiding, and often producing
suppuration in serous membranes. It is probably connected with a
diathesis--the arthritic--which may be inherited or acquired. It may
present such modifications of its ordinary characters as to justify
being called (2d) subacute articular rheumatism, and it may sometimes
pass into the (3d) chronic form.

ETIOLOGY.--There is a general consensus of opinion that acute articular
rheumatism belongs especially to temperate climates, and that it is
exceedingly rare in polar regions; but respecting its prevalence in the
tropics contradictory statements are made. Saint-Vel declares that it
is not a disease of hot climates; Rufz de Levison saw only four cases
of acute articular rheumatism, and not one of chorea, in Martinique
during twenty years' practice; while Pruner Bey says it is common in
Egypt, and Webb remarks the same for the East Indies. Even in temperate
climates, like those of the Isle of Wight, Guernsey, Cornwall, some
parts of Belgium (Hirsch), the disease is very rare--a circumstance not
to be satisfactorily explained at present.

Acute articular rheumatism is never absent; it occurs at all seasons of
the year, although subject to moderate variations depending mainly upon
atmospheric conditions. It is the general opinion that it prevails most
during the cold and variable months of spring, but this is not true of
every place, nor invariably of the same place. Indeed, Besnier,[1]
after a long and special observation of the disease in Paris, concludes
that there it is most frequent in summer and in spring. In Montreal,
during ten years, the largest number of cases of acute rheumatism
admitted to the General Hospital obtained in the spring months (March
to June {20} inclusive), when they averaged 51 a month; 33 was the
average for all the other months, except October and November, when
26-1/2 was the average. The statistics of Copenhagen, Berlin, and
Zurich show a minimum prevalence in summer or in summer and autumn.

[Footnote 1: _Dictionnaire Encyclopédique des Sciences Méd._, Troisième
Serie, t. iv.]

Occupations involving muscular fatigue or exposure to sudden and
extreme changes of temperature, especially during active bodily
exertion, predispose to acute articular rheumatism; hence its frequency
amongst cooks, maid-servants, washerwomen, smiths, coachmen, bakers,
soldiers, sailors, and laborers generally.

While no age is exempt from acute articular rheumatism, it is, par
excellence, an affection of early adult life, the largest number of
cases occurring between fifteen and twenty-five years of age, and the
next probably between twenty-five and thirty-five. A marked decline in
its frequency takes place after the age of thirty-five, and a still
greater after forty-five. It is not uncommon in children between five
and ten, and especially between ten and fifteen, but is very rare under
five, although now and then one meets with an example of the disease in
children three or four years of age. While the acute articular
affections observed in sucklings are, as a general rule, either
syphilitic or pyæmic, some authentic instances of rheumatic
polyarthritis are recorded. Kauchfuss's two cases among 15,000 infants
at the breast, Widerhofer's case, only twenty-three days old, Stager's,
four weeks old, and others, are cited by Senator.[2]

[Footnote 2: _Ziemssen's Cyclop. of Pract. Med._, xvi. 17.]

An analysis of 4908 cases of acute rheumatism admitted to St.
Bartholomew's Hospital, London,[3] during fifteen years, and of 456
treated in the Montreal General Hospital during ten years,[4] gives the
following percentages at given periods of life:

             London.                        Montreal.
  Under      10 years,  1.79 %  |  Under      15 years,  4.38 %
  From 10 to 15   "  ,  8.1  %  |  From 15 to 25   "  , 48.68 %
   "   15 to 25   "  , 41.8  %  |   "   25 to 35   "  , 25.87 %
   "   25 to 35   "  , 24.5  %  |   "   35 to 45   "  , 13.6  %
   "   35 to 45   "  , 14.2  %  |  Above      45   "  ,  7.4  %
  Above      45   "  ,  9.5  %  |

The close correspondence existing in the two tables for all the periods
of life above fifteen is very striking: the disparity between them
below the age of fifteen may, I believe, be explained by the
circumstance that the pauper population of Montreal is, when compared
with that of London, relatively very small, and by the further fact
that the practice of sending children into hospitals hardly obtains
here.

[Footnote 3: _St. Bartholomew's Hospital Reports_, xiv. 4.]

[Footnote 4: Dr. James Bell, in _Montreal General Hospital Reports_, i.
350.]

No doubt the above tables do not correctly represent the liability of
children to acute articular rheumatism, but they are probably a fair
statement of the relative frequency of the disease in the adult
hospital populations of London and Montreal. If primary attacks of the
disease only were tabulated, the influence of youth would be more
evident, for it is scarcely possible to find on record an authentic
instance of the disease showing itself for the first time after sixty.
Dr. Pye-Smith[5] has done {21} this in 365 cases, and the results prove
the great proclivity of very young persons to acute rheumatism: Between
five and ten years, 6 per cent. occurred; between eleven and twenty, 49
per cent.; from twenty-one to thirty, 32.3 per cent.; from thirty-one
to forty, 9.5 per cent.; from forty-one to fifty, 2.2 per cent.; and
from fifty-one to sixty-one, 1.1 per cent. The same author has also
shown that secondary attacks are most common in the young; so that
advancing age not only renders a first attack of the disease
improbable, but lessens the risk of a recurrence of it. The influence
of age upon acute rheumatism is further shown in the fact that the
disease is less severe, and less apt to invade the heart, in elderly
than in young persons.

[Footnote 5: _Guy's Hospital Reports_, 3d Series, xix. 317.]

The general opinion that sex exercises no direct influence beyond
exposing males more than females to some of the predisposing and
exciting causes of acute rheumatism is perhaps true if the statement be
confined to adults, to whom, indeed, most of the available statistics
apply; but it should be borne in mind that a larger proportion of men
than of women resort to hospitals, and there is some reason to believe
that in childhood the greater liability to the disease is on the part
of the female sex. Thus, the number of cases of rheumatism treated at
the Children's Hospital in London from 1852 to 1868 was 478, of whom
226 were males and 252 females.[6] Of Goodhardt's 44 cases of acute
rheumatism in children, 26 were girls and 18 were boys.[7] Of 57
examples of rheumatism in connection with chorea observed by Roger in
children under fourteen, 33 were girls and 24 were boys.[8]

[Footnote 6: Vide Dr. Tuckwell's "Contributions to the Pathology of
Chorea," in _St. Bartholomew's Hospital Reports_, v. 102.]

[Footnote 7: _Guy's Hospital Reports_, 3d Series, xxv. 106.]

[Footnote 8: _Arch. Gén._, vol. ii. 641, 1866, and vol. i. 54, 1867,
quoted by Tuckwell.]

That heredity predisposes to acute articular rheumatism is admitted by
nearly all modern authorities, even Senator, while speaking of it as "a
traditional belief," not venturing to deny it. The frequency of the
inherited predisposition Fuller placed at 34 per cent.; Beneke, quoted
by Homolle,[9] at 34.6 per cent; Pye-Smith at 23 per cent.[10] Such
predisposition favors the occurrence of the disease in early life, but
does not necessarily determine an attack of acute rheumatism in the
absence of the other predisposing or exciting causes. That the
inherited bias or mode of vital action or condition of tissue-health
may be so great as, per se, to induce an attack of the disease, is held
by some authorities. It is probable that not only acute articular
rheumatism in the parents, but simple chronic articular rheumatism and
those forms grouped under the epithet rheumatoid arthritis, may impart
a predisposition to the acute as well as to the chronic varieties of
articular disease just mentioned. But owing to the obscurity which
still surrounds the relations existing between acute articular
rheumatism and rheumatoid arthritis this point needs further
investigation. In what the inherited predisposition to acute articular
rheumatism consists we are ignorant; to say that it imparts to the
tissues or organs a disposition to react or act according to a fixed
morbid type, or that some of the nutritive processes are perverted by
it, is merely to state a theory, not to explain the nature of the
predisposition.

[Footnote 9: _Nouv. Dict. de Méd. et de Chir._, t. 31, 557.]

[Footnote 10: _Guy's Hospital Reports_, 3d Series, xix. 320.]

No type of bodily conformation or temperament can be described that
{22} certainly indicates a proclivity to acute articular rheumatism;
nor is there any change in the constitution of the tissues or fluids of
the body by which the proclivity may be recognized. We infer the
existence of the inherited predisposition--the innate bias--when
rheumatism is found in the family history; when acute rheumatism or
cardiac disease, or chorea not produced by mental causes, occurs in
childhood; when the first attack of acute articular rheumatism is
succeeded by subsequent attacks; and especially when the intervals
between the attacks are short. Goodhardt has recently furnished
valuable, but not conclusive, evidence to prove that in children
obstinate headaches, night-terrors, severe anæmia, various
neuro-muscular derangements, such as torticollis, tetany, muscular
tremors, stammering, incontinence of urine, recurring attacks of
abdominal pain, with looseness of the bowels quickly succeeding a meal,
the cutaneous affection erythema nodosum, are indications of a
rheumatic bias or predisposition.[11]

[Footnote 11: _Guy's Hospital Reports_, 3d Series, xxv.]

There is some basis for the opinion that residence in damp, cold
dwellings predisposes somewhat to acute articular rheumatism, although
not at all to the same degree that it does to the chronic articular and
muscular forms. Chomel and Jaccoud especially have insisted that it
will gradually create a predisposition to the disease, even if it has
not been inherited. All pathologists agree that cold is the most
frequent exciting cause of acute articular rheumatism, and that it is
especially effective when applied while the body is perspiring freely
or is overheated or fatigued by exercise. There is no necessary ratio
between the degree of cold or its duration and the severity of the
resulting rheumatism. A slight chilling or a momentary exposure to a
current of cold air will in some act as powerfully and as certainly as
a prolonged immersion in cold water or a night spent sleeping on the
damp grass. This circumstance, together with the fact that cold applied
in the same way may also produce a pharyngitis or a bronchitis, a
pneumonia or a nephritis, etc., is held to indicate that the cold acts
according to individual predisposition; and Jaccoud, Flint, and others
maintain that unless a rheumatic proclivity exists cold will not
produce an attack of the disease under consideration. I doubt that we
are yet in a position to assert that absolutely, although the weight of
argument is in its favor. Let it suffice to say, that while a prolonged
residence in a cold, damp dwelling may gradually develop a
predisposition to rheumatism, a short exposure to cold will be likely
to induce an attack of rheumatism if the predisposition exist.

There are other influences which may be regarded as auxiliaries to cold
in exciting an attack, as they seem to increase the susceptibility of
the patient to its operation: they establish what has been felicitously
called a state of morbid opportunity. Such are all influences that
reduce the resisting powers of the organs and organism, as bodily
fatigue, mental exhaustion, the depressing passions, excessive venery,
prolonged lactation, losses of blood, etc. It is probably in such a
manner that local injuries (traumatism) sometimes appear to induce an
attack of rheumatism. A blow on a finger (Cotain), the extraction of a
tooth (Homolle), a hypodermic injection (ibid.), etc., may act
powerfully in some persons upon and through the nervous system, and by
lessening their resisting power {23} may favor the overt manifestation
of the rheumatic predisposition. But doubtless some such cases have
been examples of mere coincidence.

There are certain pathological and even physiological conditions during
or after which an inflammatory affection of one or several joints
closely resembling acute articular rheumatism more or less frequently
arises. Thus, during the early desquamating stage of scarlatina a mild
inflammation of the joints of the hands and feet, and frequently of the
large articulations as well, is very often seen, and it is attended
with profuse perspiration, with a condition of urine like that of
ordinary acute rheumatism, and occasionally with inflammation of the
heart or pleura. During convalescence from dysentery an affection of a
single or of several articulations resembling rheumatism has been
noticed, and the two affections have even alternated in the same
patient. That singular epidemic disease dengue is attended with a
polyarticular affection closely resembling acute articular rheumatism,
occasionally pursuing a protracted course, and not seldom leaving after
it a cardiac lesion. In hæmophilia polyarticular and muscular disorders
frequently arise which closely resemble, and appear to be sometimes
identical with, ordinary acute articular and muscular rheumatism.
Gonorrhoea too is often associated with a febrile polyarthritis, and
rarely with an endocarditis at the same time. In the puerperal state an
inflammation of one or several articulations is not unfrequently
observed (puerperal rheumatism).

Respecting the real nature of these polyarticular inflammations very
much has to be made out; and it must suffice at present to say that
while many of them are of a pyæmic nature, as some examples of
puerperal and scarlatinal arthritis, in which pus forms in or about the
joints and in the serous cavities and viscera, some of them are no
doubt examples of genuine rheumatism occurring in persons of rheumatic
predisposition, which have either been induced by the lowering
influence of the disease upon which they have supervened, or by the
accidental coincidence of some of the other causes of acute rheumatism.
There remains, however, the ordinary form of scarlatinal arthritis,
which so closely resembles true acute articular rheumatism in its
symptoms, course, visceral complications, and morbid anatomy that it
cannot be said that the two affections are distinct and different. And
much the same appears to be true of the articular affection of dengue.
Yet so frequently does the articular affection accompany scarlatina and
dengue respectively that it cannot logically be referred to a
coexisting rheumatic predisposition, and must be a consequence of the
disturbing influences of the specific poison of those zymotic
affections per se.

PATHOLOGY.--The pathology of acute articular rheumatism is a very much
debated question, and is not at all satisfactorily known. Hence a mere
statement of the most prominent theories now held by different
pathologists will be given.[12]

[Footnote 12: The reader may consult with advantage Dr. Morris
Longstreth's fourth chapter in his recent excellent monograph upon
_Rheumatism, Gout, and some Allied Disorders_, New York, 1882.]

The latest modification of the lactic-acid theory of Prout is founded
upon the modern physiological teaching that during muscular exercise
sarcolactic acid and acid phosphate of potassium are formed, and carbon
dioxide set free, in the muscular tissue, and that cold, acting on {24}
the surface under such circumstances, may check the elimination of
these substances and cause their accumulation in the system. This view,
it is held, explains why the muscles and their associated organs, the
joints and tendons, suffer first and chiefly, because the morbific
influence is exerted upon them when exhausted by functional activity;
and it further accounts for the visceral manifestations and the
apparent excess of acid eliminated during the course of the disease.
The circumstance that in three cases of diabetes (Foster,[13]
Kuelz[14]) the administration of lactic acid appeared to induce
polyarticular rheumatism favors the idea that acid is the materies
morbi in rheumatism.

[Footnote 13: _Brit. Med, Jour._, ii. 1871.]

[Footnote 14: _Beiträge zur Path. und Therapie des Diabetes_, u. s. w.,
ii. 1875.]

Now it must be admitted that, as yet, no sufficient proof is
forthcoming that a considerable excess of lactic acid exists in the
fluids or solids of the body or in the excretions in rheumatism (it is
true the point has not been sufficiently investigated). On the other
hand, that acid has been found in the urine of rickets, and its excess
in the system is regarded by Heitzmann and Senator[15] as the cause of
the peculiar osteoplastic disturbances of that disease--an affection
altogether different from rheumatism. It is quite improbable that the
amount of sarcolactic acid produced by over-prolonged muscular
exertion, and whose elimination has been prevented by a chill or a
mental emotion, is sufficient to maintain the excessive acidity of the
urine and other fluids during a long rheumatic fever; and arguments can
be adduced favorable to the view that excessive formation of acid is an
effect rather than the cause of rheumatism: cases of that disease occur
in which neither excessive muscular exertion nor exposure to chill have
preceded the rheumatic outbreak. Lastly, lactic acid is not the only
principle retained when the functions of the skin are arrested by cold,
the usual exciting cause of rheumatism; why should not the retained
acetic, formic, butyric, and other acids, for example, play their rôle
in the production of the symptoms observed under such conditions?

[Footnote 15: _Ziemssen's Cyclop._, xvi. p. 177.]

The same objections apply to Latham's[16] hypothesis that
hyperoxidation of the muscular tissue is the starting-point of acute
rheumatism. He assumes, with other physiologists, the existence of a
nervous centre which inhibits the chemical changes that would take
place if the tissues were out of the body. If this centre be changed or
weakened, the muscle, instead of absorbing and fixing the oxygen and
giving out carbonic acid, disintegrates; lactic acid is formed, and,
passing into the blood, may be there oxidized and produce the pyrexia
of acute rheumatism. It need hardly be remarked that the existence of a
chemical inhibitory centre has yet to be proved, although much may be
advanced in its favor; and, secondly, the recent investigations of
Zuntz render it highly probable that in all febrile affections it is
the muscles chiefly, if not solely, which suffer increased oxidation,
and that this is due to increased innervation--views not easily
reconciled with Latham's theory.

[Footnote 16: _Brit. Med. Jour._, ii. 1880, p. 977.]

The nervous theory of rheumatism and of articular diseases originated
with Dr. J. K. Mitchell of Philadelphia[17] in 1831, and was afterward
elaborated by Froriep in 1843,[18] Scott Alison[19] in 1846, Constatt
in 1847,[20] {25} Gull in 1858, Weir Mitchell in 1864,[21] Charcot in
1872, and by very many others since. According to present physiological
doctrine, the exciting cause of rheumatism, cold, either acts directly
upon the vaso-motor or the trophic (?) nerves of the articulations, and
excites inflammation of them, or else it irritates the peripheral ends
of the centripetal nerves, and through these excites actively the
vaso-motor and trophic nerve-centres. The local lesions, on this
hypothesis, are of trophic origin; the fever is due to hyperactivity of
the centres supposed to control the chemical changes going on in the
tissues; the excessive perspiration to stimulation of the
sweat-centres; and so on. It is not held that a definite centric lesion
of the nervous system exists in rheumatism, analogous to the lesions
which in myelitis or locomotor ataxia develop the arthropathies of
those affections, but rather a functional disturbance. One of the
latest and ablest advocates of the neurosal theory of rheumatism in all
its forms (simple, rheumatoid, gonorrhoeal, urethral, etc.), Jonathan
Hutchinson, calls it "a catarrhal neurosis, the exposure of some tract
of skin or mucous membrane to cold or irritation acting as the incident
excitor influence."[22]

[Footnote 17: _Am. Jour. Med. Sci._, 1831; _ib._, 1833.]

[Footnote 18: _Die Rheumatische Schwiele_, Weimar, 1843.]

[Footnote 19: _Lancet_, 1846, i. 227.]

[Footnote 20: _Spec. Pathologie und Therapie_, 1847, ii. p. 609.]

[Footnote 21: Vide _Am. Jour. Med. Sciences_, April, 1875, vol. lxix.
339-348.]

[Footnote 22: _Trans. International Med. Congress_, 1881, ii. 93.]

In order that peripheral irritation shall thus induce inflammation of
the joints and the other affections of muscles, tendons, fasciæ, etc.
which are called rheumatic, he holds with the French School that the
arthritic diathesis must exist, or that state of tissue-health which
involves a tendency to temporary inflammation of many joints or fibrous
structures at once, or to repeatedly recurrent attacks of inflammation
of one joint or fibrous structure. If I understand Mr. Hutchinson
correctly, he also holds that a nerve-tissue peculiarity exists which
renders persons liable to rheumatism. He does not indicate either the
cause or the nature of the nerve-tissue peculiarity. But modern
pathology teaches that the functional conditions of the nervous centres
known as neuroses, whether inherited or acquired, reveal themselves as
morbid manifestations of nerve-function on the part of special portions
of or the entire nervous system, and, as Dr. Dyce Duckworth has well
pointed out, these neuroses may be originated, when not inherited, in
various ways, as by excessive activity of the nervous system, by
prolonged or habitual excesses, etc. "Thus, undue mental labor,
gluttony, alcoholic intemperance, debauchery, and other indulged evil
propensities in the parent come to be developed into definite neurotic
taint and tendency in the offspring."

But is there nothing more in acute articular rheumatism than an
inflammation of certain structures, articular and visceral, lighted up
in an individual of a neuro-arthritic diathesis? What do we learn from
that closely-allied affection, gout, which involves especially the same
organs as rheumatism, and is held by many of the ablest pathologists to
belong to the same basic diathesis as it? Duckworth[23] has very ably
advocated a neurotic theory of gout, but it is admitted on all
hands--and by Duckworth himself--that in gout a large part of the
phenomena is due to perverted relations of uric acid and sodium and to
the presence of urate of soda in the blood. May we not from analogy, as
well as from other evidence, infer that in that so-called other
neurosis, rheumatism, a considerable part of the phenomena is due to
perversions of {26} the processes of assimilation and excretion, and to
the presence of some unknown intermediate product of destructive
metamorphosis--lactic or other acid? This is admitted by Maclagan and
strongly advocated by Senator; and in this way the pathology of the
disease may be said to embrace the humoral as well as the solidist
doctrines--the resulting theory being a neuro-humoral one. No doubt
pathological chemistry and clinical investigation will ere long make
important discoveries respecting the pathology of acute rheumatism
which shall maintain the close alliance believed to exist between that
affection and gout.

[Footnote 23: _Brain_, April, 1880.]

The miasmatic theory, so ably advocated by Maclagan,[24] assumes that
rheumatism is due to the entrance into the system from without of a
miasm closely allied to, but quite distinct from, malaria. His argument
on this topic is ingenious and elaborate, yet has not been favorably
received by pathologists. Opposed to it are the following amongst other
considerations: Heredity exercises a marked influence upon the
occurrence of rheumatism; unlike malarial disease, no climate or
locality is immune from rheumatism; the many indications that a
diathesis plays a chief rôle in rheumatism; the remarkable influence
exerted by cold and dampness in the etiology of the disease.

[Footnote 24: _Rheumatism: its Nature, Path., etc._, London, 1881, pp.
60-95.]

Heuter's[25] infective-germ theory, like the miasmatic, refers
rheumatism to a principle not generated in the system, but introduced
from without. A micrococcus enters the dilated orifices of the
sweat-glands, and, reaching the blood, first sets up an endocarditis,
and then capillary emboli produce the articular inflammations. This is
a reversal of what really happens, so far as the time of invasion of
the endocardium and the synovial membranes is concerned; and
Fleischauer's case, in which miliary abscesses were found in the heart,
lungs, and kidneys, was probably one of ulcerative endocarditis, which,
after all, is a rare complication of acute articular rheumatism.
Moreover, it is a gratuitous assertion to say that endocarditis exists
in all cases of the disease. If, however, Heuter were content to say
that acute articular rheumatism was produced by a specific germ, as
held by Recklinghausen and Klebs, which on entering the system acted
specially upon the joints and the fibro-serous tissues, as the poison
of small-pox does upon the skin, while at the same time it sets up
general disturbances of the entire economy as other zymotic poisons do,
there would be nothing opposed to general pathological laws. Even the
existence of a diathesis capable of favoring the action of the specific
germ would be analogous to the tuberculous diathesis, which favors the
action of the bacillus of tubercle; and cold, its ordinary exciting
cause, might be regarded simply as a condition which renders the system
more susceptible to the action of the germ, and the modus operandi of
cold in doing this might be variously explained.

[Footnote 25: _Klinik der Gelenkkrankheiten_, Leipzig, 1871.]

SYMPTOMS.--The disease has no uniform mode of invasion. (_a_) Very
frequently slight disorder of health, such as debility, pallor, failure
of appetite, unusual sensibility to atmospheric changes, grumbling
pains in the joints or limbs, or even in some muscle or fascia,
precedes by one or more days the fever and general disturbance. (_b_)
Not infrequently a mild rigor or repeated chilliness, accompanied or
soon followed by moderate or high fever, ushers in the illness, and in
from a few hours to one {27} or at most two days the characteristic
articular symptoms ensue. (_c_) In very rare cases febrile disturbance,
ushered in by chills, may be followed by inflammation of the endo- or
pericardium or pleura before the joints become affected.

Whatever the mode of invasion, the symptoms of the established disease
are well defined, and marked febrile disturbance, transient
inflammation of several of the larger articulations, excessive activity
of the cutaneous functions, and a great proclivity to inflammation of
the endo- and pericardium constitute the stereotyped features of the
disease.

As a very general rule, the temperature early in the disease promptly
attains its maximum of 102° F. to 104° F., yet the surface does not
feel very hot; the pulse ranges from 90 to 100 or 110, and is regular,
large, and often bounding; the tongue is moist, but thickly coated with
a white fur; there are marked thirst, impaired appetite, and
constipation; the stools are usually dark; the urine scanty, high
colored, very acid, of great density, and holding in solution an excess
of uric acid and urates, which are frequently deposited when the urine
cools. The general surface is covered with a profuse sour-smelling
perspiration, whose natural acid reaction, as a general rule, is
markedly increased; indeed, the naturally alkaline saliva is also acid.
Beyond a little wandering during sleep, occasionally observed in
irritable, nervous patients, there is very rarely any delirium, and
this notwithstanding that sleep is frequently much disturbed by the
pain in the joints and the excessive sweating.

If the local articular symptoms have not set in almost simultaneously
with the pyrexia, or even preceded it, they will follow it in from a
few to twenty-four or forty-eight hours. At first one or more joints,
usually the knees or ankles, become painful, sensitive to pressure,
hot, more or less swollen, and exhibiting a slight blush of redness or
none at all. The swelling may consist of a mere puffiness, due to
slight infiltration of the soft parts external to the joint, or of a
more or less considerable tumefaction, caused by effusion into the
synovial capsule. In the knees, elbows, shoulders, and hips the
swelling is usually confined to the articulations, and there is but
little redness of the integument, but in the wrists and ankles the
inflammatory process is often more severe, and may invade the whole
dorsum of the hand or foot, rendering the integument tense, tumid red,
and shining. Pitting of the swollen parts, although quite exceptional
in acute articular rheumatism, will exist under the conditions just
mentioned. The metacarpo-phalangeal articulations are likewise often a
good deal swollen and of a bright-red color.

The pain in the affected articulations varies from a trifling
uneasiness or dull ache to excruciating anguish; sometimes the pain is
felt only on moving or pressing the joint; pressure always aggravates
it; even the weight of the bed-clothes may be intolerable; and in
severe cases the slightest movement of the joint or a jar of the bed
produces great suffering. The pain, like the swelling, sometimes
extends beyond the affected joints to the tendinous sheaths, the
tendons, and muscles, and even to the nerves of the neighborhood.

It is a striking peculiarity of acute rheumatism that the inflammation
tends to invade fresh joints from day to day, the inflammation usually,
but not invariably, declining in those first affected; and sometimes
this retrocession of the inflammation in a joint is so sudden, and so
coincident {28} with the invasion of a different one, that it is often
regarded as a true metastasis. Exceptionally, however, one or several
joints remain painful and swollen, although this occurs chiefly in
subacute attacks. In this way most of the large joints may successively
suffer once, twice, or oftener during an attack of acute rheumatism.
And as the inflammation commonly lasts in each articulation from two to
four or more days, it is usual to have six or eight of the joints
affected by the end of the first week. While the ankles and knees,
wrists, elbows, and shoulders, are especially liable to be affected,
and with a frequency pretty closely corresponding to the above order,
the joints of the hands occasionally, and the hips even more
frequently, escape. The intervertebral and tempero-maxillary
articulations have very rarely suffered in the writer's experience.

If the ear be applied to the cardiac region in acute rheumarthritis,
another local inflammation than the articular will very frequently be
detected, which otherwise would probably be unrecognized, and yet it is
the most important feature of the disease. In the first or second, or
even as late as the fourth, week of the fever the signs of endocarditis
of the mitral valve, occasionally of the aortic, and sometimes of both,
will exist in an uncertain but large proportion of cases, or those of
pericarditis, but in a less proportion, will obtain. Indeed, the
cardiac inflammation may even precede the articular, and some believe
it may be the only local evidence of rheumatic fever. As a general
rule, the implication of the endo- or pericardium in acute
rheumarthritis gives rise to no marked symptoms or abrupt modification
of the clinical features of the case, and a careful physical
examination must be instituted to discover its existence. But the
recurrence of pain or tightness either in the precordial or sternal
region, of marked anxiety or pallor of the face, of sudden increase in
the weakness or frequency of the pulse, or of irregularity in its
rhythm, of restlessness or delirium, of oppression of breathing, or of
short, dry cough,--may indicate the invasion of the endo- or peri- or
myocardium, and a physical examination will be needed to detect the
cardiac disease and to exclude the presence of pleuritis, pneumonia, or
bronchitis. Sometimes, however, especially in severe cases, an
extensive pericarditis, with or without myocarditis, will produce grave
constitutional disturbance, in which sleeplessness, delirium, stupor,
generally associated with a very high temperature and marked
prostration, will, as it were, mask both the articular and the cardiac
affection.[26]

[Footnote 26: See Stanley's case, _Med.-Chir. Trans._, 1816, vol. vii.
323, and Andral's _Clinique Médicale_, t. i. 34.]

As regards the murmurs which arise in acute rheumatic endo- or
pericarditis, while they are usually present and quite typical, this is
not always so. The only alteration of the cardiac sounds may be at
first and for some time a loss of clearness and sharpness, passing into
a prolongation of the sound, which usually develops into a distinct
murmur, or the sounds may be simply muffled. In pericarditis limited to
that portion of the membrane which covers the great vessels no friction
murmur may be audible, or it may be heard and be with difficulty
distinguished from an endocardial murmur. On the other hand, a systolic
basic murmur not due to endo- or pericarditis frequently exists,
sometimes in the early, but usually in the later, stages of rheumatic
fever.

{29} Other local inflammations occasionally arise in the course of
acute rheumatism: pneumonia is one of the most frequent; left pleuritis
is not infrequent, and is doubtless often caused by the extension of a
pericarditis; but both pneumonia and pleurisy are occasionally double
in rheumatic fever. Severe bronchitis is observed now and then, and
very rarely peritonitis, and even meningitis. These several affections,
together with delirium, coma, convulsions, chorea, and hyperpyrexia,
which are likewise occasional incidents of the disease, will be
considered under the head of non-articular manifestations and
complications of acute articular rheumatism.[27]

[Footnote 27: See observations of W. S. Cheesman, M.D., _New York
Medical Record_, Feb. 25, 1882, 202.]

Some of the symptoms of acute articular rheumatism need individual
notice.

The temperature in acute articular rheumatism maintains no typical
course, and usually exhibits a series of exacerbations and remissions,
which correspond closely in time and degree with the period, duration,
and severity of the local inflammatory attacks. As a very general rule
in average cases, the temperature attains by the end of the first or
second day to 102° F., and while the subsequent evening exacerbations
may reach 104°, 104.4°, or very rarely 105°, yet in the great majority
of cases the maximum temperature does not exceed 103° F., and in a very
considerable number falls short of 102°. An analysis of one of Dr.
Southey's tables[28] shows that in 84 cases of acute rheumatism 1
attained the temperature of 105.8°; 8, that of 104° to 105°; 15, that
of 103° to 104°; 32, that of 102° to 103°; 17, that of 101° to 102°;
10, that of 100° to 101°; and 1, that of 99.8°; that is, the
temperature was below 103° in five-sevenths, and below 104° in about
ten-twelfths, of the whole. In very mild cases, in which but a few
joints are inflamed, and only to a slight degree, the temperature may
not reach 100° at any time, and there may be intervals of complete
apyrexia. On the other hand, in a few rare severe cases of rheumatic
fever, especially when complicated with pericarditis, pneumonia, or
delirium, or other disturbance of the cerebral functions, the
temperature attains to 106°, 108°,[29] 109.4°,[30] 110.2°,[31] or even
111°,[32] or 112°. Such cases are now spoken of as examples of
rheumatic hyperpyrexia.

[Footnote 28: _St. Bartholomew's Hospital Reports_, xiv. p. 12.]

[Footnote 29: Weber, _Clinical Society's Trans._, vol. v. p. 136.]

[Footnote 30: Th. Simon, quoted by Senator, _Ziemssen's Cyclop. of
Prac. Med._, xvi. p. 46.]

[Footnote 31: Murchison and Burdon-Sanderson, two cases, _Clinical
Society's Trans._, vol. i. pp. 32-34.]

[Footnote 32: Ringer, _Med. Times and Gaz._, vol. ii., 1867, p. 378.]

There is no rule about the mode of invasion of this high temperature.
It may ensue gradually or suddenly, the previous range having been low,
moderate, or high, steady or oscillating.

Defervescence in rheumatic fever takes place, as a very general rule,
gradually--_i.e._ by lysis--but exceptionally it is completed in
forty-eight or even twenty-four hours. An interesting observation,
which will be of much prognostic value if it be confirmed hereafter,
has been made by Reginald Southey,[33] to the effect "that a short
period of defervescence, or a sudden remission and an early remission,
betokens the relapsing form of the disease, and the likelihood of
frequent relapses, as well as of slow ultimate recovery, in the direct
ratio as this defervescence has been early and abrupt."

[Footnote 33: _St. Bartholomew's Hospital Reports_, xiv. p. 16.]

{30} The characters of the urine in acute rheumatism are tolerably
uniform, but far from constantly so. Its quantity in the majority of
cases is reduced, frequently not exceeding twenty-four ounces per diem,
and occasionally not exceeding fourteen. This is owing in some degree
to profuse sweating, but also, as in other febrile affections, to
retention of water. Its density is usually high--1020 to 1030, or even
1035--which is due chiefly to its concentration, and not, as has been
generally supposed, mainly to an increase in the total solids
excreted.[34] Its color is a very dark red or deep reddish-yellow,
partly from concentration; but it is yet not known whether the deep hue
is partly from increase of the normal pigments or of one of them
(urobilin),[35] or from the presence of some abnormal coloring matter.
Its reaction is generally highly acid, and continues so for many hours
after its discharge, unless in subacute cases, when it is occasionally
neutral or sometimes alkaline at the time of its escape, or becomes so
in a very short time afterward. It is commonly toward the decline of
the attack that the urine becomes neutral or alkaline. As a very
general rule, the amount of urea and of uric acid excreted during the
febrile stage exceeds what is physiological, and begins to decline when
convalescence commences; but this may be reversed (Parkes,[36]
Lede,[37] Marrot[38]). The sulphuric acid is notably increased
(Parkes), the chlorides often diminished and sometimes absent, and the
phosphoric acid very variable (Beneke, Brattler[39]), but usually
lessened (Marrot).

[Footnote 34: See _Guy's Hospital Reports_, 3d Series, vol. xii. 441.]

[Footnote 35: Jaffe, _Virchow's Archiv_, xlvii. 405, quoted in
_Ziemssen's Cyclopæd. Prac. Med._, xvi. 41.]

[Footnote 36: _On Urine_, p. 286.]

[Footnote 37: _Recherches sur l'Urine dans le Rheumatisme Artic.
Aigue_, Paris, 1879.]

[Footnote 38: _Contribution à l'Étude du Rheumatisme Artic., etc._,
Paris, 1879, 41.]

[Footnote 39: Quoted by Parkes, _op. cit._, 290.]

During convalescence the urine increases in quantity, while, as a
general rule, the urea and uric acid lessen relatively and absolutely,
and the chlorides resume their normal proportions to the other
ingredients. The reaction frequently becomes alkaline, and the specific
gravity falls considerably, although not always as soon as the
articular inflammation subsides. Temporary albuminuria occurs very
frequently in the febrile and occasionally in the declining stage, but
generally disappears when convalescence is completed. It obtained on
admission in 8 out of 43 cases lately reported by Dr. Greenhow.[40] A
more abiding albuminuria, due very rarely to acute parenchymatous
nephritis, may be met with (Johnson, Bartels, Hartmann, Corm). Blood,
even in considerable amounts, has also rarely appeared in the
urine,[41] sometimes in connection with embolic nephritis and
endocarditis, for such appear to have been the nature of Rayer's
nephrite rheumatismale.[42]

[Footnote 40: _Lancet_, 1882, i. 913.]

[Footnote 41: _Clinical Lectures_, R. B. Todd, edited by Beale, 1861,
p. 346.]

[Footnote 42: _Traité des Maladies Reins_. See also Dr. Weber, _Path.
Trans. of London_, xvi. p. 166.]

The saliva, which is normally alkaline, has usually a decidedly acid
reaction in acute articular rheumatism, and Dr. Bedford Fenwick states
that it always in this disease contains a great excess of the
sulpho-cyanides, and that these slowly and steadily diminish, till at
the end of the third week or so they become normal in amount.

A profuse, very acid, sour-smelling perspiration is one of the striking
symptoms occurring in the course of acute articular rheumatism, and
{31} until very lately it has been generally held to indicate an
excessive formation in, and elimination of acid from, the system,
either lactic acid or some of the acids normal to the perspiration, as
acetic, butyric, and formic. However, not only have chemists failed to
detect lactic acid in the perspiration of acute rheumatism, but late
research tends to show that the excessive acidity of the perspiration
in this disease is but very partially due to the perspiration itself,
and is chiefly owing to chemical changes taking place in the overheated
and macerated surface of the skin and its epidermis, and to the
retention of solid products accumulated on that surface. Besnier says
that if in acute articular rheumatism or other disease attended with
much perspiration the surface be kept well washed, the sweat will be
found in the greater number of cases at the moment of its secretion to
be nearly neutral as soon as actual diaphoresis occurs, more decidedly
acid when the perspiration is less abundant or begins to flow, and
exceptionally alkaline. Most physicians are aware that the profuse
perspiration of acute rheumatism is non-alleviating; it is not a real
critical discharge of noxious materials from the system, nor is it
followed by prompt reduction of the temperature and other symptoms. It
is but a symptom of the disease, and occurs especially in severe cases,
and when it continues long after the reduction of the temperature it is
a source of exhaustion, and may be checked with advantage.

The blood is deficient in red globules, Malassez finding in men from
2,850,000 to 3,700,000 per cubic millimeter instead of 4,500,000 to
5,000,000, and in women 2,300,000 to 2,570,000 instead of 3,500,000 to
4,000,000. The hæmoglobin and the oxidizing power of the blood are also
considerably reduced; the fibrin is largely increased (6 to 10 parts in
1000 instead of 3); the albumen and albuminates are lessened, the
extractives increased; the proportion of urea is normal, and no excess
of uric acid is found in the blood. Instead of that fluid being less
alkaline than normal, Lepine and Conard have recently stated that its
alkalinity is increased in acute rheumatism, but constantly diminished
in chronic rheumatism,[43] and no excess of lactic acid has been proved
to exist in the blood in either acute or chronic rheumatism. A
condition of excessive coagulability of the fibrin, independently of
its excessive amount (inopexia), is an habitual character of acute
rheumatism; however, in very bad cases, especially those attended with
hyperpyrexia and grave cerebral symptoms, the blood after death has
been black and coagulated and the fluid in the serous cavities has
given an acid reaction. The above alterations in the blood usually are
proportionate to the intensity of the fever and the number of the
joints and viscera involved.

[Footnote 43: Lepine, "Note sur la determination de l'Alcalinité du
Sang," _Gaz. Méd. de Paris_, 1878, 149; Conard, _Essai sur l'Alcalinité
du Sang dans l'État de Sante, etc._, Thèse, Paris, 1878.]

The manifestations of acute articular rheumatism other than the
articular are various, and some of them, more especially those observed
in the heart, may be regarded as integral elements of the disease, for
they occur in a large proportion of the cases, often coincidentally
with the articular affection, and may even precede it, and probably may
be the sole local manifestation of acute rheumatism, although under the
last-mentioned circumstances it is difficult to prove the rheumatic
nature of the ailment.

The cardiac affections may be divided into inflammatory and {32}
non-inflammatory. The former consist of pericarditis, endocarditis, and
myocarditis; the latter embrace deposition of fibrin on the valves,
temporary incompetence of the mitral or tricuspid valves, and the
formation of thrombi in the cavities of the heart. For practical
purposes hæmic murmurs may be included in the latter group.

No reliable conclusions can be drawn respecting the gross frequency of
recent cardiac affections in rheumatic fever, for not only do authors
differ widely on this point, but they do not all distinguish recent
from old disease, nor inflammatory from non-inflammatory affections,
nor hæmic from organic murmurs. Nor does it appear probable, from the
published statistics, that these differences are owing to peculiarities
of country or race. The gross proportion of heart disease of recent
origin in acute and subacute articular rheumatism was in Fuller's[44]
cases 34.3 per cent.; in Peacock's,[45] 32.7 per cent.; in Sibson's[46]
(omitting his threatened or probable cases), 52.3 per cent.;[47] in
3552 St. Bartholomew's Hospital cases analyzed by Southey,[48] 29.8 per
cent.; in Bouilland's cases, quoted by Fuller,[49] 5.7 per cent.; in
Lebert's,[50] 23.6 per cent.; in Vogel's,[50] 50 per cent.; in
Wunderlich's,[50] 26.3 per cent. I am not aware of any analysis,
published in this country, of a large number of cases of rheumatism
with reference to cardiac complications, but Dr. Austin Flint,[51]
after quoting Sibson's percentage of cases of pericarditis, which was
(63 in 326 or) 19 to the 100, remarks, "I am sure that this proportion
is considerably higher than in my experience."

[Footnote 44: _On Rheumatism, Rheumatic Gout, etc._, 3d ed., p. 280.]

[Footnote 45: _St. Thomas's Hospital Reports_, vol. x. p. 19.]

[Footnote 46: Reynolds's _Syst. of Med._, Eng. ed., vol. iv. 186.]

[Footnote 47: Those familiar with the accuracy and diagnostic skill of
the lamented Sibson will not hesitate to add his 13 cases of very
probable endocarditis to his 170 positive cases of cardiac inflammation
in 325 examples of acute rheumatism, which will raise his percentage to
56.3.]

[Footnote 48: _Lib. cit._, vol. xiv. 6.]

[Footnote 49: _Lib. cit._, 264.]

[Footnote 50: See Senator in _Ziemssen's Cyclopæd. Pract. of Med._,
xvi. 49.]

[Footnote 51: _Pract. Med._, 5th ed., 314.]

The frequency of cardiac complications in rheumatism is influenced by
several circumstances. Some unexplained influence, such as is implied
in the terms epidemic and endemic constitution, appears to obtain.
Peacock found the proportion of cardiac complications in rheumatism to
range from 16 to 40 per cent. during the five years from 1872 to 1876,
and a similar variability is shown in Southey's statistical table[52]
covering the eleven years from 1867 to 1877. Be it observed that these
variations occurred in the same hospitals and under, it may be
presumed, very similar conditions of hygiene and therapeusis. Youth
predisposes to rheumatic inflammation of the heart, so that it may
still be said that the younger the patient the greater the proclivity.
Of Fuller's cases, 58 per cent. were under twenty-one, and the
liability diminished very markedly after thirty. Of Sibson's cases, 62
per cent. were under twenty-one. In infancy and early childhood the
liability is very great, and at those periods of life the heart, and
more especially the endocardium, rarely escapes; and the cardiac
inflammation often precedes by one or two days the articular. The
careful observations of Sibson confirm the spirit, but not the letter,
of Bouilland's original statement, and proves that the danger of heart
disease is greater in severe than in mild cases of acute rheumatism,
and that this is especially true of pericarditis. (It may be remarked
here, en parenthese, that the number of joints affected is {33} very
generally in proportion to the severity of the attacks.) However, the
mildest case of subacute rheumatism is not immune from cardiac
inflammation, and it has occasionally been observed even in primary
chronic rheumatism.[53] Occupations involving hard bodily labor or
fatigue, whether in indoor or outdoor service, render the heart very
obnoxious to rheumatic inflammation. Existing valvular disease, the
result of a previous attack of rheumatism, favors the occurrence of
endocarditis in that disease. Some authorities maintain that treatment
modifies the liability to rheumatic affection of the heart, and this
will be spoken of hereafter. The period of the rheumatic fever at which
cardiac inflammation sets in varies very much, but it may be
confidently stated that it occurs most frequently in the first and
second weeks, not infrequently in the third week, seldom in the fourth,
and very exceptionally after that, although it has happened in the
seventh. An analysis of Fuller's experience[54] in 22 cases of
rheumatic fever and 56 of endocarditis--a total of 78--shows that the
disease declared itself under the sixth day in 8; from the sixth to the
tenth in 29; from the tenth to the fifteenth in 17; from the fifteenth
to the twenty-fifth in 18; and after the twenty-fifth in 6. The
friction sound was audible in Sibson's 63 cases of rheumatic
pericarditis--from the third to the sixth day in 10, and before the
eleventh day in 30, or nearly one-half of the whole. That observer
concludes "that in a certain small proportion of the cases, amounting
to one-eighth of the whole," the cardiac inflammation took place at the
very commencement of the disease, and simultaneously with the invasion
of the joints.[55]

[Footnote 52: _Lib. cit._]

[Footnote 53: Raynaud, _Nouveau Dict. de Méd. et de Chir._, t. viii.
367.]

[Footnote 54: _Lib. cit._, pp. 77-278.]

[Footnote 55: _Lib. cit._, p. 209. See also Dickinson in _Lancet_, i.,
1869, 254; Bauer in _Ziemssen's Cyclopæd._, vi. 557.]

Of the several forms of rheumatic cardiac inflammation, endocarditis is
the most frequent, and in a large proportion of cases it may exist
alone; pericarditis is also very often observed, but it seldom is found
per se, being in the vast majority of cases combined with endo- and
occasionally with myocarditis. It is generally the ordinary verrucose
endocarditis that obtains. The ulcerative form occurs sometimes, and
should be suspected if in a mild or protracted case of acute rheumatism
endocarditis sets in with, or is accompanied by, rigors, and the
general symptoms are of pyæmic or typhoid character or both, even
although an endocardial murmur is not present, for extensive vegetating
ulcerative endocarditis frequently exists without audible murmur. It is
remarkable, as Osler has shown,[56] how few instances of ulcerative
endocarditis developing during the course of acute rheumatism are
reported; and I would add that by no means all of these were examples
of first attacks, chronic valvular lesions, the consequence of former
illness, existing in many of them at the time of the final acute
attack. Southey's[57] patient, and both of Bristowe's,[58] had had
previous rheumatic seizures. However, Peabody's case,[59] one of Ross's
three cases,[60] and Pollock's[61] case appear to have been examples of
ulcerative {34} endocarditis occurring during a first attack of acute
articular rheumatism. The united and thickened condition of two
segments of the aortic valve in one of Ross's cases indicates
old-standing disease, although no history of former rheumatism is
given. Goodhardt[62] has lately insisted upon the tendency of
ulcerative endocarditis to appear in groups or epidemics, but the
evidence is not conclusive.

[Footnote 56: _Archives Médecine_, vol. v., 1881; _Trans. International
Med. Cong._, vol. i. 341.]

[Footnote 57: _Clin. Soc. Trans._, xiii. 227.]

[Footnote 58: _Brit. Med. Jour._, i., 1880, 798.]

[Footnote 59: _Medical Record N.Y._, 24th Sept., 1881, 361.]

[Footnote 60: _Canada Med. and Surg. Journ._, vol. xi., 1882, 1, and
_ib._, vol. ix., 1881, 673.]

[Footnote 61: _Lancet_, ii., 1882, 976.]

[Footnote 62: _Trans. Path. Soc. London_, xxxiii. 52.]

Space will not permit any detailed description of the symptoms and
signs of endo- or pericarditis: these will be found in their proper
places in this work, but a few observations are needed upon
myocarditis, which occasionally occurs in combination with rheumatic
pericarditis, and is a source of much more danger than the latter is,
per se. Dr. Maclagan[63] is almost the only authority who recognizes
the occurrence of rheumatic myocarditis independently of inflammation
of the membranes of the heart. He maintains that the rheumatic poison
probably and not infrequently acts directly on the cardiac muscle; in
which case the resulting inflammation is apt to be diffused over the
left ventricle and to produce grave symptoms, while in other instances
the inflammatory process begins in the fibrous rings which surround the
orifices of the heart (especially the mitral), extends to the substance
at the base of the heart, and is there localized. As in this latter
form the inflammation usually extends also to the valves, "any symptoms
to which the myocarditis gives rise are lost in the more obvious
indications of the valvulitis." However, this limited inflammation of
the myocardium is not dangerous. Dr. Maclagan asserts that the more
diffused and dangerous inflammation of the walls of the left ventricle,
while always difficult, and sometimes impossible, of diagnosis, can be
determined with tolerable certainty in some cases. In this view,
however, he has been preceded by Dr. Hayden,[64] who states that the
diagnosis of myocarditis is quite practicable irrespective of the
accompanying inflammation of the membranes of the heart.

[Footnote 63: _Rheumatism: its Nature, Pathology, and Successful
Treatment_, 1881.]

[Footnote 64: _Diseases of the Heart and Aorta_, 1875, 746.]

From the observations of the author just named, as well as of many
others, it may be inferred that acute diffused myocarditis of the left
ventricle exists in rheumatic fever when either with or without
coexisting pericarditis there are marked smallness, weakness, and
frequency of pulse, anguish or pain or great oppression at the
præcordia, severe dyspnoea, the respiration being gasping and
suspirious, feeble, rapid, and irregular action of the heart, great
weakness of the cardiac sounds, and almost extinction of the impulse,
evidence of deficient aëration of the blood combined with coldness of
surface, tendency to deliquium, and when these symptoms and signs
cannot be fairly attributed to extensive pericardial effusion or to
pulmonary disease, or to obstructed circulation in the heart consequent
upon endocarditis with intra-cardiac thrombosis or upon rupture of a
valve. It might, however, be impossible to exclude endocarditis
complicated with thrombosis, conditions which do occur in rheumatic
endocarditis, or a ruptured valve, which, although rarely, has been
occasionally observed. Grave cerebral symptoms, delirium, convulsions,
coma, though frequently present, are not peculiar to acute
myocarditis.[65] {35} Hence, even with the above group of clinical
facts, the diagnosis at best can be but probable. The disease, too, may
be latent, or, like Stanley's[66] celebrated case, produce disturbances
of the cerebral system rather than of the circulatory.

[Footnote 65: In illustration see case by Southey in which the symptoms
and signs agree very well with the above description, and yet, although
the heart's substance was of dirty-brown color and the striation of its
fibre lost, Southey did not believe these appearances due to carditis.
(_Clin. Trans._, xiii. p. 29.)]

[Footnote 66: _Med.-Chir. Trans._, vol. vii.]

Dr. Maclagan has advanced the opinion that a subacute myocarditis is
not of uncommon occurrence in acute articular rheumatism, and may be
unattended by endo- or pericarditis. Such a condition, he says, may be
diagnosed when early in the course of the case the heart's sounds
quickly become muffled rather than feeble. As he quotes but one
case[67] in which an autopsy revealed alterations in the walls of the
heart, and as endocarditis and a little effusion in the pericardium
coexisted, it is premature to accept the evidence as final, and the
great importance of the subject demands further investigation.

[Footnote 67: _Lib. cit._, p. 175.]

Admitting with Fuller the occasional deposition of fibrin upon the
valves and endocardium in rheumatic fever independently of
endocarditis, the murmur resulting therefrom could not be reliably
distinguished from that of inflammatory origin. It remains to speak
briefly of temporary incompetence of the mitral and tricuspid valves
and their dynamic murmurs, and of hæmic murmurs. Occasionally, in
severe cases of rheumatic fever, more especially in the advanced stage,
there may be heard a systolic murmur of maximum intensity either in the
mitral area or over the body of the left ventricle, unaccompanied by
accentuation of the second sound, or, as a general rule, by evidence of
pulmonary obstruction. Such murmurs are apt to be intermittent, and as
they disappear on the return of health, they have been satisfactorily
referred to temporary weakness of the walls of the heart, so that the
auriculo-ventricular orifices are not sufficiently contracted during
the ventricular systole for their valves to close them, and
regurgitation follows. Yet, inasmuch as Stokes distinctly mentions the
absence of murmur in many cases of softening of the heart in typhus, it
is probable that an excessive weakness of the ventricular wall is
incompatible with the production of murmur, and that the presence of
murmur in such circumstances is evidence of some remaining power in the
heart.

Dr. D. West[68] has published some cases of acute dilatation of the
heart in rheumatic fever which strongly corroborate these views. The
murmur in one of them became appreciable only as the heart's sounds
increased in loudness and the dilatation lessened. One ended fatally,
and acute fatty degeneration of the heart's fibres was found in
patches.[69] I believe that some of these temporary mitral murmurs in
acute rheumatism depend upon a moderate degree of valvulitis quite
capable of complete resolution. Sibson[70] has lately stated that he
has met with the murmur of tricuspid regurgitation without a mitral
murmur in 13 out of 107 cases of rheumatic endocarditis, and with a
recent mitral murmur in 27 out of 50 {36} cases. "The tricuspid murmur
generally comes into play about the tenth or twelfth day of the primary
attack, along with symptoms of great general illness;" it appears
earlier, as a rule, in those cases in which it is associated with
mitral regurgitation than when it exists alone; it is of variable
duration, but usually short--from one to nineteen days or more. He
regards it as of non-inflammatory origin, and dependent upon
regurgitation due to the so-called safety-valve function of the
tricuspid valve; and when limited to the region of the right ventricle
he infers that it is usually the effect and the evidence of
endocarditis affecting the left side of the heart. These novel
statements are confirmed by the observations of Parrot, Balfour, and
William Russell,[71] which go to prove that tricuspid regurgitation
occurs frequently in the more advanced stages of debility. No other
authority than Sibson, however, insists upon its frequent occurrence in
acute rheumatism.

[Footnote 68: _Barth. Hosp. Repts._, xiv. 228.]

[Footnote 69: On this subject see Stokes, _Dis. Heart and Aorta_, pp.
423, 435, 502; Stark, _Archives générales de Méd._, 1866; DaCosta,
_American Journal Med. Sci._, July, 1869; Hayden, _Dis. Heart and
Aorta_, 1875; Balfour, _Clin. Lects. on Heart and Aorta_, 1876; Cuming,
_Dublin Quart. Jour. Med. Sci._, May, 1869; Nixon, _ib._, June, 1873.
I. A. Fothergill has seen several cases in which such mitral murmurs
have followed sustained effort in boys, and have disappeared after a
time: _The Heart and its Diseases_, 2d ed., 1879, p. 177.]

[Footnote 70: Reynolds's _System. Med._, Eng. ed., vol. iv. 463.]

[Footnote 71: See _Brit. Med. Jour._, i. 1883, 1053.]

The anæmia which is so striking a symptom of rheumatic fever,
especially when several joints are severely inflamed, coexists very
frequently with a systolic basic murmur, which is most often louder
over the pulmonary artery (in second left intercostal space and more or
less to left of sternum) than over the aorta. The murmur may appear
early in the disease, but sets in most frequently when the disease is
subsiding. When thus appearing late in a case accompanied by
endocarditis and pulmonary congestion, it is of favorable omen and
indicates improvement in the thoracic affection. The growing opinion,
however, respecting so-called anæmic murmurs is, that they depend
chiefly upon regurgitation through the tricuspid orifice, although Dr.
W. Russell refers them to pressure of a distended left auricle upon the
pulmonary artery.[72]

[Footnote 72: _Ib._, 1065.]

Pulmonary affections in form of pleuritis, pneumonia, or bronchitis are
common complications of rheumatic fever. Adding Latham's,[73]
Fuller's,[74] Southey's,[75] Gull and Sutton's,[76] Pye-Smith's,[77]
and Peacock's[78] cases together, we have a total of 920 in which some
one or more of the above pulmonary affections obtained in 109
instances, or 11.8 per centum. A further analysis of Latham's and
Fuller's cases shows that it is especially when rheumatic fever is
complicated with cardiac disease that the lungs suffer; thus, pulmonary
affections obtained in 26.5 per cent. of cases complicated with heart
disease, and in only 7 per cent. of cases free from that disease. It is
more especially when pericarditis complicates rheumatic polyarthritis
that pulmonary affections occur. Thus, these were found in only 10.5
per cent. of cases of recent rheumatic endocarditis, in 58 per cent. of
cases of pericarditis, and in 71 per cent. of cases of
endo-pericarditis. The tendency which inflammation of the pericardium
has to extend to the pleura probably partially accounts for the more
frequent association of the pulmonary affections with rheumatic peri-
than with rheumatic endocarditis. (Sibson found pleuritic pain in the
side twice as frequent in pericarditis, usually accompanied with
endocarditis (31 in 63), as in simple endocarditis, 26 in 108.[79]) But
the greater severity of those cases of rheumatic fever complicated with
peri- or endo-pericarditis must also have a decided influence in
developing the pulmonary affections. {37} Pneumonia and pleuritis are
very frequently double in rheumatic fever, and are often latent,
requiring a careful physical examination for their detection. So
suddenly does the exudation take place in some cases of rheumatic
pneumonia that the first stage is not to be detected either by symptoms
or signs. On the other hand, in some cases the absence of the typical
signs of hepatization, the want of persistence in the physical signs,
and their rapid removal, and even in rare instances an obvious
alternation between the pulmonary and the articular symptoms, suggest
that the process often stops short of true hepatization, and partakes
rather of congestion and splenization, with or without pulmonary
apoplexy--a view which has been occasionally confirmed by the
autopsy.[80]

[Footnote 73: Latham's _Works_, Syd. Soc., i. 98 _et seq._]

[Footnote 74: _Lib. cit._, 317.]

[Footnote 75: _Bartholomew Hospital Reports_, xv. 14.]

[Footnote 76: _Guy's Hosp. Reports_, 3d Series, xi. 434.]

[Footnote 77: _Ib._ xix. 324.]

[Footnote 78: _St. Thomas's Hospital Reports_, x. 12-17.]

[Footnote 79: Reynolds's _System Med._, iv. 233.]

[Footnote 80: Vide Sturges, _Natural History and Relations of
Pneumonia_, 1876, pp. 70-78; T. Vasquez, Thèse, _Des complications
Pleuro-pulmonaires du Rheumatisme Artic. Aigue_, Paris, 1878, pp.
25-31; M. Duveau, _Dictionnaire de Méd. et de Chir._, t. xxviii. p.
443.]

Active general congestion of the lungs has occasionally been observed
in this disease, and has proved fatal in five minutes[81] and in an
hour and a half[82] from the invasion of the symptoms. The rheumatic
poison frequently excites pleuritis, some of the characters of which
are--the suddenness with which free effusion occurs; the promptness
with which it is removed, only perhaps to invade the other pleura, and
then to reappear in the cavity first affected; the diffusion of the
pain over the side and its persistence during the effusion; and its
frequent concurrence with pericarditis, and in children with
endocarditis; its little tendency to become chronic, and its marked
proclivity to become double. It is often latent and unattended with
pain. Sibson asserts that if in rheumatic pericarditis "pain over the
heart is increased or excited by pressure over the region of the organ,
it may with an approach to certainty be attributed to inflammation of
the pleura," etc. The product of the inflammation is commonly serous,
but occasionally purulent.

[Footnote 81: _Thèse d'Aigue pleur._, 1866, par B. Ball.]

[Footnote 82: M. Aran, quoted by Vasquez, _lib. cit._, p. 14.]

The disturbances of the nervous system are amongst the most important
complications of acute rheumatism, and are due either to functional
disorder or very rarely to obvious organic lesions of the nerve-centres
or their membranes. The dominant functional disturbance may be
delirium, which is greatly the most frequent; or coma, which is rare;
or chorea, very frequently observed in children; or tetaniform
convulsions, which occur very seldom per se. As a rule, two or more of
these forms coexist or alternate with or succeed one another, and the
grouping, as well as the variety, of the symptoms may be greatly
diversified. In 127 observations there were 37 of delirium only, 7 of
convulsions, 17 of coma and convulsions, 54 of delirium, convulsions,
and coma, 3 of other varieties (Ollivier et R., cited by Besnier).

Rheumatic Delirium.--Either with or without subsidence of the articular
inflammation, about from the eighth to the fourteenth day of the
illness, but occasionally at its beginning, or sometimes on the eve of
apparent convalescence, the patient becomes restless, irritable,
excited, and talkative; sleep is wanting or disturbed; some excessive
discharge from the bowels or kidneys occasionally occurs; profuse
perspiration is usually present, and may continue, but frequently
lessens or altogether ceases; the skin becomes pungently hot, the
temperature generally--not always, however--rising rapidly toward a
hyperpyrexial point, and ranging from {38} 104° to 111°; and transient
severe headache and disturbances of special sense sometimes obtain. At
a later period, or from the outset in hyperacute cases, flightiness of
manner or incoherence in ideas is quickly succeeded either by a low
muttering delirium, twitchings of the muscles, violent tetaniform
movements and general tremors, and a condition perhaps of coma-vigil,
or by an active, noisy, even furious, delirium. The articular pains are
no longer complained of, and sometimes the local signs of arthritis
also quickly disappear; but neither statement is uniformly true. The
pulse becomes rapid; prostration extreme; semi-consciousness or marked
stupor gradually or rapidly supervenes; the temperature continues to
rise; the face, previously pale or flushed, becomes cyanotic; and very
frequently death ensues, either by gradual asthenia or rapid collapse,
often preceded by profound coma or rarely by convulsions. Deep sleep
often precedes prompt recovery.

The duration of the nervous symptoms varies from one or two, or more
usually six or seven, hours in very severe cases, to three or four days
in moderate ones, or occasionally seven, eight, or sixteen[83] or
twenty-nine days[84] in unusually protracted cases. In the
last-mentioned, however, the delirium is not usually constant, and
frequently disappears as the temperature falls, and recurs when its
rises. Moreover, a rapid and extreme elevation of temperature is
frequently altogether wanting.

[Footnote 83: Southey's case, _Clin. Soc. Trans._, xiii. p. 25.
Sleeplessness preceded it for four days, and there was no
hyperpyrexia.]

[Footnote 84: Graham's case, _ib._, vi. p. 7. Delirium set in on the
seventh day of illness, and three days after invasion of joints.
Temperature 104.8° early in disease; never exceeded 106°, probably
owing to repeated use of cold baths. Temperature at death, 104.2°.]

No real distinction can be established between these protracted cases
of rheumatic delirium and so-called rheumatic insanity, in which occur
prolonged melancholia, with stupor, mania, hallucinations, illusions,
etc., often associated with choreiform attacks. This variety may be of
short duration or continue until convalescence is established, or may
rarely persist after complete recovery from the articular affection.

Coma may occur in acute rheumatism without having been preceded or
followed by delirium or convulsions, although it is very rare; and,
like delirium, it may obtain without as well as with peri- or
endocarditis or hyperpyrexia. It usually proves very rapidly fatal. In
Priestly's case, an anæmic woman of twenty-seven, during a mild attack
of acute rheumatism, one night became restless; at 3 A.M. the pain
suddenly left the joints; apparent sleep proved to be profound coma,
and at 6 A.M. she was in articulo mortis.[85] Southey relates the
history of a girl of twenty who, without previous delirium or high
temperature, suddenly became unconscious, and died in half an hour.[86]
One of Wilson Fox's cases had become completely comatose, and was
apparently dying nine hours after the temperature had rapidly risen to
109.1°, when she was restored to consciousness by a cold bath and ice
to her chest and spine.[87]

[Footnote 85: _Lancet_, ii., 1870, 467.]

[Footnote 86: _Clin. Soc. Trans._, xiii. p. 29.]

[Footnote 87: _The Treatment of Hyperpyrexia_, 1871, 4.]

Convulsions of epileptiform, choreiform, or tetaniform character
frequently succeed the delirium, but in exceptional cases they occur
independently of it, and may even prove fatal.

Besides the choreiform disturbances which occur in connection with
delirium, stupor, tremor, etc. in cerebral rheumatism, simple chorea is
{39} frequently observed as a complication or a sequence, or even as an
antecedent, of acute articular rheumatism, and they occasionally
alternate in the same patient and in the same family. Chorea is perhaps
most frequently seen in mild cases and in the declining and
convalescent stages of rheumatic fever, and, while very common in
childhood and adolescence (five to twenty), it is very rare later in
life.

Such are the chief functional disturbances of the brain met with in
rheumatic fever, and the post-mortem examination reveals in them either
quite normal naked-eye appearances, or more frequently, especially in
rapidly fatal cases, general congestion of the pia mater, and to a less
degree of the cerebral substance, or in more protracted cases a greater
or less increase of transparent or opalescent serum in the subarachnoid
space and ventricles. The serum may be slightly or deeply tinged with
blood. If the serous or sero-sanguinolent effusion be considerable, the
encephalic mass or portions of it may be anæmic. But besides these
conditions, which are also commonly observed in many other febrile
diseases, and which are probably only concomitants of the functional
disturbance arising in the advanced stage of acute articular
rheumatism, certain organic affections of the nervous centres or their
membranes occasionally occur in this disease, and are plainly the cause
of the cerebral disturbance observed during life. Cerebral meningitis,
although very rare as a complication of acute articular rheumatism,
except in certain hot climates, like that of Turkey,[88] does occur,
and lymph or pus is found, usually over the convexity of the brain, but
sometimes at the base and down the cord.[89] The symptoms of rheumatic
cerebral meningitis are very like those of rheumatic delirium;
vomiting, and even, but less frequently, pain in the head, may be
absent, while hyperpyrexia may coexist (Foster's case), although not
necessarily present. Should the pulse from being frequent become slow
and irregular, and any paralytic symptoms ensue, meningitis may be
suspected. In some of these cases the meningitis is a consequence of
ulcerative endocarditis and embolism of the cerebral vessels,[90] but
in others it obtains without endocarditis or any purulent formation
elsewhere than in the meninges, as there is probably a true rheumatic
localization like pericarditis. The articular inflammation may continue
after the invasion of the meningitis, or the latter may promptly follow
the disappearance of the former, as though a metastasis of morbid
action had taken place.[91] In many instances, according to Ollivier,
Ranvier, Behier, and others, although the macroscopic signs of
meningitis are absent, the microscope detects proof of its presence in
the existence of an increased number of vessels, fatty granulations on
their walls, proliferation of nuclei and capillary
extravasations--histological conditions identical with those found in
the mild degrees of rheumatic inflammation of the joints.

[Footnote 88: Senator, in _Ziemssen_, xvi. 50.]

[Footnote 89: Watson's _Prac. Physic_, 1872, Am. ed. vii. 335; Fyfe,
_Med. Gazette_, vol. xxix. 703; Fuller, _lib. cit._, 302; Leudet,
_Clin. Médicale_, 139; Dowse, _London Lancet_, ii. 1872, 9; Foster,
_ib._, ii. 1868, 115; Hicks, _New York Medical Record_, Nov., 1878,
404.]

[Footnote 90: That ulcerative endocarditis frequently produces
meningitis is illustrated by Osler's cases, 4 out of 7 of which were
complicated with purulent meningitis: _Transactions of International
Med. Congress_, 1881, i. 344.]

[Footnote 91: See a case reported by W. L. Ramsey in _New York Medical
Record_, i., 1881, p. 9.]

Embolism of the cerebral arteries, producing meningitis, or more
frequently softening of the cerebral substance or hemorrhage, or
proving {40} fatal before necrobiosis has time to set in, is an
occasional complication of acute articular rheumatism. A young lady,
while under my care suffering from her first attack of articular
rheumatism complicated with endocarditis, became suddenly hemiplegic
and aphasic, and died twelve hours later. In a girl of thirteen, the
subject of acute articular rheumatism complicated with ulcerative
endocarditis, right hemiplegia suddenly occurred, and at the autopsy
Bristowe found an embolon in the left middle cerebral artery and a
softened area in the left corpus striatum. Bradbury reports a primary
acute rheumatism with endocarditis, delirium, and coma, but without
paralysis, in which a plug was found in the right middle cerebral
artery, but the brain was quite healthy.[92]

[Footnote 92: _Lancet_, ii., 1870, 148; also a case in _Lancet_, i.,
1882, p. 605: in eighth week of subacute articular rheumatism;
embolism; right hemiplegia. Autopsy: large vegetations on valves;
obstruction in middle cerebral artery.]

Very much the same observations are applicable to the disturbances of
the spinal cord and its envelopes in rheumatic fever as have been made
in reference to those of the cerebrum and its coverings. They may exist
with or without any alteration of the cord or membranes to which they
can be reliably referred; that is to say, they may be simply functional
in the peculiar sense in which that word is now understood, or they may
be connected with obvious structural changes, and chiefly with those
indicating inflammation of the membranes or substance of the cord. The
spinal symptoms may precede the articular affection, but generally
appear after it. They sometimes closely resemble those of idiopathic
tetanus,[93] or of spinal meningitis, or of myelitis, or of
meningo-myelitis; and in the last case, along with severe rachialgia,
muscular rigidity, cutaneous and muscular hyperæsthesia, and neuralgic
pains, there will occur numbness and more or less paralysis of the
lower extremities,[94] bladder, and rectum (paraplegia). These spinal
disturbances may or may not be accompanied by hyperpyrexia, and when
simply functional they are usually less severe and persistent, have a
greater tendency to alternate with one another and with the articular
affection, and are more amenable to treatment, than when due to those
very rare complications of rheumatic fever, spinal meningitis or
meningo-myelitis. The inflammation may involve both the cerebral and
spinal membranes at the same time.

[Footnote 93: Bright's case, 2, _Med.-Chirurgical Transactions_, xxii.
4; Dr. E. C. Mann, _N.Y. Medical Record_, 1875, 38; Bouilland, _Traité
sur les Maladies du Coeur_, t. i. p. 33.]

[Footnote 94: Leudet, _lib. cit._, p. 139; Dowse, _Lancet_, i., 1872,
9.]

The causes of these disturbances of the nervous system, when not
attributable to appreciable lesions, such as congestion, inflammation,
hemorrhage, embolism, thrombosis, and softening, are not established.
The following appear to be reasonable conclusions from the facts at
present known:

The most constant condition, and without which these cerebral symptoms
very rarely arise, appears to be some susceptibility or vulnerability
of the nervous system, inherited or acquired, rendering it apt to be
disturbed by influences which less susceptible centres would
successfully resist. Trousseau, who has especially advocated this
view,[95] considered intemperance in the use of spirits to be a
frequent source of this nervous predisposition. Accepting this neurotic
predisposition as the factor generally present when acute articular
rheumatism is complicated {41} with disturbances of the nerve-centres,
we may inquire what are the circumstances in the disease capable of
developing into activity the predisposition.

[Footnote 95: _Clin.-Med._, Syd. ed., i. 513 _et seq._]

Unquestionably, the existence of acute pericarditis, or of
endocarditis, or of inflammation of the lungs or pleura, is one of
those conditions.

Probably hyperpyrexia acts in some cases as an exciting cause of the
nervous phenomena, for while the delirium preceded the hyperpyrexia in
6 cases, it accompanied it in 19 and followed it in 10;[96] and the
nervous symptoms disappear when the hyperthermia is removed by the
employment of cold, and recur with the return of high temperature. The
phenomena of sunstroke and heat-apoplexy prove that a high temperature
is capable of producing convulsions and coma. That these grave cerebral
disturbances are so infrequent in acute rheumatism (obtaining in about
3 or 4 per cent. only) is probably owing to the usual moderate range of
temperature and the rarity of hyperpyrexia in the disease. Still, while
hyperpyrexia is a disturber of cerebro-spinal function, too much
importance must not be attached to it, for not only does such
disturbance very frequently precede the hyperpyrexia, but there are
many facts indicating that the hyperpyrexia is itself very frequently,
like the delirium, tremor, and coma which precede or accompany it, but
a consequence of disorder, usually of a paralyzing kind, of the
nerve-centres. It has been met with in lesions of the pons, in tetanus,
in injuries of the cord, in some cases of non-inflammatory softening of
the brain and of cerebral hemorrhage; that is, in a class of affections
not belonging to the specific fevers, but to those directly disturbing
or destroying the functions of the nerve-centres. And cases of acute
rheumatism do rarely occur in which a very high temperature is not
accompanied by cerebral disturbances. Sibson quotes two such,[97] one
of which, with a temperature of 110.8°, was only restless and talked
when asleep, and the other, with a temperature of 106.3°, presented
only vomiting and dyspnoea. Cardiac inflammation was absent in both.
DaCosta relates one in his valuable paper upon cerebral rheumatism in
which, although the temperature was 110°, no cerebral symptoms nor
cardiac affection existed.[98]

[Footnote 96: "Abstract Report upon Hyperpyrexia in Ac. Rheum.," _Brit.
Med. Jour._, 1882, p. 807.]

[Footnote 97: _Lib. cit._, p. 264.]

[Footnote 98: This essay contains a record of 11 cases of cerebral
rheumatism and several autopsies: _Am. Jour. Med. Sci._, 69, 1845, p.
36, case xi.]

The goodly number of instances lately published in which grave cerebral
symptoms have obtained in acute articular rheumatism at ordinary
febrile temperatures, while they prove that hyperthermia is not an
essential condition productive of such symptoms, require to be
explained. Some such, no doubt, have been instances of marked
predisposition, so that a moderate febrile temperature or some
complication sufficed to disturb the brain, as we see in typhoid and
other fevers, in pneumonia, etc. If there be a rheumatic poison--which
has not yet been proved--it may, in predisposed persons, produce the
cerebral symptoms. The argument[99] that such poison should produce
inflammation of the nervous centres if it acted directly on them is not
convincing. It need not necessarily produce similar alterations in
serous or synovial membranes and in nervous tissues. Many toxic agents
disturb, and even suspend, the {42} cerebro-spinal functions, and leave
no appreciable changes in them. Do these cases prove that there is
something peculiar to rheumatic fever which tends to disturb the
nervous centres? Hardly; for while such disturbance is comparatively
rare in that disease, it is observed frequently in many other febrile
affections, notably in typhus, scarlatina, and small-pox; and as in
these, so in rheumatic fever, it is more often observed in the severe
than in the mild cases, as though it were a part of the systemic
disturbance incident to the febrile affection and largely proportionate
to its severity.

[Footnote 99: Maclagan, _Rheumatism: Its Nature, Pathology, etc._,
1881, 287.]

Yet there is something special in acute rheumatism which perhaps has to
do with the occurrence as well as the severity of the cerebro-spinal
symptoms and of the hyperpyrexia; viz. the long duration and severity
of the pain, and the number and importance of the parts, in addition to
the articulations, which are one after the other or simultaneously
involved in severe inflammation--peri-, endo-, myocardium, lungs,
pleura, etc. Perhaps in no other acute febrile disease are so many
distinct and important organs involved in inflammation at the same time
or in rapid succession; and it is no wonder that the functions of the
nervous system should in consequence become greatly depressed,
exhausted, or disturbed.

The kidneys appear very rarely to suffer serious disease in acute
rheumatism, if we except embolism of their arteries due to
endocarditis; and it is very doubtful whether the rare instances[100]
in which an acute parenchymatous nephritis has been observed in acute
rheumatism can be referred to direct rheumatic inflammation, or not,
rather, to the operation of the exposure which induced the rheumatism.
Further investigation is needed to determine whether interstitial
nephritis is even very exceptionally an indirect consequence of
rheumatism, as Lancereaux admits.

[Footnote 100: See DaCosta's cases 1 and 2, _Cerebral Rheumatism, lib.
cit._; case 1 certainly favors the view that either the rheumatic
poison, if there be such, or the constitutional disturbance incident to
acute polyarticular rheumatism, may sometimes produce nephritis. See
also a case by A. Deroye, Thèse, Doctorat, Paris, 1874, quoted by P.
Coubere in _Contribution à l'Étude des Complications Renales du
Rheumatisme Artic. Aigue_, Paris, 1877.]

The other complications, being of less importance, must be but barely
alluded to. A pharyngitis attended with severe dysphagia and high fever
occasionally precedes the other symptoms or occurs in the early stage
of the disease. Gastralgia, enteralgia, simple serous diarrhoea, and
dysentery also rarely occur in acute rheumatism. That they are
sometimes, at least, truly rheumatic appears probable from the
circumstance that they may precede, follow, or alternate with the
articular affection, and are all intensely painful. I have but once met
with acute peritonitis as a complication of acute rheumatism; the
immunity of this serous membrane from rheumatic inflammation is an
inexplicable anomaly in view of the proclivity of the pericardium and
pleura to that process. Cystitis and orchitis are rare.

Several cutaneous affections are not unfrequently observed in relation
with acute rheumatism. Besides sudamina and miliaria rubra, which are
very common as consequences of the excessive perspiration,[101] there
{43} are others which may be themselves rheumatic manifestations. Such
are especially erythema marginatum,[102] e. papulatum, and e. nodosum.
A well-marked urticaria frequently precedes acute rheumatism in a
friend of the writer's; it may occur during its course or soon after
the cessation of the pains. Scarlatiniform eruptions are occasionally
observed, and very rarely punctiform hemorrhages--peliosis rheumatica
or rheumatic purpura. The purpuric symptom may be accompanied by
erythema or urticaria, and may precede, accompany, or alternate with
other rheumatic manifestations. Unlike purpura variolosa and idiopathic
purpura hæmorrhagica, this variety appears to be free from danger.

[Footnote 101: Dr. J. T. Metcalfe of New York many years ago showed me
a case of rheumatic fever in which the sweat-vesicles had run together,
forming, instead of the usual pearly globular vesicles, irregular flat
blebs, some of them equal in area to seven or nine primary vesicles,
filled with transparent fluid, and this fluid could be displaced by
pressure to adjacent parts, as though it lay simply under the
superficial epidermic layer. I have seen several similar cases since.]

[Footnote 102: Dr. Palmer relates a case complicated with erysipelas
and peritonitis in _Boston Med. and Surg. Journal_, 1868.]

Besides a slight local oedema affecting the malleoli, scrotum, eyelids,
etc., or accompanying the cutaneous eruptions just mentioned, a more
decided infiltration of the subcutaneous cellular tissue occasionally
exists in the vicinity of the inflamed joints and tendinous sheaths,
and more rarely extends to an entire limb, which may not only be
considerably enlarged and painful and resemble a milk leg, but may be
red, hot, and tender, and excite suspicion of phlegmonous erysipelas.
Phlebitis, although infinitely less frequent than in gout, has been
observed in acute articular rheumatism.[103] Jaccoud in 1871[104]
mentioned the exceptional occurrence of subcutaneous nodosities in
rheumatism, which he says Froriep first pointed out;[105] but Homolle
states that they had been previously mentioned by Sauvage and
Chomel.[106] Since then several independent observers have met with
this affection, and Drs. Thomas Barlow and Francis Warner of London
have lately written a short valuable paper upon the subject based upon
27 cases which they had separately or conjointly investigated. From
their paper the following account is chiefly derived:[107] These
nodules may vary in number from one to fifty, and in size from that of
a pin's head to the volume of an almond, and are quite subcutaneous,
firm and elastic, painless, and freely movable. They are not usually
attached to the skin, but to the tendons, deep fasciæ, pericranium,
periosteum, etc.; the integument over them is free from heat, redness,
and infiltration, although exceptionally tenderness on pressure and
slight redness may exist over them. They are found most frequently on
the back of the elbow, the malleoli, and margins of the patella, but
occur occasionally on the extensor tendons of the hand and foot, the
scapular spine and iliac crest, the temporal ridge and superior
occipital curved line, the ear, etc. These nodules occur singly or in
clusters, and are often symmetrical; they are very rapidly developed in
crops or in succession, and last sometimes for a few hours, more
frequently from three or four days to four or five months, or even
eighteen to thirty months. The original formations may disappear, and
be succeeded by fresh ones; and sometimes, when no longer perceptible
by touch, they may be found post-mortem. Their development is
unattended by pyrexia, unless pleuritis, pericarditis, or other
condition coexist to which the pyrexia might {44} be referred. These
nodosities do not appear to suppurate or ossify or become infiltrated
with urate of soda, and histologically they resemble organizing
granulative tissue. As regards their pathological associations, Drs.
Barlow and Warner found evidences of rheumatism in 25 out of 27 cases;
a morbid condition of the heart existed in all of them, and chorea in
10 of them. Two of the conclusions formulated by the authors just
mentioned are of great importance: that these subcutaneous nodosities
"may be considered as in themselves indicative of rheumatism, even in
the absence of pain;" that, while unimportant in themselves, they are
"of serious import, because in several cases the associated heart
disease has been found actively progressive." Dr. Dyce Duckworth has
reported two cases in which these nodules occurred in adults, lasted
eighteen months in one, and were still present in the other case after
thirty months, and were attached to the skin and periosteum. In one of
them the nodules were very painful and ached more in cold weather, and
the patient had no history of rheumatism or of chorea, although her
mother and one sister had.[108] In Dr. Stephen Mackenzie's case the
woman was the subject of tertiary syphilis, and had no personal history
of rheumatism or chorea, and she was free from heart disease; but her
family history was not given.[109]

[Footnote 103: _Phlebite Rheumatismale Aigue_, Paris, 1869, par M.
Lelong. In _Revue de Méd._, t. i. 492-499, 1881, a case by Dr.
Launois.]

[Footnote 104: _Pathologie Interne_, ii. 546, 1871.]

[Footnote 105: _Die Rheumatische Schwiele_, Weimar, 1843.]

[Footnote 106: _Lib. cit._, p. 628.]

[Footnote 107: _Trans. International Medical Congress_, London, vol.
iv. pp. 116-128, 1881. In this paper, and in an article by MM. E.
Troisier and L. Brock, to be found in _Revue de Médecine_, t. i.
297-308, 1881, are references to the authors who had written upon it.]

[Footnote 108: _Brit. Med. Journ._, i., 1883, 868.]

[Footnote 109: _Ibid._, i., 1883, 867.]

The course and duration of acute polyarticular rheumatism vary very
much, and are apparently influenced by several circumstances, such as
the severity or the mildness of the articular affection, as well as of
the constitutional disturbance; the presence or not of complications;
the state of health of the patient about the time of the attack, and,
probably, the existence or not of a proclivity to the disease; and
whether the disease present the continued or the relapsing type. As a
tolerably general rule, when the constitutional symptoms are acute, the
skin hot, the perspiration free and very acid, the urine of high
density, color, and acidity, and several of the articulations are
swollen and very painful--when no serious complication, and especially
no severe cardiac affection, exists, and when the patient is endowed
with a fair constitution and with organs not damaged by previous
disease, the course of the fever is tolerably short and continuous, and
the recovery more or less prompt. Amongst the most reliable evidences
of approaching recovery in such cases is the tongue becoming clean and
losing its red color and the urine increasing considerably in quantity,
but containing a large proportion of solid matter, as indicated by a
high density.

On the other hand, a large proportion of cases run a more irregular and
protracted course, and more or less marked relapses succeed real but
temporary improvements, the local disturbance affecting fresh joints or
reappearing in those previously attacked, and the general symptoms
resuming renewed activity. The duration of the active symptoms in these
cases is considerable, seldom under six weeks, and frequently occupying
seven, eight, or more. In these protracted cases the symptoms, as a
rule, are usually rather milder, the perspiration not as profuse or
sour, the urine of less density and acidity, the articulations less hot
and painful, than in the previously described group. Sometimes, indeed,
the perspiration and the urine are of neutral or even faintly alkaline
reaction. It is not only the unexplained tendency to relapse which
protracts these {45} cases, but sometimes in addition an established
proclivity to the disease--the rheumatic habit--or a condition of
previous unsound or frail health.

Such cases occasionally pass into the subacute form, or the mild
febrile symptoms gradually and finally decline, and the joints may
either remain tender, swollen, and stiff some time longer, or these
signs of recent inflammation may soon disappear and leave the
articulations merely weak.

Many cases of acute rheumatism embody several of the features of the
two groups just described, and no definite course or duration of acute
articular rheumatism can be accurately laid down.

The course and duration of acute polyarticular rheumatism have received
a good deal of attention of late years. But Dr. Austin Flint[110] was
one of the first to study the natural history of the disease
uninfluenced by active treatment, and he was followed in 1865,[111]
1866,[112] and 1869[113] by Sir William Gull and Dr. Sutton, who
treated a series of cases without medicine, unless mint-water be so
regarded. The mean duration of Flint's 13 cases from the date of attack
to convalescence, excluding one in which pericarditis and pneumonia
occurred, was a fraction under twenty-six days. It is unfortunate that
the number of cases was so small, and that 11 of the patients were
females, who appear to be especially subject to the milder and more
protracted attacks of the disease. A larger number, with an equal
proportion of the sexes, would probably have given a different result.

[Footnote 110: _American Journal of Med. Sciences_, July, 1863.]

[Footnote 111: _Ib._, vol. xii.]

[Footnote 112: _Medico-Chirurgical Transactions_, vol. lii.]

[Footnote 113: _Guy's Hospital Reports_, 2d Series, vol. xi.]

Gull and Sutton have published the natural histories of 62 cases--viz.
of 41 in their first series, of 8 more in their second, and of 13 more
in their third. The average duration of the acute symptoms was, in the
first series, 8.5 days, in the second, 9 days, and in the third, 10
days, giving an average of 9.1 days for the duration, after admission
to hospital, of the acute symptoms of acute polyarticular rheumatism
when there is no very severe cardiac disease. In their third paper,
based upon 13 new cases and 12 of those published in their two previous
communications, they conclude "that rheumatic fever uncomplicated with
any very severe heart affection tends to run its course in nineteen
days, calculating from the time the rheumatic symptoms first set in to
their termination."[114] Yet an analysis of the 23 of the 41 cases
contained in their first series[115] respecting which the duration of
the rheumatic symptoms before admission and from admission to complete
convalescence is given, shows that the period occupied from the setting
in of the rheumatic symptoms to convalescence was in the 13 male
subjects 25.8 days, and in the 10 female 42 days, or, including both
sexes, the average duration was 32.8 days--_i.e._ 6.8 days longer than
Flint's result.

[Footnote 114: _Med.-Chir. Trans._, lii. 82.]

[Footnote 115: _Guy's Hospital Reports_, xi. 435.]

As Gull and Sutton had especially pointed out the class that tends to
assume acute characters and recover more quickly than any other, and
the class that runs a protracted course and tends to relapse, it is
somewhat remarkable that they did not tabulate the cases belonging to
those classes separately, and show distinctly their differences in
duration and {46} modes of convalescence. This has been attempted by
Southey,[116] but, unfortunately, his conclusions, as will hereafter
appear, have not been confirmed by other observers.

[Footnote 116: _St. Bartholomew's Hospital Reports_, xiv., and _ib._,
xv.]

Finally, in this connection, after carefully weighing ten subjects of
acute articular rheumatism during their illness and until they had
regained their usual weight, A. Roussel[117] found that the time during
convalescence occupied in regaining the weight previously lost was
inversely proportional to the duration of the attack.

[Footnote 117: _Essai sur la Convalescence du Rheumatisme Artic.
Aigue_, Paris, 1881, 66.]


Subacute Articular Rheumatism.

Under this head Charcot, Besnier, and Homolle describe an affection
which corresponds closely with one variety of the disease commonly
called rheumatoid arthritis, but the writer employs the term with the
same significance as most modern English authors (Garrod, Sutton,
Flint, Maclagan). It is milder yet more enduring than the acute form,
but their symptoms are identical in kind. It is usually subacute from
the outset, although occasionally succeeding the acute type. The
febrile disturbance is but slight, rarely reaching 101°, and the
perspiration is less abundant; there is less pain, heat, and tenderness
in the joints, and only a few of them are involved together; but
although the articular affection moves from joint to joint, it persists
for weeks or months in several of them or in one only, improving and
relapsing generally without apparent reason. However, it does not
seriously damage the articulations, and they ultimately quite recover.
Mild cardiac affections also occur, but less frequently, and the
serious disturbances of the cerebral and respiratory systems are very
seldom met with. The gradations between subacute articular rheumatism
and the acute form on the one hand, and the simple chronic form on the
other, are almost innumerable. Marked anæmia is as much a feature of
subacute as of acute articular rheumatism, and its victims are often of
unhealthy or asthenic constitution, and subject to recurring attacks of
the disease on but slight provocation. The return of warm weather often
relieves such cases.

THE MORBID ANATOMY OF ACUTE AND SUBACUTE ARTICULAR
RHEUMATISM.--Although opportunities of ascertaining the conditions of
the articulations in acute articular rheumatism are rare, yet it is now
established that the process is an inflammation involving chiefly the
synovial membrane, and to a less degree the cartilages, ligaments,
tendinous sheaths, and in some cases even the bones and periarticular
soft parts. The synovial membrane is more or less injected and reddened
diffusely or in patches, especially where it forms fringe-like folds
and at its line of union with the cartilage. It is somewhat thickened,
opaque, and devoid of its satin-like lustre, and in somewhat protracted
cases covered here and there with a thin, easily detached
neo-membranous formation. Within the articulations will be found from a
few drops to one or two ounces of a viscid, pale, citron- or
reddish-colored fluid, like synovia, but more fluid, and generally
turbid and containing transparent or semi-opaque gelatinous masses or
albumino-fibrinous flocculi. The {47} microscope reveals in the
effusion large detached spherical epithelial cells in various stages of
germination or of fatty degeneration, and a variable number of red
blood-corpuscles and pus-cells. Very exceptionally, the effusion is
mixed with more or less true pus. In two out of the eight fatal cases
reported by Fuller, in which the joints were examined, pus in moderate
quantity was found along with other products in some, but not in all,
of the inflamed articulations, and one of them was complicated with
erysipelas, the other with sloughs over both trochanters. In very
severe forms complicated with hemorrhagic tendencies the inflammatory
products have contained a large proportion of blood. Cornil et
Ranvier[118] insist that even in slight cases of rheumatic arthritis
the diarthrodial cartilage constantly suffers changes arising from
nutritive irritation and proliferation of the cartilage-cells. At first
the cartilage loses here and there some of its polished hyaline
appearance, and the microscope reveals a finely-striated condition of
its structure which gives it a velvety aspect. When the inflammation
has been more severe and of longer duration, so that the deeper layers
have been involved, the unaided eye will perceive local swellings in
which the natural elasticity and resistance of the cartilage are
impaired, and its surface is fissured or villous-like in appearance.
"In certain rare cases of mono-articular acute arthritis true
ulcerations of the cartilage are observed."

[Footnote 118: _Manual d'Histologie pathologique_, Paris, 1869, 406.]

The soft parts in the immediate vicinity of the inflamed joints may be
in some cases more or less congested and oedematous, and the tendinous
sheaths, and even the bursæ mucosæ, inflamed and distended with
inflammatory products like those in the articulations. Charcot,[119]
holding the opinion that arthritis deformans is but a chronic variety
of articular rheumatism, quotes Gurlt's statement that in acute
articular rheumatism "the medullary tissue of the ends of the bones
undergoes a great increase of vascularity, with proliferation of its
corpuscles," and remarks that Hasse and Kussmaul have also referred to
lesions of the bone and periosteum in that disease. But the condition
of the osseous parts of the joints in acute articular rheumatism can
hardly be said to be known, and it is premature to speak positively
respecting it.

[Footnote 119: _Clinical Lectures on Acute and Chronic Diseases_,
Sydenham Soc., 1881, p. 148.]

Finally, in subacute rheumatism the alterations in the synovial
membrane, and especially in the cartilages just described, are likely
to be more marked than in the acute form.

The DIAGNOSIS of acute polyarticular rheumatism is seldom difficult in
adults, but when acute rheumatism localizes itself in one joint or
occurs in infancy or early childhood, a diagnosis, especially an early
one, sometimes cannot be easily established. The considerations by
which acute polyarticular rheumatism may be distinguished from acute
gout, subacute rheumatoid arthritis, and gonorrhoeal rheumatism will be
given in connection with those topics.

Pyæmia has perhaps been confounded with acute articular rheumatism more
than any other disease, but the rheumatic affection, unlike the pyæmic,
is not necessarily connected with any pre-existing condition capable of
causing purulent infection of the blood or system, such as a wound,
fracture, abscess, or a local inflammation of bone, periosteum, vein,
pelvic organ, or a specific fever (variola, relapsing, typhoid, {48}
glanders, etc.); it does not present severe rigors, which recur at
irregular intervals and are attended with teeth-chattering and a high
temperature, 104° to 105°, rapidly attained; its type of fever is not
so intermittent or markedly remittent as that of pyæmia; its profuse
sweating continues although the temperature remains febrile, but that
of pyæmia coincides with the decline of the temperature; unlike pyæmia,
it only very rarely produces profound constitutional disturbance of a
typhoid character, and has no tendency to run a rapidly fatal course in
eight to ten days or in two or three weeks; its visceral inflammations
are chiefly cardiac, pleural, and pulmonary, and tend to resolve; those
of pyæmia are especially pulmonary, pleural, and hepatic, although
frequently cardiac also, and generally produce suppuration and
destruction of tissue. Multiple subcutaneous abscesses and cutaneous
blebs and pustules do not occur in acute articular rheumatism, and its
articular affection differs in many respects from that of pyæmia; many
more joints are involved; the inflammation is erratic, very rarely
fixed, and generally resolves without damage to the articulation; the
affected joint is usually hotter, redder, more painful, and more
sensitive, and the swelling is less diffused, and its outline
corresponds more accurately with that of the synovial capsule.
Sometimes acute articular rheumatism is complicated with the phenomena
of pyæmia, as when so-called ulcerative endocarditis obtains.

The acute inflammations which are occasionally observed in one or
several articulations of newly-born infants are generally pyæmic. It is
only in the early stage of acute glanders that the severe muscular and
articular pains sometimes present in that very rare disease in man
might lead to its being confounded with acute articular rheumatism; but
the patient's occupation and history, the early and severe prostration,
the absence, as a rule, of redness and swelling around the painful
articulations, and, in some instances, the early appearance of pustules
and blebs on the skin and of abscesses in the deeper tissues, will
suggest the real nature of the case.

Acute periostitis frequently occurs in children in close proximity
either to one joint, or less frequently to more than one, and may
readily be confounded with acute articular rheumatism. But the
constitutional disturbance in acute periostitis is prompt and severe at
the outset; the swelling increases rapidly, is firmer than that of
arthritis, does not involve the joint proper and its capsule, but, like
the tenderness on pressure, exists above or below the articulations,
especially around the head of the bone; there are no visceral
complications, provided pyæmia has not supervened; the constitutional
symptoms early assume a typhoid character, and unless an early incision
be made a fatal issue soon ensues.

The enlarged ends of the long bones and the pains in the limbs of
rickets might lead to a suspicion of acute articular rheumatism, but
the early age of such children, the absence of pain and swelling in the
joints, the beaded condition of the sternal ends of the ribs, the late
dentition and locomotion, the peculiarly shaped head, and other
evidences of that affection, would prevent a careful observer from
making a mistake. Inherited syphilis in infants, like rickets, may
produce fusiform swelling and thickening at the ends of the long bones,
especially the humerus and femur, and sometimes pain in the joints on
movement; but at first the swelling {49} is confined to the epiphyseal
line, and only later extends to the joint; there is a pseudo-paralysis
of the limb, and but little pain or fever; bony osteophytes may often
be felt under the skin at the line of union of the epiphysis with the
shaft; the epiphysis often becomes separated from the shaft, and
suppuration may ensue around the bone and in the articulation;
sometimes adhesions and perforation of the integument take place,
allowing of the escape of disintegrating osseous and cartilaginous
tissue; and there will coexist either on the skin or mucous membrane
some of the ordinary evidences of inherited syphilis.[120] The acute
and subacute articular inflammations occasionally observed in cerebral
softening and hemorrhage, in injuries and inflammation of the spinal
cord and caries of the vertebræ, may be distinguished from acute and
subacute articular rheumatism by the following circumstances: the
existence of some one of these diseases of the brain or cord, the
articular affection being usually confined to the paralyzed limbs; its
invasion about the time of the setting in of the late rigidity, or even
still later; the absence of cardiac complications and the presence of
other trophic or neuro-paralytic lesions, such as acute sloughings,
rapid atrophy of the palsied muscles, cystitis, ammoniacal urine,
etc.[121]

[Footnote 120: Vide Parrot, _Archives de Physiol. Norm. et Path._, 1872
and 1876; R. W. Taylor, _Bone Syphilis in Children_, New York, 1875.]

[Footnote 121: See J. K. Mitchell, _Am. Jour. Med. Science_, vol.
viii., 1831, and _ib._, 1833; Scott Alison, _Lancet_, i., 1846, 276;
Brown-Séquard, _Lancet_, i., 1861; Gull, _Guy's Hosp. Repts._, 1858;
Charcot, _Archives de Physiologie_, t. i. p. 396, 1868, and many
others.]

Acute articular rheumatism in children presents peculiarities. It often
affects but one joint, and has little tendency to become general; the
joints of the lower extremity, ankle, and knee are most obnoxious; the
local signs of inflammation, redness, swelling, and pain, are feebly
developed, and the child may walk as if nothing were wrong; the disease
is usually subacute; the temperature rarely very high; the perspiration
not profuse; the urine not scanty, and not often loaded with lithic
acid. Cardiac and the other internal complications, except the
cerebral, are more frequent than in adults; endocarditis is especially
frequent, pericarditis and pleuritis not rare. It is almost exclusively
in childhood that acute articular rheumatism becomes associated with or
followed by chorea, and yet the delirium, coma, and convulsions
frequently observed during rheumatic fever in the adult are very rarely
seen in the child. Muscular rheumatism, however, in the form of
torticollis, frequently coexists, and so do erythema nodosum and the
subcutaneous fibrous nodules previously described.


Mono- or Uni-Articular Acute and Subacute Rheumatism.

It is very rarely indeed that acute rheumatism invades a single joint
to the exclusion of the rest; and it is perhaps impossible to be
certain that such an arthritis is rheumatic unless some of the other
symptoms or complications of articular rheumatism supervene, or unless
it have succeeded a polyarticular rheumatism, which it very rarely
does. Mono-articular rheumatism is very generally of the subacute type,
and unattended with fever from the outset, or only a moderate pyrexia
obtains for a few days; there is generally considerable effusion into
the joint, with {50} swelling, pain, and moderate local heat; visceral
complications very rarely arise, but the local inflammation persists
most obstinately for six or eight weeks or three or four months, and
often leaves the joint tender, stiffs, and weak for a long time or even
permanently. In both the acute and subacute forms, before concluding
that the uni-arthritis is rheumatic, we must exclude the probability of
its being traumatic, strumous, syphilitic, gonorrhoeal, neurotic, or,
above all, of the nature of rheumatoid arthritis, which many such cases
really are.

PROGNOSIS.--The disease is rarely directly fatal during the attack, yet
as the frequency of the complications varies unaccountably from time to
time, so the mortality may be exceptionally large or small for even
prolonged periods. It may be said that the average mortality ranges
between 1.16 and 4 per cent. in the experience of modern authors. The
average mortality in the Paris hospitals for four years (1868-69,
1872-73) Besnier fixes at 1.65 per cent.;[122] in St. Bartholomew's,
London, Southey found it for fifteen years (1861-75) to be 1.16 per
cent.;[123] Pye-Smith fixes the rate at 4 per cent. in 400 cases
treated in Guy's;[124] W. Carter gives 2.5 per cent. as the rate during
ten years at the Southern and Royal Southern Hospitals of
Liverpool.[125] The death-rate appears to vary remarkably with age, as
Southey's figures show:[126] under ten years, 3.40 per cent.; between
ten and fifteen, 1.5 per cent.; between fifteen and twenty-five, 1.4
per cent.; between twenty-five and thirty-five, 0.9 per cent.; between
thirty-five and forty-five, 0.8 per cent., the mortality declining very
greatly after the tenth, after the twenty-fifth, and after the
forty-fifth year of life.

[Footnote 122: _Dictionnaire Encyclopédique_, Troisième serie, t. iv.,
p. 463.]

[Footnote 123: _Barth. Hospital Reports_, vol. xiv., p. 4.]

[Footnote 124: _Guy's Hospital Reports_, xix. p. 327.]

[Footnote 125: _The Liverpool Medico-Chirurgical Journal_, July, 1881,
p. 88.]

[Footnote 126: _Lib. cit._, p. 4.]

The danger of the case is usually proportionate to the youth of the
patient, the degree of the pyrexia, the number of the joints involved,
and the number and the character of the complications, the habits, and
previous health of the patient. A fatal issue is most frequently
observed in connection with hyperpyrexia alone, or in combination with
delirium or coma. A rapid rise of temperature and a temperature over
105°, especially if cerebral disturbance coexist, indicate danger; and
so does arrested perspiration while the temperature is high. In a much
smaller number of cases death is due to some other complication,
especially to purulent pericarditis or to that combined with pleuritis
or pneumonia; in not a few cases the prior existence of chronic
valvular disease, with fibroid induration of liver and kidneys, renders
a fresh rheumatic endo- or pericarditis, occurring as part of acute
articular rheumatism, fatal. There is good if not conclusive evidence
that rather sudden death in acute articular rheumatism is occasionally
due either to diffuse myocarditis or to fatty degeneration of the
muscle of the heart. In Greenhow's 2 deaths out of 50 cases treated by
sodium salicylate the pericardium was universally adherent and the
heart's fibre fatty in one and pale and flabby in the other. Sudden
death in this disease is very rarely due to embolism of the pulmonary
artery or of the cerebral vessels, while ulcerative endocarditis is
very exceptionally one of the sources of a fatal issue.[127] But
although acute articular rheumatism rarely kills {51} directly, it
frequently lays the foundation of subsequent ill-health, and ultimately
proves fatal through organic disease of the heart and its many
consequences. However, it is an interesting circumstance that while
acute rheumatic inflammation is prone to damage the heart permanently,
it very rarely, quite exceptionally, impairs the structure or functions
of the articulations. It is almost solely the subacute form that now
and then becomes chronic or renders a joint for a long time painful,
swollen, and crippled in its movements. Whether acute rheumatism,
however intense per se, ever ends in destructive suppuration and
ulceration of a joint is doubted by some authorities, notwithstanding
the cases published by Fuller and others. No doubt some of the cases
were really pyæmic, or perhaps gonorrhoeal; and it must be borne in
mind that acute articular rheumatism occasionally develops pyæmia, and
then an arthritis might be considered rheumatic when truly pyæmic. The
question of acute rheumatic arthritis exciting a chronic rheumatoid
affection will arise hereafter.

[Footnote 127: See an article on the mortality among rheumatic risks by
A. Huntingdon, M.D., in _N.Y. Medical Record_, 1875, p. 195.]

TREATMENT.--Owing to our imperfect knowledge of the real nature of
acute articular rheumatism, its treatment is still largely either
empirical or intended to combat certain prominent symptoms or
complications of the disease. Of the various methods of treatment which
have been employed space will not permit a description; even of those
advocated by authorities of the present hour only very few will be
considered.

The method which is now unquestionably the favorite one in both Europe
and America, and which in its power of promptly relieving the articular
and muscular pains and reducing the fever of acute rheumatic
polyarthritis may without exaggeration be compared to that exercised by
quinia over the paroxysms of ague, is that in which salicylic acid or
salicylate of sodium is given in repeated and full doses. It was in
July, 1875,[128] that Buss first asserted that salicylic acid was a
specific for rheumatism, and in March, 1876,[129] Maclagan, after
having employed salicine from 1874, published his experience of it as a
valuable remedy in the treatment of acute rheumatism, its beneficial
action being "generally apparent within twenty-four, always within
forty-eight, hours of its administration in sufficient dose." Perhaps a
sufficient time has now elapsed to permit of a just opinion of the
power of these new remedies, the salicyl compounds, over acute
articular rheumatism. The facts presented at the discussion recently
held at the Medical Society of London[130] are sufficiently numerous
and authoritative to justify, at least provisionally, some definite
conclusions as to the remedial relations of the salicylates to acute
articular rheumatism.

[Footnote 128: "Die Antepyr. Wirkung der Salycylsäure," _Centralbl. f.
d. Medic. Wissenschr._, 1875, 276.]

[Footnote 129: _The Lancet_, March 4 and 11, 1876.]

[Footnote 130: _The Lancet_, Dec. 17, 24, 31, 1881; Jan. 7, 14, 28,
1882.]

1. The articular pain and the fever of acute rheumatic polyarthritis
are more or less speedily removed by the salicyl remedies (salicylic
acid, sodium salicylate, and salicine); the pains very frequently
persist after the temperature has become normal. Both symptoms were
removed by five days' use of such agents in 50 per cent., and by eleven
days' use in 80 per cent., of 355 cases treated at Guy's Hospital, and
tabulated by Fagge,[131] and by five days' use in 60 per cent., and by
eleven days' use {52} in 66 per cent., of the 60 severe cases treated
and severely criticised by Greenhow.[132]

[Footnote 131: _Ibid._, ii., 1881, 1031.]

[Footnote 132: _Clinical Society's Transactions_, vol. xiii., 1880. See
Dr. Fagge's table iv., _Lancet_, ii., 1881, 1032.]

Again, in 190 cases of acute and subacute rheumatism the average
duration, under salicyl remedies, of pyrexia was 5.5 days and of joint
disease, 5.3 days (Warner[133]); in 156 cases at St. George's Hospital
the average duration of pyrexia was 3.66 days, of pain 4 days
(Owen[134]); in 82 at the Middlesex the average duration of pyrexia was
5 days, of pain 5.6 days (Coupland[135]); and in 55 at the Westminster
the average duration of pyrexia was 7 days, of pain 7.25
days[136]--that is, a general average duration in the whole series for
the pain and pyrexia of 5.4 days.

[Footnote 133: _Ibid._, p. 1080.]

[Footnote 134: _Ibid._, p. 1081.]

[Footnote 135: _Ibid._, i., 1882, 10.]

[Footnote 136: _Ibid._, ii., 1881, p. 1080.]

Further, 36 per cent. of Fagge's cases and 58 per cent. of Greenhow's
were relieved of both the above symptoms on the fourth day; 24.8 per
cent. of Fagge's and 50 per cent. of Greenhow's on the third day; and
13.5 per cent. of Fagge's and 26.6 per cent. of Greenhow's on the
second day. In Clouston's 27 cases, treated in private, 66.6 per cent.
were free from pain and 59 per cent. from fever within three days, and
85.2 per cent. were devoid of pain and 72.7 per cent. of fever within
four days.[137] Finally, all who have had much experience of this
method of treating acute rheumatism will agree that the first or second
dose frequently relieves the articular pains like a charm, and the
local swelling then frequently subsides in from sixteen to forty-eight
hours.

[Footnote 137: _The Practitioner_, i., 1882.]

2. Relapses are more frequent--probably considerably more
frequent--under treatment by salicylates than under other methods.
Thus, the average of relapses in eight different tables of cases
treated by the salicyl remedies ranged from 16.6 per cent. to 35 per
cent., giving a general average of 26 per cent.;[138] while under other
methods in three different tables the average ranged from 5.4 per cent.
to 27.6 (this last under the full alkaline), giving a general average
of 16 per cent.[139] Relapses appeared to recur less frequently in
those cases which yielded to the salicylates within five days than in
those which took from six to eleven days to yield, in the ratio,
according to Fagge's figures, of 26.6 per cent. for the first, and 29.4
per cent. for the second day; and, according to Hood's, as 18.4 per
cent. to 24.4 per cent. There does not appear to be any regularity in
the order of occurrence or recurrence of relapses, nor is Southey's
definite statement that in "relapsing cases the temperature is nearly
or quite normal on the eighth evening, and a slight relapse occurs on
the thirteenth morning," borne out by the statistics produced at the
London Medical Society. Moreover, W. Carter's cases[140] have not
confirmed Southey's precise statement respecting the gradual remission
of the temperature on the eighth and ninth days of illness in the
continued or non-relapsing, uncomplicated forms. Irregularity and
inconstancy are the typical features of articular rheumatism. The
relapses under the treatment by the salicylates have been referred to
the premature disuse of those remedies, but they do occur
notwithstanding {53} the continued employment of them. It is a general
opinion that exposure to cold, errors in diet, and an early return to
work are frequent causes of relapse; and Broadbent refers the increased
liability to relapse under the salicyl compounds to the rapidity with
which those remedies relieve the acute symptoms of articular
rheumatism, in consequence of which sufficient care is not observed
either by the patients or their nurses, and they are exposed to some of
the above exciting causes of relapse. All the above causes do probably
play their part so long as the materies morbi (if that really exist
either as a chemical principle or as a germ) has not been wholly
eliminated or destroyed. Indeed, the short intervals which frequently
obtain between the primary invasion of the so-called relapses, and the
failure of the salicyl compounds to prevent peri- and endocarditis,
render it probable that what are commonly spoken of as relapses are not
due to a new infection, as in the case of the relapse of typhoid fever,
but to the recrudescences of a disease not yet terminated, but over
some of the manifestations of which--the articular inflammation and the
pyrexia--the salicylates exercise some control.

[Footnote 138: Fagge's, 26.2 per cent.; Greenhow's, 35; Warner's, 33.6;
Owen's, 30.2; Hood's, 18.8; Coupland's, 35.3; Broadbent's, 16.6;
Powell's, 18.7; total, 214 ÷ 8 = 26 per cent.]

[Footnote 139: Hood's, 5.4; Warner's, 14.9; Owen's, 27.6; total, 47.9 ÷
3 = 16 per cent.]

[Footnote 140: _The Liverpool Med.-Chirurgical Journal_, July, 1881, p.
101.]

3. Authorities are generally agreed that the salicyl compounds do not
arrest or control rheumatic inflammation of the endo- or pericardium or
pleura, or subdue the pyrexia, if these complications in well-marked
degree exist; and there is strong evidence to show that they do not at
all constantly prevent the disease from involving those organs, even
after the articular affection has subsided under their use. Inestimable
as is the benefit conferred by these remedies in promptly relieving the
articular pain and fever, they do not secure the great desideratum in
the treatment of acute articular rheumatism--protection of the heart.

In 352 cases treated with salicylate of soda at the Westminster
Hospital, heart disease developed in 13.6 per cent.; in 267 treated
without the salicylate, heart disease developed in 14.2 per cent.
(Warner's cases).[141] In 350 cases treated with salicylates at Guy's,
heart complications obtained in 68 per cent., while in 850 treated
without them, the cardiac complications occurred in 58.8 per cent.
(Hood).[142] Gilbart-Smith collected a large number of cases from
several of the London hospitals, and analyzed them with the following
results: Of 1727 cases of acute rheumatism treated before the
introduction of the salicyl compounds, the proportion of cardiac
complications was 54.4 per cent.; in 1748 cases treated subsequently to
their introduction, the cardiac affections obtained in 63.4 per cent.;
and in 533 cases treated by the salicyl compounds, those affections
obtained in 68.4 per cent.[143]

[Footnote 141: _The Lancet_, ii., 1881, 1080.]

[Footnote 142: _Ibid._, ii., 1881, 1120.]

[Footnote 143: _Ibid._, i., 1882, 136.]

These facts certainly seem to prove that the salicyl compounds do not
prevent the occurrence of the visceral complications or manifestations
of acute articular rheumatism; and if space permitted instances might
be quoted from many authors in which either endo- or pericarditis or
pleuritis or pneumonia or other visceral manifestation had set in after
the patient had been taking the salicylates long enough to have
produced their usual physiological effects; some of these will be
mentioned under the next section.

It may be objected that in the above estimates sufficient attention has
not been paid to the period of the disease at which the treatment by
the {54} salicylates was begun, the time it was continued, the doses
given, the age of the patient, the severity and other characters of the
illness, such as whether acute or subacute, first or second attack,
complicated or not.

4. It must be admitted that there are a few facts which render it very
probable that the salicyl compounds do really reduce the frequency of
these complications, and thus give some protection to the heart in
rheumatism. Of Powell's 32 cases, 19 = 60 per cent. had heart disease
when admitted; and of the remaining 13, 6 = 46 per cent. developed
cardiac disease after admission and while under the salicylates.[144]
Of Dr. Jacobi's[145] 150 cases, 78 = 52 per cent. were admitted with
unsound hearts, and of the other 72, only 5 = 6.9 per cent. developed
cardiac disease after beginning salicylate treatment. Of Southey's 51
cases, 24 = 47 per cent. were admitted with diseased hearts; and of the
remaining 27, only 4 = 14.8 per cent. developed a cardiac affection
subsequent to beginning treatment by the salicylates.[146] Of the
Boston Hospital cases, 38 per cent. were affected with heart disease at
entrance, and only 4.76 per cent. afterward. No heart affection was
developed in any of Clouston's 27 private cases--a result he attributes
to the early period at which the remedies are given in private
practice. But the number is too small to permit of any conclusion being
drawn, and 4 of the cases were examples of recurrence of the disease at
short intervals (three and four weeks) in the same patient, in whom
there appears to have existed no proclivity to cardiac complication,
for he had had four attacks before he came under Clouston's care.
Moreover, his cases were mild, but 16 of them being acute, and of these
only 3 attaining a temperature of 103° and upward. Finally, Herman[147]
estimates the percentage of heart affections that developed after
beginning the salicylates in the London Hospital at 18.7 per cent., and
after other treatment at 30 per cent. Omitting Clouston's, the general
average of the above results is, that in 49.2 per cent. cardiac disease
existed before the patients began the salicyl treatment, and that in
18.2 per cent. it developed after that, while 30 per cent. of cardiac
disease developed after other methods of treatment were begun.

[Footnote 144: _Lancet_, i., 1882, 134.]

[Footnote 145: _St. Thomas's Hospital Reports_, New Series, viii. 252.]

[Footnote 146: _St. Bartholomew's Hospital Reports_, xvi. 10.]

[Footnote 147: Quoted by T. G. Smith, _Lancet_, i., 1882, 137.]

The subject is one beset with difficulties, and still needs
investigation. It is reasonable to infer that as the salicylates
promptly arrest the articular inflammation and allay the fever of
uncomplicated acute rheumarthritis, they will prevent the visceral
inflammations so apt to develop when the disease runs its course
uninfluenced by treatment; but experience has shown that they do not
control or arrest rheumatic inflammation of the heart or pleura or the
attending pyrexia, although capable of subduing the articular
inflammation and the pyrexia that accompanies it. The most eminent
therapeutists are divided on the subject. Maclagan, while admitting
that the salicyl compounds do not ward off cardiac complications, or
cure them when they exist, maintains that their existence is an
additional reason for giving those remedies freely and in large
doses.[148] Broadbent,[149] while believing in the protective influence
of the salicylates "when brought to bear upon the fever in the first
days of its existence," finds in the presence of any cardiac
inflammation a reason for at once discontinuing those remedies.
Flint[150] believes that rheumatic endo- and {55} pericarditis are more
common since the introduction of the salicyl treatment than when the
alkaline method was relied upon almost entirely, and advises[151] the
administration of alkalies with the salicylates to protect the heart.
Vulpian[152] thinks the protective power in question probable, but not
established; while the latest French authority, Homolle, is of opinion
that "cardiac affections are really less frequent in patients treated
by salicylate of sodium than in others."[153]

[Footnote 148: _Lib. cit._, pp. 266, 275.]

[Footnote 149: _Lancet_, i., 1882, 138.]

[Footnote 150: _New York Med. Record_, 1882, 66.]

[Footnote 151: _Pract. Med._, 5th ed., 1098.]

[Footnote 152: _Du Mode d'Action du Salicylate du Soude dans le
Traitement du Rheum. Artic. Aigue_, Paris, 1881, 11.]

[Footnote 153: _Nouveau Dict. de Méd. et de Chir._, xxxi., 1882, 648.]

5. The occurrence of hyperpyrexia is not always prevented by the
salicyl remedies, even when they have produced their full physiological
effects. Fagge endeavors to explain away the two cases of hyperpyrexia
which occurred under Greenhow and the other two which happened amongst
the cases tabulated by himself, and remarks that if the temperature
should begin to fall under the use of salicylic acid, and then should
change its course and rapidly attain a dangerous height, that would
really show that the drug is sometimes incapable of preventing the
occurrence of hyperpyrexia. This actually happened in one of Powell's
two cases,[154] and the patient died suddenly at a temperature of 107°.
In Greenhow's first case the patient had been taking the salicylate for
four days, and was deaf and delirious when the temperature became
105.8°.[155] Finney reports a case in which drachm iss of salicine were
given daily for two days, and drachm ij on the third day, when
pericarditis set in, and on the fourth day hyperpyrexia
supervened.[156] Haviland Hall records an instance in which the
temperature fell from 103.5° to 100.6° after twenty-grain doses of
salicylate soda, every three hours, taken for two days; on the third
day the medicine was given every four hours; the temperature rose in
the evening to 103.4°, and on the next day it rose rapidly to 108.7°,
and the patient became delirious. Patient recovered rapidly after two
baths.[157]

[Footnote 154: _Lancet_, i., 1882, 135.]

[Footnote 155: _Clin. Soc. Trans._, xiii. 264.]

[Footnote 156: _Brit. Med. Journ._, ii., 1881, 932.]

[Footnote 157: _Lancet_, ii., 1881, 1082. See also two cases in _Med.
Times and Gaz._, ii., 1876, 383.]

Pericarditis is not always present when hyperpyrexia arises during the
administration of salicylic acid; it was absent in Powell's cases, is
not mentioned in Hall's, and did not ensue in one of Greenhow's until
two days after the temperature had reached 105.4° F. However, either
pericarditis or pneumonia is very frequently present when the
temperature is excessive. It is generally admitted that the salicylates
do not control rheumatic hyperpyrexia once it exists.

6. Notwithstanding the prompt removal of the pain and reduction of the
fever by the salicyl compounds, the average duration of acute articular
rheumatism is not very considerably lessened by those remedies. Thus,
of Hood's[158] 350 cases treated by salicylates the average duration of
the illness was 35.95 days as against 38.75 under other methods. The
average time spent in bed by Warner's 342 cases was 19.5 days under the
salicylates, and by 352 patients under other remedies 23.5 days. Both
estimates show a curtailment of the duration of the disease by the new
treatment of three to four days only; which is not a very material
improvement.

[Footnote 158: Calculation from Dr. Hood's Tables 1 and 1_a_, _Lancet_,
ii., 1881, 1119.]

{56} 7. Nor do the salicylates materially alter the time spent in
hospital by rheumatic patients; some evidence indicates that they
actually prolong that period. The following are the average residences
in hospital under the salicylates, according to several recent authors,
and they are remarkably uniform with two exceptions: Coupland, 36 days;
Warner, 34.9; Hall, 34; Southey, 32.5; Broadbent, 31.2; Powell, 31;
Finlay and Lucas, 29.7;[159] Owen, 23; Brown, 21.9;[160] or a general
average of 30.4 days for the salicyl remedies. Under full alkaline
treatment: Owen, 26 days; Dickinson, 25;[161] Fuller, 22.2;[162]
Blakes, 24;[163] or a general average of 24.3 days for full alkaline
treatment. And if to these we add Finlay and Lucas's results, 27.7
days, under but two to three drachms of alkaline salts in the
twenty-four hours--a quantity only the fourth of that given under the
full alkaline method--the general average residence in hospital under
alkaline treatment was but 25.4 days; that is, five less than under the
salicylate.

[Footnote 159: _Lancet_, ii., 1879, 420.]

[Footnote 160: _Boston Med. and Surg. Journ._, Feb., 1877. The four
cases excluded by the reports are included in this calculation, that it
may more fairly be compared with other reports.]

[Footnote 161: _Lancet_, i., 1869.]

[Footnote 162: _The Practitioner_, i., 1869, p. 137.]

[Footnote 163: _Boston City Hospital Reports_, 1st Series.]

These several estimates of the time spent in hospital under the
salicylates, with the exception of Owen's and Brown's, correspond
closely with that of the time spent by Gull's and Sutton's patients
under mint-water--32.8 days--although the general average of them falls
short of the latter by 2.4 days.

The following table (iii.) of Hood's[164] shows that under the
salicylate method 45.7 per cent. remained in hospital beyond forty
days, and 39 per cent. under other methods, and that about 50 per cent.
more were discharged within twenty days under the other methods than
under the salicylate:

  350 cases treated with salicylates:
                        Days.
  Under 10.   Under 20.      Under 30.     Under 40.     Ill longer.
  3 = 0.84%.  31 = 8.88%.    76 = 21.7%.   84 = 24%.     160 = 45.7%.

  850 without salicylates:
                        Days.
  Under 10.   Under 20.      Under 30.     Under 40.     Ill longer.
  12 = 1.4%.  105 = 12.35%.  175 = 20.1%.  182 = 21.4%.  331 = 39%.

[Footnote 164: _The Lancet_, ii., 1881, 1120.]

These statistics favor Greenhow's opinion that patients treated with
salicylate of sodium regain their strength slowly, and are long in
becoming able to resume their ordinary occupations. Some allowance,
however, must be made for the precautions against relapse under
salicylates observed in hospitals since the great tendency thereto has
been recognized.

8. Certain unpleasant or toxic effects are produced by salicylic acid
and salicylate of sodium; such are nausea, vomiting, abdominal pain,
frontal headache, tinnitus, incomplete deafness, vertigo, tremor,
quickened respiration, very rarely amblyopia and even temporary
amaurosis, and not unfrequently delirium. A feeling of prostration and
general misery is not uncommon. These phenomena of salicylism are in
great measure proportionate to the dose employed, but they have
followed moderate {57} doses, owing sometimes to idiosyncrasy, and
perhaps frequently to retarded elimination consequent upon previous
disease of the kidneys or disturbance of their function by the
salicylic acid or its salt. Those agents are usually completely
excreted in forty-eight hours, but in one of Powell's[165] cases
elimination was not completed before the fifth day, and not before the
eighth in Byanow's case.[166] Possibly uræmia may in some cases cause
the delirium.[167] The delirium, which may be violent or not, is often
preceded by dryness of the tongue, restlessness, and rapid breathing.
Impurities in the acid may account for the inconstancy with which
delirium has been noticed by different observers. While but 2 instances
in 82 cases were met with by Coupland, 3 out of 90 cases by Broadbent,
and 3 out of 109 by Brown,[168] Charles Barrows[169] encountered 8
instances in 28 cases. In one of these a boy of eleven became delirious
in eighteen hours, having taken 10 grs. of salicylate of sodium every
three hours. In another instance the drug had been in full use for five
days before the delirium manifested itself. These phenomena of
salicylism rapidly disappear when the medicine is stopped, and delirium
has not always recurred on its resumption. They are less frequent in
children, in whom elimination by the kidneys takes place very rapidly
and a marked tolerance of salicyl compounds exists. Occasionally more
serious effects appear to be produced by the salicylates, owing to
their direct action on the heart, impairing its power, as evidenced by
feeble impulse and sounds, increased frequency of the pulse, and
diminution of the arterial pressure.[170] But, notwithstanding the very
large number of cases of acute rheumatism that have been treated by the
salicyl compounds, very few clear instances of their toxic action on
the heart have been recorded, and even in some of these there were
other conditions present that may have played some part, perhaps a
chief part, in the production of cardiac failure. In Greenhow's
case[171] the autopsy revealed a dilated fatty heart and slightly
granular kidneys, and the cardiac failure coincided with a fall of
temperature to 97° F. Goodhardt's[172] patient died in nine hours after
beginning the salicylic acid, of which she took but one drachm, in
divided doses, every three hours. The pulse rose rapidly to 160; she
was restless and moaning, but died quietly and suddenly. Recent
pericarditis, with one or two points of fatty degeneration of the
heart's substance, and sound kidneys were found. The reporter of the
case inclines to the opinion that the acid produced sudden collapse and
cardiac failure, while Bristowe referred them to the rheumatic poison
itself. I have not been able to refer to Hoppe Seyler's paper,[173] in
which he relates that having given 5 grammes of salicylic acid to a
child of seven and a half years affected with articular rheumatism,
shortly afterward there occurred deafness, agitation, profuse sweating,
dyspnoea, and finally fatal collapse. The condition of the heart and
kidneys before and after death is not given. Weber {58} published[174]
an instance in which 15-gr. doses of salicin given to a woman of
twenty-seven produced in thirty-four hours a rapid fall of temperature
from 103° to 96° F., accompanied by delirium and serious but not fatal
collapse. It is well to remember that a similar failure of cardiac
power is occasionally observed in other fevers when rapid defervescence
occurs, although the salicyl compounds have not been taken; and it is
certainly necessary to give these remedies cautiously, and often to
administer alcohol with them, when the heart's action is at all
enfeebled by protracted pyrexia and pain, or by disease (inflammatory
or degenerative) of its substance or envelope. Indeed, if severe
cardiac inflammation obtain in rheumatism, the remedies are powerless
and perhaps unsafe. The sudden reduction of the temperature when much
exhaustion obtains, even in the hyperpyrexia of rheumatic and other
fevers, whether by salicylic acid or quinia or the cold bath, may be
attended with fatal collapse of the heart.

[Footnote 165: _Lancet_, i., 1882, 135.]

[Footnote 166: Quoted by Wood in his _Therapeutics and Mat. Med._,
1880, from _Centralb. für Chir._, 1877, 809.]

[Footnote 167: See DaCosta's observations in _Am. Med. Journal_, vol.
lxix., and Ackland's in _B. Med. Journal_, i., 1881, 337.]

[Footnote 168: _Boston Med. and Surg. Journal_.]

[Footnote 169: _N.Y. Med. Record_, April 29, 1882, 456.]

[Footnote 170: Köhler, _Centralb. f. Med. Wissensch._, 1876, and
Dunowsky, _Arbeiter Pharm. Labor._, Moskau, i. p. 190, quoted by H. C.
Wood, _Therapeutics, Mat. Med., etc._, 3d ed., p. 639.]

[Footnote 171: _Clin. Soc. Trans._, xiii. p. 266, c. iii.]

[Footnote 172: _Ibid._, p. 123.]

[Footnote 173: Quoted by D. Seille, Thèse, _De la Méd. Salicylée dans
le Rheumatism_, Paris, 1879, p. 54.]

[Footnote 174: _Clin. Soc. Trans._, x. p. 70, 1877.]

Instead of the frequent weak pulse above mentioned, I have many times
found salicylate of sodium render the pulse very slow, labored, and
compressible in typhoid fever, and generally at the same time the
temperature has been considerably reduced below what it had been.

A temporary albuminuria is not infrequent; excluding mere traces, it
obtained in 52 per cent. of cases treated by the salicylates alone or
in conjunction with full doses of alkali, and in but 25 per cent. of
those in which full doses of alkali, with or without quinia, were
employed.[175]

[Footnote 175: Isambard Owen, _Lancet_, ii., 1881, p. 1081.]

Very rarely hæmaturia and even nephritis have occurred. The active
principle is chiefly eliminated by the kidneys, which may account for a
local irritating influence upon those organs.

Salicine is much preferred by Maclagan to salicylic acid and to
salicylate of sodium, on the grounds that it is a bitter tonic and
produces less debility and more rapid convalescence than those agents,
and that it never produces delirium nor depresses the heart's action.
Ringer[176] and Charteris[177] state that they have never seen
salicine, even in large doses, cause delirium; and Prof. Gairdner has
not found it produce any unfavorable symptoms.[178] On the other hand,
Greenhow[179] found that marked depression of the heart's power ensued
in 4 out of 10 cases whilst the patients were taking salicine, and
entirely subsided after it was discontinued. Further careful and
extended observation is needed before the relative value of salicine
and salicylate of sodium can be reliably stated. It is probable that
the salt is more active and prompt than the bitter principle; and this,
with the greater cheapness of the former, may perhaps account for the
more general employment in hospitals of the salicylate than of
salicine. The latter, moreover, is often tolerated when the former is
not.

[Footnote 176: _Handbook Therapeutics_, 8th ed., 1880, 587.]

[Footnote 177: _Brit. Med. Jour._, i., 1881, 229.]

[Footnote 178: _Lancet_, i., 1882, in table giving experience of
British hospitals, prepared by Maclagan.]

[Footnote 179: _Trans. Path. Soc._, xiii. 262.]

As regards the doses of these agents required in acute rheumatic
arthritis, practitioners are not agreed; Maclagan, Stricker, Fagge,
Broadbent, Ringer, Flint, Sée, recommend large doses at short intervals
at the outset, with the view of getting the patient rapidly under the
influence of the drug. Maclagan gives salicine scruple i-ij at first
hourly, then every two hours {59} as the acute symptoms begin to
decline; after the second day he allows 20 to 30 grs. every four hours
for two or three days; "and for a week or ten days more that quantity
should be taken three times a day." Stricker, Fagge, Broadbent, and Sée
recommend about 20 to 30 grs. of salicylate of sodium every hour or two
for six doses (= drachm ij-iij in the day), and Ringer would employ 10
grs. hourly, and if in twenty-four hours this dose has not either
modified the disease or produced its characteristic symptoms, he would
increase it to 15 and then to 20 grains hourly. On the other hand,
Owen's[180] results show practically no difference in the duration of
pain and pyrexia and in the average duration of illness from the
commencement, whether drachm iij or drachm ij or drachm iss were given
every twenty-four hours; and C. G. Young[181] found that 10 to 15 grs.
every one, two, or three hours are sufficient.

[Footnote 180: _Lancet_, ii., 1881.]

[Footnote 181: _Dub. Journ. Med. Sci._, Sept., 1880, 193.]

Indeed, exceptionally good and exceptionally indifferent results are
reported under similar doses. No such good results are reported as
those of the Boston City Hospital under doses of drachm ij to drachm iv
per diem, the average residence in hospital being only eighteen days if
four cases which became chronic are excluded, or 21.9 days if they are
included.

The plan in vogue at our hospital here and in my own private practice
is to give about 15 grains every two or three hours, according to the
severity of the case and until the articular pain and pyrexia are
relieved. After the pain and pyrexia have yielded, the remedy should be
continued in smaller doses, say 10 to 15 grs., three or four times a
day, according to the severity of the case, for eight to ten days
longer, to prevent relapse, and during this period exposure, exercise,
and dietetic excesses must be carefully guarded against.

The salicine may be given dissolved in milk or enclosed in wafers; the
salicylate of soda, in a solution of any aromatic water, to which
extract of liquorice or syrup of lemon and a few drops of spirits of
chloroform may be added. The French add a little rum to flavor the
mixture. Should severe cardiac inflammation exist, and, even although
not severe, should there exist signs of failure of cardiac power,
salicylates and salicine had better be avoided. If the secretion of
urine diminish considerably under their use, or hæmaturia supervene, or
organic disease of the kidneys exist, they must be employed cautiously,
and may require prompt suspension. If marked debility exist,
stimulants, especially the alcoholic, should be combined with them.

The oil of wintergreen has recently been well spoken of by F. P.
Kinnicutt of St. Luke's Hospital, New York,[182] as a substitute for
salicylate sodium. It is itself a methyl salicylate 90 per cent., plus
terebene 10 per cent. Its officinal name is oleum gaultheria, and it is
given in doses of minim x-xv every two hours except during sleep, and
in severe cases of articular rheumatism during the twenty-four hours,
either by floating the oil upon a wineglass of water or milk or in
capsules or upon lumps of white sugar. It resembles in its influence
upon acute rheumatism very closely the sodium salicylate, for which it
may perhaps be substituted, and Kinnicutt maintains that it is quite as
effectual, pleasanter to take, and free from the intoxicating
properties of the salt and the salicylic acid. It requires to be
continued during convalescence just like the salicylate.

[Footnote 182: _Med. Record of New York_, Nov., 1882, 505.]

{60} The alkalies--in this country at least--were the favorite remedies
in the treatment of acute articular rheumatism before the powers of
salicine and salicylic acid became generally known, and there are still
authorities who maintain their excellence, if not their superiority
over the salicylates, in protecting the heart against the recurrence of
rheumatic inflammation (Flint, Dickinson, Sinclair, Stillé).

Under the term the alkaline treatment unfortunately are included two
distinct methods of administering the salts composed of potash and soda
and the vegetable acids, carbonic, tartaric, citric, etc.--viz.: that
in which about half a drachm of one of these salts is given three or
four times a day; and the other known as Fuller's method, in which
large doses are prescribed, so that from an ounce to an ounce and a
half is given in the first twenty-four hours, with the view of rapidly
rendering the urine alkaline, and if possible the perspiration also;
for I have frequently produced the former effect in less than twelve
hours, yet have found the perspiration still redden litmus on the
second, and even the third, day and later. A disregard of the essential
differences existing between these two methods of employing alkalies in
acute rheumatism may partially account for the differences of opinion
existing as to the value of the alkaline treatment, and for the
differences in the statistical results thereof published by various
observers--a remark applicable to other methods and statistics also.
Fuller commonly ordered every three or four hours bicarb. sodium drachm
iss and acetate of potassium drachm ss dissolved in ounce iij of water
and rendered effervescing at the moment of administration by the
addition of an ounce of lemon-juice or drachm ss of citric acid. As
soon as the urine presents an alkaline reaction--which is usually the
case in twelve to twenty-four hours--the quantity of the alkali is
reduced by one-half, or to about 8 drachms, during the succeeding
twenty-four hours, and provided the urine continues alkaline to 3
drachms on the third day. On the fourth day and subsequently only a
scruple to half a drachm of alkali is given three times a day,
sufficient to keep the urine alkaline, and to each dose are added 3
grains of quinia dissolved in lemon-juice; and this combination is
continued till convalescence sets in. An aperient pill is given
whenever needed, but is administered "only under conditions of extreme
nervous irritation." The method is not an exclusively alkaline one.

Space will not allow of a lengthened analysis of the statistics that
have been published on this subject, and I will give only some of the
more important statistical results. While, as we have seen, the average
duration of pyrexia and articular pain under salicylate treatment is
about 5.4 days, under moderate alkaline treatment, according to the
recent statistics of Finlay and Lucas,[183] the average duration of
pyrexia was 10.3 days and of articular pain 12.2 days, and of Owen[184]
6.5 days for the first and 8 days for the second, or a general average
for the pain and pyrexia together of 9.25 days, or about 3.85 days
longer than under the salicylate treatment. Nor can it be said even of
the full alkaline plan that the first or second dose frequently
relieves the articular pains like a charm. On the other hand, it has
been already shown that the average time spent in hospital was five
days less under the full alkaline than under the salicylate treatment.

[Footnote 183: _Lancet_, ii. 1879, 420.]

[Footnote 184: _Ibid._, ii., 1881, 1081.]

As regards the relative power of the salicylates and of full alkaline
{61} treatment in protecting the heart, the following analysis and
calculation deserve attention. The percentage of cases in which cardiac
disease set in after the salicylate treatment began was, according to
Powell, 18.75; according to Haviland Hall, 37.1; according to Finlay
and Lucas, 11.60; Southey, 8; Brown, 4.76; Jacobi, 3.35, or a general
average of 14 per cent.; whereas cardiac disease developed after the
alkaline treatment had commenced in 13.6 per centum according to
Blake;[185] in 10.7 per cent. according to Dickinson;[186] in 7 per
cent. according to Owen; in 6.6 per cent. according to Finlay and
Lucas; and in 2 per cent. according to Fuller; making a general average
of only 7.8 per cent.

[Footnote 185: _Med. and Surg. Reports of Boston City Hospital_, 1st
Series, 1870.]

[Footnote 186: This percentage is obtained by adding together all the
cases treated by alkalies given by Dickinson in his IX., X., XI., and
XII. tables. Their total was 65 cases in which the heart was affected
seven times. In table IX. from drachm ii-iv of alkaline salts were
given daily, and in table X. about drachm iij daily.--_Lancet_, i.,
1869.]

Judging from these statistics, it is not improbable that a combination
of sodium salicylate, with full doses of bicarbonate of sodium or
chlorate of potassium, will give better results in the treatment of
acute rheumatism than either of those classes of remedies singly.
Indeed, Flint and others have advised such combinations, and Bedford
Fenwick has recently stated, as a result of his experience in 30 cases,
that if, after giving a free purge, followed by scruple doses of sodium
salicylate hourly for six hours, that salt be stopped, and in twelve
hours afterward half-drachm doses of citrate of potassium be
administered every four or six hours until the saliva becomes alkaline,
relapses will be extremely rare, and that this is the safest and most
successful method of treating acute and subacute articular
rheumatism.[187]

[Footnote 187: _Lancet_, i., 1882.]

Having spoken somewhat fully upon the remedies of which I have most
personal experience, and which have the largest number of advocates at
the present time, and having advised the combination of these remedies,
I shall only glance at some of the other remedies or methods of
treating the disease still more or less employed.

Quinia, given in divided doses to the extent of 15 to 30 grains in the
day, is still highly thought of in France in the early stages, during
the course of and on the occurrence of relapses, in acute (especially
febrile poly-) articular rheumatism. It is claimed by Briquet,
Monneret,[188] Legroux, and others that although not a specific for the
disease it moderates the general disturbance, diminishes the local
affections, and even retards the development or lessens the gravity of
the cerebral symptoms--that, although it does not control the cardiac
inflammations, it is not contraindicated by them. The only recent
English authority who has strongly advocated full doses of quinia in
this disease is Garrod,[189] but he mixed the drug, in five-grain
doses, with half a drachm of bicarbonate of potassium, a little
mucilage, and spirits of chloroform, and gave it every four hours until
the fever and articular affection had completely abated. Sufficient
facts have not been published to permit of the formation of a reliable
judgment as to the actual or the comparative value of either the simple
quinia or the quino-alkaline treatment of acute and subacute articular
rheumatism. There can be no doubt as to the value of quinia to meet
certain conditions incident to the disease, such as debility, lingering
{62} convalescence, periodical relapse, excessive perspiration, failure
of appetite, and perhaps, in some instances, high temperature. Barclay
has found quinia of much service when depression has followed the long
continuance of the alkaline treatment and is attended with alkaline
urine and a deposit of the earthy phosphates.[190] It may be given by
the rectum if not tolerated by the stomach or if the alkalines are
being taken.

[Footnote 188: _La Goutte et le Rheumatisme_, Paris, 1857.]

[Footnote 189: Reynolds's _Syst. Med._, 1870, p. 951.]

[Footnote 190: _St. George's Hospital Reports_, vol. vi. p. 111 _et
seq._]

Greenhow[191] has treated 43 cases with iodide of potassium and
quinine, and says that his experience of this method contrasts
favorably with that of salicine and salicylate of soda. However,
pneumonia supervened in 3 cases while under treatment; cardiac
inflammation arose in 6 cases (= 14 per cent.) after admission; single
relapses of short duration occurred in 21 per cent.; and, excluding two
cases in which the treatment was soon discontinued and 7 very mild
cases, the remaining 34 cases were on the average each thirty-six days
in hospital. Under this method relapses were less frequent (21 per
cent. instead of 26 per cent.), and stay in hospital longer (36 instead
of 30.4 days), than under that by the salicylates; but the number of
cases treated is too small to base a final opinion upon. He prescribed
5 grains each of iodide of potassium and carbonate of ammonia three or
four times a day, and 2 grains of quinia with three of extract of
hyoscyamus in pill as often. This method, in principle at least,
resembles that recommended by DaCosta, who administers in uncomplicated
cases bromide of ammonium in 15- to 20-grain doses every three hours,
and as soon as the acute symptoms have disappeared follows it by quinia
in fair doses. It has not come into general use in this country,
although its eminent proposer published his cases in 1869.[192]

[Footnote 191: _The Lancet_, i., 1882, 913.]

[Footnote 192: _Pennsylvania Hospital Reports_, vol. ii., 1869; _New
York Medical Record_, September, 1874, p. 481.]

Notwithstanding the encomiums passed upon propylamine--or, more
correctly, trimethylamine--as a remedy for acute and chronic rheumatism
by Awenarius of St. Petersburg in 1856, by Gaston of Indiana in 1872,
by Dujardin-Beaumetz in 1873, and Peltier in 1874 (both of France), and
Spencer of England in 1875, it has not been much employed, especially
since the salicylates have attracted attention. It appears that in a
considerable proportion of cases the articular pains have subsided in
two or three days under its employment, and then the temperature has
declined, but the visceral complications have not been prevented. From
4 to 8 minims of trimethylamine in an ounce of peppermint-water, with a
drachm of syrup of ginger, may be given every hour or two, the
intervals to be increased as the pains diminish. When pain has quite
ceased the drug may be stopped and quinia given its place. It merits
further study in this disease,[193] and Dr. Shapter of the Exeter
Hospital has very recently stated that he is so convinced or the value
of propylamine that salicylic acid has not fully commended itself[194]
to him. Senator has recently recommended benzoic acid or its sodium
salt in large doses (about ounce ss in the day) in those cases of acute
rheumatic arthritis in which {63} the salicylates have failed, although
he admits that it scarcely rivals them.[195] His 22 patients were
relieved in 4.4 days as the average, and no complications occurred in
any of them. Benzoic acid is said not to produce the nausea,
depression, or unpleasant head phenomena of salicylic acid, to which it
is closely related in chemical composition.

[Footnote 193: On this subject see Farier-Lagrange's _Essai sur la
Trimethylamine_, Strasbourg, 1870; _Journal de Méd. et de Chirurgie_,
1873, No. 2; _Medico-Chir. Rev._, i., 1873, 497; _Lancet_, ii., 1875,
675; _The Practitioner_, London, i., 1875; _Le Progrès Médicale_, Jan.
10, 1874; _ibid._, Aug. 9, 1879.]

[Footnote 194: _The Brit. Med. Jour._, 1881, p. 1012. See also Tyson,
_Philadelphia Med. Times_, 1879, vol. x. 359.]

[Footnote 195: _Centralb. f. d. Med. Wiss._, 1st May, 1880, quoted in
_Practitioner_, Sept., 1880. See also McEwan's experience, _Brit. Med.
Journ._, i., 1881, 336; F. A. Flint, M.D., _N.Y. Med. Gazette_, 1880.]

Space will not permit of any notice of lemon-juice, perchloride of
iron, the mineral acids, or the blistering treatment. Of this last my
experience enables me to say that it frequently relieves the pains
promptly, but does not at all always protect the heart. In my opinion
it deserves an extended employment in conjunction with early and full
doses of the sodium salicylate. As Andrews has not by any communication
made since the publication of his paper in 1874[196] maintained the
value of the treatment of the disease by an exclusively non-nitrogenous
diet of arrowroot, and as he had then treated but eight cases in that
way, it is hardly necessary to consider it as a method of treatment.

[Footnote 196: _St. Barth. Hospital Reports_, vol. x. 359.]

Having spoken of the treatment of the general disease acute articular
rheumatism, it remains to speak of the treatment of its visceral
manifestations and of some of its more important incidental symptoms
and complications. As the treatment of the various forms of cardiac
inflammation will be given in extenso in the articles specially devoted
to those topics, I will be very brief in my notice of them.

In every case of rheumatic fever it is our primary duty to employ those
measures as early and deftly as possible which in the present state of
knowledge appear to promptly relieve the pyrexia and articular
symptoms, and lessen the tendency to, but do not altogether prevent,
the visceral complications. Such measures have been already said to be
the administration of the salicylates and alkaline salts together in
full doses, and the observance of certain dietetic and hygienic details
to be given hereafter. If, notwithstanding, peri- or endocarditis, or
both, supervene, as it frequently happens, what is to be done? I reply
that even in pericarditis active interference is seldom necessary; the
general treatment previously employed may be continued in the hope that
it may mitigate the cardiac inflammation by reducing the pyrexia and
subduing the polyarthritis, even although it be incapable of directly
controlling the pericardial inflammation. If the pain in pericarditis
be really severe and the heart's action much disturbed, a dozen leeches
may be applied over the heart, and be followed by anodyne fomentations
or hot poultices applied, as Lauder Brunton advised, over several
layers of flannel interposed between the skin and them. Leeching,
however, is seldom needed, a hypodermic injection of morphia generally
sufficing to relieve the pain. Should these measures not relieve the
pain and allay the cardiac excitement, small and repeated doses of
chloral, which Balfour observes "is not more useful as a sedative than
as an antiphlogistic," may be given. If there be, as so frequently
happens, but little pain or cardiac disturbance, there being only a
friction sound revealing the inflammation, the hot poultices or anodyne
fomentations, or even covering the front of the chest with wadding or a
belladonna plaster, which I prefer, will suffice. Should pericardial
effusion ensue, the diet must be improved, and if much {64} debility
exists, the salicylate and alkalies should be stopped, and wine may be
given along with quinine alone or with pretty full doses of muriate of
iron. As the strength returns absorption commonly takes place; but if
it is delayed, either the iodide of potassium or the infusion of
digitalis may be employed along with the quinia; or, if no special
contraindication exist, a pill containing a grain each of blue mass,
digitalis, squill, and quinia may be given three times a day and its
effects carefully watched. Much difference of opinion obtains as to the
value of flying blisters on the præcordia. Although not often required,
they appear to be more useful than iodine applications. In those
comparatively rare instances in which the effusion is abundant and
remains unabsorbed, either because it is largely sero-purulent or
purulent, it is proper to aspirate the pericardial sac, which should
certainly be done if marked signs of cardiac oppression and failure
coexist. Having once hesitated to aspirate in recent rheumatic
pericarditis with copious effusion in a lad, and found a large amount
of pus in the sac after death, I would warn against hesitancy under
such circumstances. Careful employment of the instrument can hardly do
harm if even no large amount of effusion exist.

Active treatment is quite uncalled for, as a rule, in acute rheumatic
endocarditis unattended by pericarditis. If the valvulitis occur
notwithstanding the employment of the anti-rheumatic remedies, it is
very doubtful if we have any others capable of directly controlling
that inflammation. Inasmuch, however, as, owing to the inflamed surface
being in constant contact with the fluid, many of our remedies may be
applied directly to the diseased part, it is well neither to be
dogmatic on the point nor to abandon hope that agents may yet be found
that will prove directly useful. While carefully treating the rheumatic
fever, the main indications remaining to be filled appear to be to
quiet the cardiac excitement and secure as much rest to the inflamed
valves as possible. The alkaline salts, salicine, and the salicylate of
sodium do usually greatly reduce the frequency of the heart, and, pro
tanto, secure rest. The tincture of aconite given hourly, so as to
slacken the heart's speed, is useful in the sthenic stage of endo- and
of pericarditis; and the benefit of absolute rest of the body in bed
and of the joints in splints during the entire course of rheumatic
fever, in preventing cardiac inflammations and in treating them, has
been shown by Sibson.[197] When signs and symptoms of cardiac weakness
arise, whether from the pressure of pericardial effusion or from
myocarditis or any other cause, the employment of salicylates,
alkalies, aconite, and chloral should be at once stopped and alcoholic
stimulants and tonics (strychnia, quinia, iron) and good food should be
freely administered. The most valuable point made of late in the
therapeutics of acute inflammations of the valves is Fothergill's
development of Sibson's principle--viz. that "general quietude for
weeks after an attack of acute endocarditis is indicated," as the
cell-growth in the valve may not be quite over in a less time,[198] and
the work of repair, we may add, not completed. The same principle is
specially applicable in myocarditis.

[Footnote 197: Reynolds's _System of Med._, vol. iv. p. 527, Eng. ed.]

[Footnote 198: _Diseases of Heart, with their Treatment_, 2d Series,
1879, 149.]

The disturbances of the nervous system were divided into those {65}
dependent upon gross organic alterations of the nervous centres and
their envelopes, and those not so related, but which we commonly speak
of as functional. Were it possible generally--which it is not--to
diagnosticate rheumatic meningitis from the merely functional form of
so-called cerebral rheumatism, then its treatment would resolve itself
into a vigorous use of the anti-rheumatic remedies, salicylates,
alkalies, etc., and the active employment of ice and leeches to the
scalp, purgatives, full doses of the iodide and bromide of potassium,
ergot, etc. If, together with the symptoms of that often obscure and
comparatively rare complication of rheumatic fever, ulcerative
endocarditis, there occurred severe headache, delirium, or paralysis,
we might find great difficulty in determining the cause of the cerebral
disturbance, and would naturally vary our measures according as we
suspected meningitis, embolism, or simple functional disturbance, and
the treatment adapted to these several conditions will be found under
their respective heads in this work.

Coming now to the functional disturbances of the nervous centres, which
are the ordinary forms met with in acute articular rheumatism, they may
be divided, for therapeutical reasons, into two groups: (1) Those
unattended by hyperpyrexia, and (2) those preceded, accompanied, or
followed by hyperpyrexia.

(1) When any sign of disturbance of the nervous system, delirium,
restlessness, taciturnity or talkativeness, insomnia or somnolence,
deafness, tremulousness, vacancy, stupor, or what not, occurs in
rheumatism with but a moderate temperature, 101° to 103°, while we
anxiously watch the temperature from hour to hour, prepared to combat
any tendency to hyperthermia the moment it is discovered, we endeavor
to control the cerebral disturbance as in other febrile affections, but
with greater diligence, knowing that in this disease these nervous
symptoms very often precede hyperpyrexia. We persist with the
salicylates to reduce the rheumatic element of the affection, employ
remedies to control the cardiac or pulmonary inflammations which are so
frequent in such circumstances, sustain the general powers by food,
wine, and quinia, if, as frequently happens, there are evidences of
failing strength, and meet any other special indication that may arise.
For example, we procure sleep and allay motor and mental excitement by
opium or chloral and by evaporating lotions or the ice-cap to the head.
We reduce temperature, allay restlessness, preserve the strength, and
promote sleep by lightening the bed-clothes, drying frequently the
entire surface of the body if it is perspiring freely, or by sponging
it with tepid water hourly if dry and hot. We act on the kidneys,
bowels, and if necessary the skin, if from the scantiness of the urine
or other evidence we suspect uræmia. Should these means fail and the
delirium and other symptoms which occur in cerebral rheumatism
continue, and especially should they be severe, it would be, in the
writer's opinion, proper to employ the methods that are now resorted to
when hyperpyrexia accompanies those symptoms; for patients suffering
from cerebro-spinal disturbance or rheumatic fever, although unattended
by hyperthermia, do die if those symptoms continue. Moreover, the
hyperthermia may at any moment supervene; it is itself perhaps as much
a nervous disturbance as delirium, and apt to succeed the latter. It
was in these very cases in which the delirium preceded the hyperpyrexia
that the London committee to be presently mentioned found the highest
{66} mortality. If along with these nervous symptoms the articular pain
or the sweating disappear suddenly, or if the pulse suddenly increase
in frequency without demonstrable increase of cardiac mischief, there
is reason to anticipate the supervention of hyperpyrexia.

(2) When the cerebro-spinal disturbance of rheumatic fever is followed,
preceded, or accompanied by hyperpyrexia, there is one indication for
treatment which dominates all others, and that is the prompt reduction
of the hyperthermia. The terrible danger of this condition in rheumatic
fever is known to all persons who have had much experience of the
disease. Wilson Fox in 1871 had not known a case recover after a
temperature of 106° unless under the use of cold, yet that is not an
alarming temperature in intermittent or relapsing fever, and is often
recovered from in typhoid fever. Thanks to Wilson Fox,[199]
Meding,[200] H. Thompson,[201] H. Weber,[202] I. Andrew,[203] Maurice
Raynaud,[204] Black,[205] Fereol,[206] and many others since, it has
been established that when the hyperthermia is removed by external cold
the nervous disturbances also usually at once disappear or lessen very
much. And thus we are brought to the treatment of the hyperpyrexia of
acute articular rheumatism. On this important topic it will be most
satisfactory and convincing to give some of the conclusions arrived at
respecting hyperpyrexia in acute rheumatism by a committee of the
Clinical Society of London.[207] I will condense some of them.

[Footnote 199: _Treatment of Hyperpyrexia_, 1871, and _Lancet_, ii.,
1871.]

[Footnote 200: _Archiv für Heilkunde_, 1870, xi. 467.]

[Footnote 201: _Brit. Med. Jour._, ii., 1872; _Lancet_, ii., 1872; and
_Clinical Lectures_, 1880.]

[Footnote 202: _Clin. Soc. Transactions_, v. 136.]

[Footnote 203: _St. Bartholomew's Hosp. Repts._, x. 337.]

[Footnote 204: _Journal de Thérap._, No. 22, 1874.]

[Footnote 205: _Gaz. Hebdomad. de Méd. Sci._, 1875.]

[Footnote 206: _Soc. Méd. des Hôpitaux_, 8 Juin, 1877.]

[Footnote 207: _Brit. Med. Jour._, i. 82, 807.]

1. "Cases of hyperpyrexia in acute rheumatism prevail at certain
periods;" "such excess corresponds in a certain degree, but not in
actual proportion, to a similar excessive prevalence of acute
rheumatism generally. The largest number of cases of hyperpyrexia arise
in the spring and summer months, whereas rheumatism is relatively more
common in the autumn and winter." 2. "Whilst very little difference
obtains between the two sexes in regard to proclivity to rheumatism,
the proportion of males to females exhibiting hyperpyrexial
manifestations is 1.8 to 1." (3 omitted.) 4. "The cases of hyperpyrexia
preponderate in first attacks of rheumatic fever." 5. "Hyperpyrexia is
not necessarily accompanied by any visceral complications, but may
itself be fatal. The complications with which it is most frequently
associated are pericarditis and pneumonia." 6. "The mortality of these
cases is very considerable, hyperpyrexia being one of the chief causes
of death in acute rheumatism." 7. "Although present in a certain number
of cases, and these of much value from their prodromal significance,
neither the abrupt disappearance of articular affection, nor the
similarly abrupt cessation of sweating, is an invariable antecedent of
the hyperpyrexial outburst." (8, 9, 10 omitted.) 11. "The post-mortem
examinations in a certain proportion elicited no distinct visceral
lesions, and when present the lesions were not necessarily extensive."
12. "The prompt and early application of cold to the surface is a most
valuable mode of treatment of hyperpyrexia. The chances of its efficacy
are greater the earlier it is had recourse to. The temperature cannot
safely be allowed to rise above 105° F. Failing the most {67} certain
measure--viz. the cold bath--cold may be applied in various ways: by
the application of ice, by cold affusions, ice-bags, wet sheets, and
iced injections."

Whatever differences of opinion may obtain as to the value of cold in
the treatment of the hyperthermia of typhoid fever, there is a
tolerable consensus of opinion that it is our most reliable and
promptest resource in those formidable cases of rheumatic fever
attended with hyperpyrexia, both when alarming delirium and coma
coexist and when they are absent.[208] Space will not allow of details
here in the employment of cold to reduce hyperpyrexia--a subject
discussed elsewhere in this work. Suffice it to say, that besides the
cold bath (70° or 60°) which the committee regards as the most certain,
the tepid bath (96° to 86°) is employed by Fox and regarded as the best
by Andrews; it may be cooled down to 70° by adding ice or cold water to
it (Ziemssen). The cold wet sheet-pack is still thought much of, like
the last, in old and feeble people. Kibbie's method deserves more
attention than it has received. He pours tepid water (95° to 80°) over
the patient's body, covered from the axillæ to the thighs with a wet
sheet and laid upon a cot, through the open canvas of which the water
passes and is caught on a rubber cloth beneath the cot, and conveyed
into a bucket at the foot of the bed.

[Footnote 208: The powerful depressing effects of high temperature on
the human body, and the remarkable opposite influences of a cool
temperature, have been personally experienced by the writer in the last
three days. For two or three days the weather has been very hot, and he
has experienced the usual feeling of exhaustion, incapacity for thought
and action. After a thunderstorm last evening the temperature fell 25°,
and this morning, twelve hours later, he feels vigorous, refreshed, and
capable of intellectual and physical labor. The change is remarkable.]

The existence of polyarthritis, of peri- or endocarditis, of pneumonia
or pleurisy, does not contraindicate the cold bathing. If much weakness
of the heart obtains, it is well to give some wine or brandy before
employing the bath, and perhaps while in it, and the patient should not
be kept in the bath until the temperature reaches the norm, for it
continues to fall for some time after his removal from the bath. If the
temperature fall rapidly 2° to 3° in five or six minutes, remove the
patient from it as soon as the temperature recedes to 102° or 101° F.
If it fall very slowly, the bath may be continued till the temperature
declines to 99.5°, when he should be taken out. Should marked symptoms
of exhaustion or of cyanosis arise, the bathing should be at once
stopped. After it has been found necessary to employ cold in this way,
the thermometer should be used every hour, and if the temperature tend
to rise rapidly again, the diligent application of a succession of
towels wrung out of iced water and applied to the body and limbs, or of
Kibbie's method, may suffice; but should they not, and a temperature of
103° or 104° be rapidly attained again, the cold or tepid bath should
be at once resumed. In severe cases of this kind a liberal
administration of alcohol and liquid food is generally needed, and it
is well to try antipyretic doses of quinia by mouth or rectum, although
they are usually very disappointing in these cases. It is admitted that
cold baths have in a few rare instances caused congestion of the mucous
membrane, pneumonia, pleurisy, and even fatal syncope. This is a reason
for the exercise of care and constant oversight on the part of the
physician, but hardly an excuse for permitting a person to die in
rheumatic hyperpyrexia without affording {68} him at least the chance
of recovery by the use of the cold or tepid bath.

If delirium and deafness supervene during the employment of the
salicylates, it is prudent to suspend their use and take the
temperature every couple of hours, as one cannot feel confident that
hyperpyrexia may not be impending. Both Caton and Carter have found
that the addition of bromohydric acid to the sodium salicylate
mitigated or controlled the tinnitus and deafness produced by full
doses of that salt.

SUMMARY OF TREATMENT OF ACUTE RHEUMATIC POLYARTHRITIS.--As a general
rule, commence at once with a combination of sodium salicylate, say 10
grains, and citrate of potass. gr. xv, every hour for twelve doses,
after which give the citrate alone every two hours during the rest of
the day. Repeat these medicines in the same way daily until the
temperature and pain have subsided, when only half the above quantities
of the drugs are to be given every twenty-four hours for about a week
longer, after which three 15-gr. doses of the salicylate, with a like
quantity of the citrate, are to be administered every day for another
week or ten days, to prevent relapses. It is in this third week that
quinia is most likely to be required, and as a general rule it may be
given with benefit at this period in doses of 2 grains three times a
day between the doses of the salicylate. Should the above dose of
salicylate not relieve the pains sensibly in twenty-four hours,
increase next day the hourly dose to 15 or 20 grains; and if this free
administration of the medicine afford no relief after four or five
days' use, substitute for the salicylate salt the benzoate of ammonia
in 15- to 20-grain doses hourly, continuing the citrate of potassium
and conducting the treatment in the manner first advised. Should the
benzoate likewise fail after four or five days' trial, omit it, and
employ the full alkaline method together with the quinia, of which
about 10 to 15 grains may be given in the day between the doses of the
alkaline salt.

For the local treatment no uniform method is invariably applicable. In
many cases simply painting the joints with iodine daily, or enveloping
them in cotton wool, with or without the addition of belladonna or
laudanum, and securing it by the smooth and gentle pressure of a
flannel roller, proves sufficient. Hot linseed poultices containing a
teaspoonful of nitre or of carbonate of soda often afford relief, and
so does Fuller's lotion, applied to the articulations by means of
spongio-piline, or lint covered with oiled silk. It consists of liq.
opii. sed. fl. ounce j, potass. carb. drachm iv to drachm vj,
glycerinum fl. ounce ij, aqua fl. ounce ix. It must be plentifully
applied. If the articular affection be very severe and not relieved by
the above measures, absolute immobility of the joints, secured by means
of starch and plaster-of-Paris bandages, has been shown to be very
useful, relieving the pain, shortening the duration of the local and
the general disturbance, and protecting neighboring joints from
invasion.[209]

[Footnote 209: See Heubner in _Archiv der Heilkunde_, vol. xii., and
Oehme in _ibid._, vol. xiv., and a striking case in _St. Barth. Hosp.
Reports_, 1876, p. 174, by R. Bridges, M.D.]

We have little experience in this country of ice continuously applied
to the joints until all the symptoms of acute rheumatism have
disappeared (Esmarch and Stromeyer).

Circlets of blistering fluid applied above all the affected joints {69}
simultaneously, as practised especially by Herbert Davies,[210] often
afford prompt relief to the pain, but they do not invariably protect
the heart, in my experience.

[Footnote 210: _London Hospital Reports_, vol. i., 1864, 292.]

The hygienic and dietetic management of acute articular rheumatism
demands careful attention. While the room should be well supplied with
fresh air and sunlight, it should be kept at a uniform temperature and
free from draughts. Feather and other very soft beds should be
prohibited. Many authorities put the patient between heavy blankets,
which I regard as a mistake. The bed-clothing should be light and just
sufficient to keep the patient agreeably warm; the night-gown may be of
thin flannel and the sheets of cotton. The excess of perspiration
should be removed by gentle rubbing with a warm towel at regular
intervals, and the sheets should be changed frequently before they
become almost saturated with the perspiration. Fatigue and exposure of
the patient's person when taking food, attending to his natural calls,
or having his personal or bed-clothing changed should be specially
guarded against.

The diet in the early actively febrile stage should consist of panada,
corn-meal or oat-meal gruel, milk, and barley-water, or even pure milk.
Where persons will not take milk the various thin animal broths to
which good barley-water or arrowroot or well-boiled rice has been
added, jellies, sago and other starchy puddings, may be allowed.
Suitable drinks are--plain water, Seltzer and Apollinaris water,
carbonic-acid water, lemonade. This low, unstimulating diet should be
observed until all fever and articular inflammation have subsided, the
tongue become clean, and the visceral inflammations declined, and a
return to solid food, and especially to animal food, should be made
cautiously. Eggs are to be regarded as of very doubtful safety in this
disease. As a very general rule, ales, wines, and the stronger
alcoholic liquids are objectionable, but they may be required under the
same conditions as in other fevers. Should the salicylates depress the
heart, old wine or whiskey may be given with advantage.

During convalescence the patient should not be permitted to leave his
bed for several days after complete removal of the fever and articular
pain, and for the first four days he should occupy a sofa or
easy-chair. Premature walking may induce relapse. An occasional
alkaline or sulphur bath, if cautiously taken, sometimes appears to
complete the recovery. If endocarditis have existed, a longer rest is
desirable, more especially in severe cases, in order that the
reparative process going on in the lately inflamed valves may not be in
the least disturbed.


Chronic Articular Rheumatism,

synonymous with rheumarthritis chronica, rheumatisme articulaire
chronique simple (Besnier), polyarthritis synovialis chronica (Heuter),
is defined here as a chronic idiopathic inflammation of one or a few
articulations, which is more prone to become fixed than the acute form,
and which, notwithstanding its protracted duration, produces no
profound structural alterations in the joints.

ETIOLOGY.--It may be the direct sequel of a single attack or more {70}
commonly of several attacks, of acute, or more especially of subacute,
articular rheumatism. But it is generally a primary affection,
occurring in persons who have not had either acute or subacute
rheumarthritis, yet owning the same causation as these, and
occasionally in its course exhibiting acute or subacute symptoms. The
specially predisposing conditions are inheritance; repeated attacks of
subacute or acute articular rheumatism, which in accordance with
general laws impair the resisting power of the affected joints;
prolonged residence or employment in cold, damp, or wet rooms or
localities; repeated exposure to bleak, cold currents of air or to
frequent wettings of the body or lower limbs. For these reasons it is
most common amongst the poor, who are especially exposed to the
influences just mentioned; and amongst them cellar-men and sailors,
washerwomen and maid-servants, are very liable to the disease. It is
chiefly an affection of advanced life, or at least of mid-age, and is
rare in youth. The first attacks, and especially exacerbations, are apt
to be induced by the direct action of a draught of cold air or by
unusual exposure to cold and damp air, especially when the body has
been fatigued or overheated. In many cases no distinct exciting cause
can be traced.

The morbid anatomy of simple chronic articular rheumatism will vary
with the severity and duration of the disease. The alterations are such
as chronic inflammation of a non-suppurative character might be
expected to produce in the joints by one who had learned those
characteristic of acute rheumarthritis. In the simple chronic form the
proliferating process involves chiefly the synovial membrane, the
capsular and other ligaments, and the periarticular tissues; to a less
degree the cartilages, and to a much less degree, and exceptionally,
the osseous surfaces. The synovial membrane is thickened, slightly
injected, and its fringes hypertrophied and more vascular than
normally. Little fluid usually exists in the joint unless during an
exacerbation, when a moderate amount of thin, cloudy serum may be
present; generally only a trace of thick, turbid fluid, containing
oil-globules, and in severe cases débris of the cartilages, but no pus,
is found. The fibrous capsule and ligaments become thickened, dense,
and stiffened by hyperplasia; and sometimes the adjacent tendons and
their sheaths, the fasciæ and aponeuroses, undergo similar alterations,
so that the movements of the joints become seriously interfered with.
In some cases this irritative hyperplasia specially involves these
periarticular fibrous structures, and these, undergoing retraction,
produce marked deviations, subluxations, and deformities of the
articulations very like those observed in rheumatoid arthritis,
although the osseous components of the joints are unaffected. Jaccoud
gave to such cases the title of chronic fibrous rheumatism.[211] It is
worth noting that Jaccoud's, Charcot's,[212] and Rinquet's[213] cases
of so-called "chronic fibrous rheumatism" developed out of acute
articular rheumatism, while Besnier's was primarily chronic. In simple
chronic rheumatism, if protracted, the cartilages also proliferate,
lose their semi-transparency and polish, and become opaque and white;
they are often rough and traversed by fissures, and occasionally
present erosions; and these erosions {71} are either naked or covered
with a layer of newly-formed connective tissue, which may occasionally
produce fibrous adhesions between the articular surfaces. Points of
calcification occur in the cartilages and tendons in very chronic
cases. Instances are observed in which the bones exhibit, to a slight
degree, the alterations found in rheumatoid arthritis, and are probably
transitional between the two affections. The muscles which move the
affected articulations in severe cases are often atrophied, and the
wasting imparts to the joints an appearance of considerable
enlargement.

[Footnote 211: Vide Jaccoud, _Clin. Méd. de la Charité_, 23e Leçon,
Paris, 1867.]

[Footnote 212: Besnier, _Dictionnaire Encyclopéd., etc._, t. iv., p.
680 _et seq._]

[Footnote 213: _Du Rheum. Artic. Chronique, etc._, par Martial Rinquet,
Thèse, Paris, 1879, pp. 28-33.]

SYMPTOMS AND COURSE.--Simple chronic articular rheumatism presents many
varieties. In the milder forms the patient experiences trifling or
severe pain in one, or less frequently in two or more, joints, more
especially in the knee or shoulder, or both, attended with want of
power in the member or with stiffness in the affected articulation. The
pain frequently is likewise felt in the soft parts, muscular and
tendinous, near the joints, and is usually increased by active or
passive movement; it is not always accompanied by tenderness, and
rarely with local elevation of temperature or swelling. The wearying
aching in the joint is of an abiding character, but is very liable to
exacerbations, especially at night; and these come on just before
atmospheric changes, such as a considerable fall of temperature, the
approach of rain, variations in the direction of the wind, etc., and
they usually continue as long as the weather remains cold and wet. A
very common symptom is a creaking or a grating which may be felt and
heard during the movements of the joint.

The above symptoms may rarely prove more or less constant by night and
day for years, but far more frequently, at least at first, they last an
indefinite period and disappear to recur again and again, especially in
the cold and changeable seasons of the year. Although in the earlier
attacks, and often for a long time, no alteration of structure is
perceptible in the painful joints, yet in some instances slight
effusion into the articulation may be observed during the
exacerbations, or the capsule and ligaments may at length become
slightly thickened, or the muscles may waste and produce an apparent
enlargement of the joint; and this prominence of the articular surfaces
may be increased by retraction of the tendons and aponeuroses--a
condition which causes real deformities (deviations, subluxations,
etc.) of the articulation and impairs more or less its movements. In
very chronic cases a fibrous ankylosis may be established.

These last-mentioned conditions often entail great and long-continued
suffering, and may even cause some anæmia and general debility; but
very frequently the general health and vigor continue good,
notwithstanding the permanent impairment of the functions of one or
several of the large articulations, and the liability to exacerbations
often amounting to attacks of subacute rheumarthritis from changes in
the weather, fatigue, or exposure.

Besides the above varieties may be mentioned a not infrequent one
consisting of a series of attacks of subacute articular rheumatism
recurring at short intervals, involving the same joints, and attended
with slight elevation of temperature, febrile urine, perspiration, and
moderate local evidences of synovitis, heat, pain, tenderness,
swelling, and effusion into the affected joints. This is an obstinate
variety, and is often associated with rheumatic pain in the muscles and
fibrous tissues of the affected member.

{72} Simple chronic articular rheumatism, like the acute form, is most
apt to affect the larger articulations, knees, shoulders, etc., but it
frequently also involves the smaller ones of the hands and feet.
Although usually polyarticular, it is prone to become fixed in a single
joint, but even then it may attack several other articulations, and may
migrate from one to another without damaging any.

The course of the disease is usually one of deterioration during
persistent or recurring attacks, and in many cases the intervals of
relief become shorter and less marked; the joints become weaker and
stiffer; and although the pain may not increase and the general health
may not be seriously impaired, yet the patients may continue for many
years or the rest of their lives severe sufferers, unable to work, and
often hardly able to walk even with the aid of a stick. Occasionally,
after several years of pain and weakness, a sudden or slow improvement
may set in and the patient become free from pain and lameness, and only
experience some stiffness in the movements of the joints after several
hours of rest, and slight thickening of the ligaments and capsule of
one or more articulations. The duration of the disease is indefinite;
the danger to life trifling.

The complications of simple chronic articular rheumatism are held by
many, and especially by those who regard the disease as constitutional
or diathetic, to be the same as those of the acute form, and that they
may precede, follow, alternate, or occur simultaneously with the
articular affection. All admit that they are observed much less
frequently in the former than in the latter. Other pathologists either
deny the occurrence of the visceral complications (Senator, Flint) or
do not mention them (Niemeyer). It is not denied that cardiac disease
may be found in chronic articular rheumatism which has succeeded the
acute form, and which may then be referred to the acute attack. The
tissue-changes then set up may not have produced at the time the
murmurs indicative of endocarditis, but these tissue-changes may have
ultimately roughened the endocardium, puckered a valve, or shortened
its cords, so that cases of chronic articular rheumatism having a
history of an acute attack cannot be safely included when inquiring
into the influence of the chronic form upon the heart or other internal
organ. Attention has not been sufficiently given to ascertain the
frequency of the occurrence of these complications in primary chronic
articular rheumatism, and reliable evidence is not at hand. It is not
unlikely that the chronic form may slowly develop cardiac changes, as
the acute form rapidly does; but when the advanced age of the persons
most liable to chronic rheumatism is borne in mind, it must be admitted
that valvular and arterial lesions (endarteritis) are observed at such
periods of life independently of rheumatism, and referable to such
causes as repeated muscular effort, strain, chronic Bright's disease,
senile degeneration, etc. Somewhat similar observations are applicable
to the attacks of asthma, of subacute bronchitis, of neuralgia, and of
dyspepsia, which are frequently complained of by sufferers from simple
chronic rheumarthritis. Such affections are common in elderly people in
cold and damp climates; they may be mere complications rather than
manifestations of rheumatism, or outcomes of the confinement and its
attendant evils incident to chronic articular rheumatism, as is
probably the relationship of the dyspepsia. There is {73} no doubt of
the frequent coexistence of muscular rheumatism with this variety.

DIAGNOSIS.--Simple chronic articular rheumatism may be confounded with
rheumatoid arthritis, with the articular affections of locomotor ataxia
and other spinal diseases, with chronic articular gout, with syphilitic
and with strumous disease of the joints. The reader may consult the
observations made on four of these affections in connection with the
diagnosis of rheumatoid arthritis. A few additional remarks are called
for in distinguishing chronic articular rheumatism from chronic
articular gout, which is often a very difficult problem. Both are apt
to be asymmetrical in distribution, to have paroxysmal exacerbations,
to recur frequently without damaging the articulations, to have been
preceded by acute attacks of their respective affections, and to be
uncomplicated by endo- or pericarditis. But chronic rheumarthritis has
no special tendency to attack the great toe; it is more persistent than
gouty arthritis; it does not, even when of long standing, produce the
peculiar deformities of the articulations or the visible chalk-like
deposits in the ears or fingers observed in chronic gout. The etiology
of the two diseases is dissimilar. There is no special liability to
interstitial nephritis in articular rheumatism, nor is urate of soda
present in the blood in that disease.

In chronic strumous or tubercular disease of a joint the youth, the
personal and family history, and sometimes the evident defective
nutrition, of the patient; the moderate degree of local pain compared
with the considerable progressive and uniform enlargement of the joint;
the evident marked thickening of the synovial membrane, either early or
late according as the disease has originated in the synovial membrane
or in the bones; the continuous course, without marked remissions or
exacerbations, of the disease; the rarity with which more than one
joint is affected; and the tendency to suppuration, ulceration, marked
deformity, and final destruction of the joint,--will prevent the
disease from being mistaken for chronic rheumatism.

The PROGNOSIS in simple chronic rheumarthritis is unfavorable as
regards complete recovery, and it is chiefly while comparatively
recent, and when the sufferer can be removed from the conditions
productive of the disease, that permanent improvement, and sometimes
cure, may be expected. As a rule, the disease once established recurs.
It does not, however, endanger life.

TREATMENT.--All are agreed that hygienic treatment constitutes an
essential, if not the most valuable, part of the curative and
palliative management of chronic rheumarthritis. A dry and uniform
climate is the most suitable, and there is much evidence in favor of a
dry and warm rather than a dry and cold climate. Protection of the body
against cold and damp by means of flannel next the skin, sufficient
clothing, residence in dry and warm houses, etc., is of prime
importance. In fact, all the known or suspected causes of the disease
should be as far as possible removed.

The direct treatment of the disease resolves itself into general and
local, and is essentially the same as that recommended for rheumatoid
arthritis, to which subject the reader is referred. A few observations
only need be made here. Although, like everything else in chronic
rheumarthritis, it often fails, no single remedy has in the writer's
{74} experience afforded so much relief to the pain and stiffness of
the joints as the sodium salicylate; and he cites with pleasure the
confirmatory testimony of J. T. Eskridge of Philadelphia,[214] of whose
28 cases 75 per cent. were decidedly benefited. Jacob of Leeds also
reports some benefit in 75 per cent. out of 87 cases treated by the
same agent.[215] It must be given in full doses, and be persevered
with. Salicylate of quinia should be tried if there be much debility or
if the sodium salt fail. Propylamine or trimethylamine is deserving of
further trial in this disease. From 100 to 200 grains are given in the
day in peppermint-water. Iodide of potassium, cod-liver oil, arsenic,
iodide of iron, and quinia are all and several remedies from which more
or less benefit is derived in chronic articular rheumatism. The
combination of iodide of potassium with guiaiac resin--gr. ij-iij of
each three times a day in syrup and cinnamon-water--is sometimes very
useful. The writer has no experience of the bromide of lithium
(Bartholow). When the skin is habitually dry and harsh a dose of
pilocarpine every other night for a few times will often prove very
useful.

[Footnote 214: _Phila. Med. Times_, vol. ix. pp. 75-77, 1878, and _The
Medical Bulletin_, Phila., July, 1879, pp. 44-48.]

[Footnote 215: _Brit. Med. Jour._, ii., 1879, 171.]

Cod-liver oil, iron, quinia, etc., the various forms of baths and
mineral waters, electricity, and the several local measures recommended
for the treatment of rheumatoid arthritis, are all occasionally very
useful in, and constitute the appropriate treatment of, simple chronic
articular rheumatism. The dietetic management of the two affections
should be the same.


Muscular Rheumatism.

SYNONYMS.--Myalgia rheumatica or myopathia; _Fr._ Rheumatisme
musculaire; _Ger._ Muskelrheumatismus.

DEFINITION.--The affections included under this term are certain
painful disorders of fibro-muscular structures. They are commonly found
in persons the subjects of the rheumatic diathesis, and are
characterized by pain and often spasm, and sometimes a slight degree of
fever. No doubt as our knowledge increases so many attacks connected
with painful states of muscles and fasciæ are eliminated from the
somewhat uncertain group of muscular rheumatism. True inflammation is
not believed to exist, and pathological investigation has rarely shown
any morbid changes in the affected parts. The symptoms, therefore, have
been attributed to some temporary hyperæmia, slight serous exudation,
or neuralgic state of the sensory nerve-filaments. The strongest
support is given to this statement from the absence of any marked
tenderness in such affected muscles as can be sufficiently examined. In
certain cases, undistinguishable clinically, it is quite probable that
a periarthritis is in reality the principal factor in the case. In
others, again, a subacute rheumatism affecting a joint seems to spread
to the adjoining tendinous sheaths, and thus secondarily to attack the
muscles themselves, the affection of which may ultimately remain the
only condition present.

ETIOLOGY.--Muscular rheumatism is a very common affection. All ages are
liable to its occurrence, but the part affected varies with the time
{75} of life, children and young adults being much more subject to
torticollis, and older persons to lumbago and general rheumatism of the
limbs. Amongst hospital patients the disease prevails more amongst men
than women, owing doubtless to the greater exposure of the former to
the cold; but amongst other classes the same difference is not seen. It
is observed in all countries, but according to some writers it is
unusually frequent in tropical climates, although there acute
rheumatism is very uncommon. The causes of muscular rheumatism are
mainly exposure to cold and strain or fatigue of muscles. If these two
conditions coexist--_e.g._ standing in a draught of cold air or lying
on the ground when fatigued--the chances of the affection coming are
greatly enhanced. Strain, a twist of the body, or a false step can
actively start an attack of this kind, and by the sufferers themselves
it is constantly attributed to this cause. The part played by this
element is difficult to determine, a very slight strain being often
followed by great pain and distress from the subsequent rheumatic
affection. Some individuals are specially prone to attacks, the
slightest current of air, change of clothing, etc. being sufficient to
determine its occurrence. These persons are often found to have
suffered from rheumatism in some other form, and thus in them we must
consider that the rheumatic diathesis furnishes the reason for their
unusual susceptibility. It only remains to mention the fact that a
disposition to gout seems to favor the development of muscular
rheumatism. In gouty families, therefore, it has been observed to be
common.

SYMPTOMS.--In all cases pain is the prominent, and in many cases the
only, symptom present. In all except the more aggravated attacks pain
is felt only when the affected part is disturbed. In such when complete
rest or fixed immobility is maintained there is comfort, or at most a
somewhat dull, uneasy sensation, but when any contraction of the
muscles in question is produced, whether voluntary or otherwise, severe
often excruciating pain is at once experienced, often giving rise to a
sudden cry or causing the features to be contracted in a grimace. The
suffering ceases almost at once when the muscular contraction is
relaxed. In more aggravated attacks the pain is more severe, and
besides persists, though to a less degree, even when there is no
contraction. In rare cases when the maximum degree has been attained
there is continuous pain, but the affected muscles are persistently
maintained in a relaxed condition by means of true spasm in the
surrounding muscles. Slow passive movement affects the subject of
muscular rheumatism, and may often be accomplished with a little
management without causing pain. If, at the same time, these muscles be
handled by pinching and slight pressure, it will be found that they are
very sensitive to the touch. When some tenderness does exist, it is
slight and is not located in the district of the lower nerve-trunks.
Pressure even sometimes allays pain. The constant effort to avoid pain
gives rise to a feeling and appearance of stiffness, and thus
characteristic attitudes and positions of the head, trunk, or limbs are
voluntarily and persistently maintained. There is no spasm of the
affected muscles; the distortion is the result of stiff contraction of
the associated muscles, which thus forcibly fix the faulty one and hold
it in a state of relaxation. Cramp or spasmodic contraction of a single
muscle of a painful character does, however, sometimes occur in
rheumatic subjects, and much resembles the condition above described.
In {76} the same persons also muscular rheumatism may occur in a much
more fugitive or erratic form, frequently being nothing more than a
slightly painful condition of some group of muscles which have in some
way been exposed to cold. This may last but a short time, and either
spontaneously disappear or be readily removed by exercise or friction.
Muscular rheumatism is generally confined to one muscle or a single
group of muscles. Those most liable to it are the very superficial and
those easily exposed to cold (_e.g._ the deltoid and trapezius),
powerful muscles often subjected to violent strain (_e.g._ the lumbar
muscles), and those aiding in the formation of the parietes of the
great cavities.

This affection very commonly exists without any constitutional
disturbances, but sometimes there are present the symptoms of
pyrexia--slight elevation of temperature and temporary disorder of the
digestive organs--loss of appetite, constipation, and general malaise.

The acute forms generally last but a few days, terminating by gradual
subsidence and final disappearance of the pain. The fugitive kind,
already alluded to, may, however, be present more or less during
several weeks.

DIAGNOSIS.--Errors of diagnosis between muscular rheumatism and a
variety of other disorders are common. Laymen especially are only too
apt to attribute pain felt in muscles at once to rheumatism of these
muscles--a term which is badly abused. Some of these errors are of no
great interest, but others are of the highest importance, for they may
cause the onset of a serious disease to be overlooked. The principal
affections to be borne in mind with reference to diagnosis are the
following: organic diseases of the spinal cord (notably tabes
dorsalis), causing peripheral pains as an early symptom; functional
disorder of the same part, as hysteria or spinal irritation;
intra-thoracic inflammation; the onset of an exanthem; the pains
produced by the chronic poisoning of lead and mercury; neuralgia;
painful spasm of muscle from deep-seated inflammation or suppuration.
It is sufficient to indicate these various sources of fallacy, which,
if remembered, can generally be guarded against by a consideration of
the special features characteristic of each one.

TREATMENT.--The indications for the treatment are mainly two--viz. to
relieve the pain and to counteract the diathetic condition generally
present. The relief of the pain is accomplished in various ways,
according to the seat of the trouble. In severe cases it is proper to
resort to the hypodermic use of morphia, to which may be advantageously
added some atropia. When the pain is seated in large muscles, the
injection will produce better results if thrown not merely under the
skin, but into the substance of the muscle. Sometimes perfect rest in
bed is necessary to secure the required immobility; in other cases this
can better be secured by plaster or firm bandages. Soothing anodynes
are extremely useful locally, and counter-irritants also may be used
with benefit. Liniments give us a convenient form of application. The
best are those containing a considerable proportion of chloroform with
either aconite or belladonna, or both. The repeated application of
tincture of iodine often gives great relief. Galvanism sometimes proves
a rapid cure. Continuous heat is nearly always grateful, and may be
applied either in the dry form or by means of soft warm linseed
poultices with or without a {77} percentage of mustard. When these are
discontinued, care should be taken to protect the affected muscles from
cold by keeping them enveloped in flannel or woollen coverings.

Whilst these local measures are being adopted the constitutional
disorder should also receive attention. A diaphoretic action should be
set up. For this purpose the hot-air or Turkish bath at the outset
would seem to be sometimes really abortive. Of medicinal means amongst
the most reliable are liquor ammonii acetatis and Dover's powder.
Pilocarpine occasionally proves useful. The fixed alkaline salts are
also sometimes beneficial, such as the acetate and citrate of potassium
and, at a later stage, the iodide of potassium. In a certain number of
cases of muscular rheumatism the sodium salicylate acts promptly and
well. This drug will succeed well in proportion as the evidence of the
rheumatic constitution is well marked, as shown by the tendency on
other occasions to attacks of acute articular rheumatism.

Persons who are subject to muscular rheumatism should be made to wear
warm clothing, avoid draughts, guard against strains and twists, and in
other respects to be careful of their general hygiene. Obstinately
recurring cases will very often receive benefit from a visit to some of
the natural springs known to possess antirheumatic qualities.

The chief varieties of muscular rheumatism, divided according to the
locality affected, require some separate description.

1. Lumbago, or myalgia lumbalis, is that common form which attacks the
lumbar muscles and the strong aponeurotic structures in connection with
these. It is more frequently than any other form attributed to some
effort of lifting or sudden twist of the trunk, but in many cases it
owes its origin directly to exposure to cold. The pain comes on
suddenly and renders the person helpless, the body, if he is able to go
about, being held stiffly to prevent any movement or bending; if
severe, he is absolutely compelled to observe complete rest in bed. The
muscles, when handled, appear slightly sore, but no local point of
acute tenderness can be found. This fact, with the characteristic
shrinking from any movement, distinguishes lumbago from neuralgia and
from abscess. Pain in the loins, more or less severe, is such a
frequent accompaniment of disorder of several organs and parts that
careful examination should always be instituted lest some serious
organic disease with lumbar pain as a symptom be mistaken for a simple
lumbago. The most important of these are perinephritis, lumbar abscess,
spinal disease, abdominal abscess, and disease of the rectum and
uterus.

2. Pleurodynia, myalgia pectoralis or intercostalis. Here the affected
muscles are the intercostals, and in some cases the pectorals as well.
Spasmodic pain is felt in one or other side of the chest, and is
especially aggravated by the movements of respiration; it is rendered
intense by the efforts of coughing or sneezing. Pleurodynia may be
confounded with pleurisy, the distinguishing features being the absence
of fever and the friction sound of pleurisy. Intercostal neuralgia is
sometimes with difficulty known from pleurodynia, but in the former the
pain is more circumscribed, more paroxysmal, and more easily aggravated
by pressure than in pleurodynia, and when severe there are tender
points in the course of the nerve a little outside of the middle line
posteriorly (dorsal point) and anteriorly (sternal point). Now and then
the hyperæsthetic {78} areas become anæsthetic, and even patches of
herpes may form in the course of the nerve, when doubt can no longer
remain. From periostitis of a rib pleurodynia may be known by the fact
that in the one the tenderness is marked in the intercostal space, and
in the other in the rib itself. Pleurodynia is a frequent accompaniment
of thoracic affections, causing cough, the frequent paroxysms of
coughing tending to induce a painful state of the overworked muscles.
The pain, which may be very great, can often be controlled by fixing
the chest with imbricated plaster or a firm bandage. Dry cups sometimes
answer very well; if more active measures are necessary, then
hypodermic injections of morphia must be resorted to.

3. Torticollis, myalgia cervicalis, stiff neck or wry neck, caput
obstipum. This term includes those cases of rheumatic idiopathic
affection of one or more of the muscles of the side and nape of the
neck, which fixes the head firmly in the median line or else in a
twisted fashion, with the face turned toward the sound side. The
disease can be recognized at a glance by the peculiar manner in which a
person will turn his whole body round instead of rotating his head
alone. It is much more common in children than in adults. The
sterno-mastoid is the muscle chiefly affected, but any of the muscles
of the neck may become rheumatic in the same way, and frequently
several of them suffer at the same time. The most important point at
the outset of an attack of wry neck is to determine whether we have to
do with a true rheumatic (idiopathic) disorder, or whether the muscular
stiffness is secondary to some spinal or vertebral lesion. The
diagnosis is usually founded upon the suddenness of the onset, the
absence of other symptoms of nerve disease, and the rapid course of the
case, terminating in a cure in a few days. There is nothing special in
the treatment of torticollis beyond what has been already said under
the general heading.

Other forms of muscular rheumatism which have received special names
and have been separately described are the following: myalgia
scapularis or omalgia, when the surroundings of the shoulder are
affected; myalgia cephalica or cephalodynia, an affection of the
occipito-frontalis; and abdominal rheumatism, when the external muscles
of the abdomen are involved.


Rheumatoid Arthritis.

SYNONYMS.--Nodosity of the joints (Haygarth); Chronic rheumatic
arthritis, or rheumatic gout (Adams); Arthritis, rheumatismo
superveniens (Musgrove); Goutte asthénique primitive; Arthritis
pauperum; A. sicca; Usure des cartilages articulaires (Cruveilhier);
Arthrite chronique (Lute); Progressive chronic articular rheumatism;
General and partial chronic osteo-arthritis;[216] Arthritis deformans.

[Footnote 216: _Nomenclature of Diseases R. C. Physicians_, London.]

Neither my space nor time will permit of a history of this disease; it
must suffice to say that Sydenham in 1766-69 appears to have first
tersely described it and distinguished it from gout; that in 1800,
Landré-Beauvais in his inaugural thesis made some observations upon the
disease under the title of primary asthenic gout; that in 1804,
Heberden, and {79} more especially Haygarth, in 1805, pointed out some
of the more striking clinical features of this disease, and
distinguished it from both gout and chronic rheumatism under the title
nodosity of the joints. The latter author, in the work mentioned,
claims to have written a paper upon the subject twenty-six years
previously, although it was not published; and to him belongs the merit
of having so described the disease as to have given it a place in
nosology. Incidental allusions were made to the affection in 1813 by
Chomel, in 1818 by Brodie, and by Aston-Key in 1835; in 1833, Lobstein,
and about the same time Cruveilhier, pointed out some of the more
striking characters of the morbid anatomy of the affection. But it is
to Adams of Dublin that we are indebted for the most complete account
of the anatomy and of many of the clinical features of the
disease--first in a paper read before the British Association in 1836,
next in his article on "The Abnormal Conditions of the Elbow, Hand,
Hip, etc.,"[217] and finally in his able monogram "On Rheumatic Gout"
in 1857. The contributions to this subject since that date have been
very numerous as well as valuable from the leading countries of Europe,
and I must not here attempt to assign to each investigator his proper
portion of the work.

[Footnote 217: Todd's _Cyclop. of Anat. and Phys._ (1836-39).]

It may be here remarked that Landré-Beauvais and Haygarth described
more particularly that form of the disease which, beginning in the
small joints of the extremities, tends to extend to the larger joints
in a centripetal way, and to involve many of them--peculiarities which
have given rise to the epithets progressive polyarticular chronic
rheumatism, peripheral arthritis deformans, and which is the form of
the disease usually described by physicians as rheumatic gout,
rheumatoid arthritis, nodular rheumatism, and by the other names just
mentioned. On the other hand, Key, Colles, Adams in his earlier paper,
and R. W. Smith described the disease as it affects the larger joints,
hip, shoulder, or knee, to one or two only of which it may be confined;
and as this variety is frequently observed in elderly persons, and in
them often involves the hip, it is often spoken of as senile arthritis,
malum senile articulorum, morbus coxe senilis, mono-articular arthritis
deformans, partial chronic rheumatism, and has been described by
surgeons rather than by physicians. However, even when beginning in the
hip or shoulder, the disease is apt to involve several of the
intervertebral articulations, and not unfrequently to extend to other
joints than the one first affected, and even to the peripheral joints.
Its progressive and general nature is thus evidenced, whether it invade
from the beginning a single large joint or several symmetrical small
articulations. Finally, on this topic Charcot has insisted that
Heberden's nodi digitorum contributes a special form of the disease
under consideration, and proposes to call it Heberden's rheumatism or
nodosities.[218]

[Footnote 218: _Lectures on Senile Diseases_, Syd. ed., 1881, p. 137.]

Rheumatoid arthritis presents the clinical varieties or groupings of
phenomena just mentioned, at times quite distinctly appreciable from
one another, but sometimes more or less blended, yet even then
manifesting in their periods of invasion and early stages an adhesion
to all of these typical groupings. Charcot has especially dwelt upon
these: 1st, the general or polyarticular and progressive form; 2d, the
partial or oligo- or mono-articular form; 3d, Heberden's nodosities.

{80} 1st. The symptoms and clinical history of general or polyarticular
and progressive rheumatoid arthritis. This is the most common form of
so-called chronic rheumatic arthritis, the classical rheumatic gout, or
rheumatisme noueux, and it may declare itself, as Garrod and Fuller
pointed out, very rarely in an active or acute form, or, as it usually
does, in a chronic and insidious form.

The acute form of rheumatoid arthritis closely resembles the milder
varieties of acute articular rheumatism or the best marked examples of
the subacute form of that disease. But it presents the following
particulars, by which it may generally perhaps, but not always, be
distinguished: while the temperature, the thirst, the furring of the
tongue, the frequency of the pulse, the articular pains and tenderness,
etc., are less developed than in acute articular rheumatism, there is
wanting the profuse and continued perspiration, the early involvement
of the endo- or pericardium in the inflammation, and the prompt
prostration of the strength so commonly witnessed in that disease. On
the other hand, while the rheumatoid affection may involve the larger
joints--knees, ankles, elbows, and wrists--it almost certainly
implicates the smaller joints of the fingers, and often of the toes.
There is apt to be greater effusion into the synovial capsules
(McLeod's capsular rheumatism) and into the synovial sheaths and bursæ
about the affected joints than in ordinary acute or subacute
rheumatism; further, the inflammation does not migrate from joint to
joint, but obstinately persists in several of them, and more especially
in the wrist and in the metacarpo-phalangeal joints of the index and
middle finger, perhaps also in the ankles and in the
metatarso-phalangeal articulation of the great toe. Instead of
disappearing in four to six weeks, the articular inflammation
continues, although the pain may abate very much, and the capsules of
the joints continue swollen and rather tense. The muscles of the
extremities waste, and are the seat of painful reflex spasms which
interfere with the movements of the joints; and although the patient is
capable of moving about, and is free from all febrile disturbance, one
or several of his joints remain permanently swollen, painful, and
crippled. Perfect restoration of all the affected joints seldom if ever
occurs. In common with other observers, I have met with this acute form
most frequently in young women twenty to thirty years of age--several
times in connection with recent delivery or rapid child-bearing, or
lactation; once after what was regarded by the medical attendant as an
attack of acute rheumatism occurring not long after labor. It has been
observed in children, and is not uncommon after forty. These patients
usually suffer in their general health--become weak, pale, depressed in
spirits, and lose flesh. In several cases of this form marked intervals
of improvement have occurred; the local disease has ceased to progress,
and tolerable comfort has been experienced, perhaps, till pregnancy,
delivery, or lactation again determined a fresh outbreak of the
disease. Sometimes, however, this acute form steadily advances, and in
a year or two establishes changes in the cartilaginous and osseous
structure of the affected joints. Such a case I met in a lady of
twenty-one who had had a good deal of anxiety as a mathematical
teacher, and whose illness set in during vacation while at the seaside.
It proved obstinately progressive for several years, until several of
the larger joints, as well as the smaller, were badly crippled.

{81} The primary chronic form is much the more frequent, although
between it and the acute variety there are many intermediate grades.
For weeks or months the patient may experience numbness or formication
and rheumatic pains in the limbs, perhaps with a sense of stiffness in
the joints, especially felt after rest or the day after unusual
fatigue. Then one or more joints--most frequently the
metacarpo-phalangeal of the fingers--become painful, swollen, tender
when touched, and inordinately hot; these symptoms may subside under
rest or treatment, and after weeks or months recur, either without
known cause or from exposure, fatigue, or some impairment of the
health. Usually, the original joint is again affected, but frequently
one or two more of the same on the other hand suffer likewise. More or
less complete remissions of the pain and local inflammation now tend to
take place from time to time and alternate with exacerbations or fresh
attacks of the local disturbance, and the disease extends, as it were,
centripetally and more or less symmetrically to the wrists, then to the
elbows, and then to the shoulders, or from the toes to the ankles and
thence to the knees--although there is no invariable sequence of this
kind--and next to the hands; the knees are specially liable to
invasion. Of Haygarth's 34 cases, in 2 the knees alone suffered, and
"in all or nearly all the rest the hands, chiefly the fingers, were
probably affected." In Charcot's 45 cases the début took place in the
small joints of the hands and feet 29 times; in the hands, feet, and
one large articulation, 7 times; in one large joint, and later in the
fingers, 9 times. Even in this primary chronic form there is usually in
the earlier stages some effusion into the joints; the soft parts of the
articulation are thickened and swollen; obscure fluctuation in the
smaller and very distinct fluctuation in the larger joints may be felt.
The pain may be severe, especially at night, and during the
exacerbations of the disease it varies greatly in its degree and
persistency. The position and shape of the joints are altered, partly
by spasmodic retraction of the muscles, and more or less by the
effusion into the capsules and adjacent bursæ and sheaths, and the
thickening of the soft parts covering the articulations. As the disease
progresses further deformities ensue from the growth of new bone around
the heads of the bones, the absorption of the articular cartilage, the
development of masses of cartilage in the hypertrophied synovial
processes and beneath the synovial membrane at the margin of the bones;
the relaxation of the articular ligaments; and the displacements and
subluxations of the unshapely bones composing the joint. The great
wasting of the muscles of the member affected has some share in
producing its unnatural appearance. In the advanced stage there is more
or less abiding pain, soreness, and stiffness in the affected
articulations, violent cramps are experienced in the course of the
adjacent muscles, and pains either along the nerves or vaguely down the
limbs. Crackings or creakings are to be heard, and grating is to be
felt during the movements of the joints; these movements become more
and more restricted, so that an immobility almost equal to that of true
bony ankylosis is established, this result seldom occurring except
amongst the carpal, tarsal, tibio-tarsal, and the vertebral
articulations. Interlocking of the osteophites formed on and around the
articular surfaces, and in other cases union of these surfaces by the
interposition of newly-formed fibrous tissue, produce a spurious
ankylosis {82} destructive of the articular functions. In the very
advanced stages the feet, ankles, and legs are often considerably
enlarged and the integument thickened by a chronic oedematous
infiltration, or the bones and soft parts are atrophied and the
integument is pale, smooth, and attenuated, resembling parchment or the
condition seen in certain stages of scleroderma and tightly drawn over
the wasted rigid fingers. This primary chronic form is especially apt
to progress steadily for many years, the joints earliest affected
becoming gradually more distorted and crippled, and fresh joints
becoming invaded until there may hardly remain a single sound
articulation in the limbs, or even in the body; and at length the
patient may be unable to feed himself or masticate or raise his chin
from his sternum or rotate his head or stand.

The deformities of the several joints, being largely the result of
muscular contraction, observe certain general types, which, however,
are not peculiar to the disease, but occur in various affections of the
nerve-centres, involving paralysis or spasm or both. Charcot has
carefully described those met with in the hands, and I must refer to
his masterly article upon chronic articular rheumatism for his account
of them.

(1) It must suffice to say here that the predominant features of the
hand in chronic rheumatoid arthritis are the following: The first
phalanx of the fingers is either flexed upon the metacarpus or
extended, and the terminal phalanx in like manner is either markedly
flexed or extended upon the second, or these two phalanges are
maintained in a straight line, while the first phalanx is, as usual,
decidedly flexed upon the metacarpus.[219] In all these varieties the
hand is pronated; there is a great tendency to deviation of the fingers
toward the ulnar border of the hand, although sometimes the deformed
fingers stand out, not unlike a bunch of parsnips. The thumb escapes
longer than the other fingers, and its metacarpo-phalangeal joint is
usually flexed, rarely extended.

[Footnote 219: _Lectures on Senile Diseases_, Syd. ed., trans. 1881.
Figs. 1 and 2, Pl. II., on the hand, give good illustrations of these
deformities.]

(2) The great toe, enlarged at the metacarpo-phalangeal articulation,
is usually drawn to the outer border of the foot, across and above, but
rarely below, the other toes, and the foot is usually abducted and
flattened, the prominent internal border resting on the ground. The
wrist, elbow, and knee-joints are generally flexed; the distal ends of
the ulna and radius, more or less enlarged, project backward; the
semi-flexed tibia is drawn backward on the femur and rotated outward,
thus rendering the internal condyle of the femur prominent and
displacing the patella toward the external condyle, and foreign bodies
may frequently be felt in the enlarged knee- and elbow-joints. Finally,
the extremities of the affected bones will, as a rule, be found
enlarged and misshapen, and nodosities, rims, tips, ridges, and
stalactiform growths of new bone may be felt on them.[220]

[Footnote 220: Figs. 12 to 18 and 22 in Adams's _Treatise on Rheumatic
Gout_ are nice illustrations of these deformities.]

The general condition in this chronic form varies in different
individuals, and there is no characteristic disturbance of the
functions, such as obtains in chronic gout. There is no elevation of
temperature, unless to a slight degree during an active crisis of the
disease; the tongue may be clean, the pulse tranquil, the appetite and
digestion satisfactory, and {83} the urine normal or perhaps pale and
of low density. Fuller, however, says that "more generally the
complexion is sallow and the skin sluggish, and evidence of mischief is
furnished by yellowishness of the conjunctivæ, constipation of the
bowels, a pale and unhealthy character of the dejections, excessive
flatulence after meals, turbidity of the urine, and fulness of the
pulse." My own experience hardly harmonizes with this, and I have seen
many persons suffering for years from the general and partial form in
the enjoyment of excellent general health. Should, however, the disease
develop in a person the subject of menorrhagia or other uterine
disorder, or of repeated child-bearing, or after prolonged mental
anxiety, some disturbance of the general health fairly referable to
such disturbing conditions may be certainly looked for. In the advanced
stages the prolonged suffering and confinement often induce anæmia,
dyspepsia, and failing health.

More numerous and exhaustive analyses of the perspiration, urine, and
blood in the disease are needed. There is no uniform condition of the
skin; general perspirations, chiefly at night, often obtain, but I know
of no authoritative report as to the chemical reaction of the sweat in
this disease; Garrod[221] and Charcot[222] vouch for an absence of uric
acid in the blood, while Marrot[223] found both this acid and the urea
below the normal quantity in the urine, although the acid increased
notably under baths of high temperature.

[Footnote 221: Reynolds's _Syst. Med._, i. 918.]

[Footnote 222: _Loc. cit._, p. 190.]

[Footnote 223: _Contribution à l'Étude des Rheum. Artic., Examen de
l'Urine et du Sang_, Paris, 1879, p. 42.]

Certain affections other than the articular have been occasionally
observed in persons suffering from rheumatoid arthritis, but many even
of those authors who regard the disease as a form of rheumatism speak
of these affections as coincidences, and not as essential
manifestations of the disease. Charcot and Besnier, however, maintain
the latter to be their true relation to the articular affection which
they regard as chronic rheumatism. The two authors just named allege
that all the visceral localizations that occur in acute articular
rheumatism may obtain in the nodular form, but that such localizations
are infinitely less frequent and serious than in the acute, subacute,
or simple chronic forms of articular rheumatism--that endo- and
pericarditis undoubtedly do occur in nodular rheumatism, and appear
especially where there is an exacerbation of the disease and where
there is some approach to the acute state.[224] As Charcot has adduced
these cardiac affections in proof of the rheumatic nature of rheumatoid
arthritis, it is deserving of mention that he had personally met with
but two instances of endocarditis and five of pericarditis, four of the
latter having been discovered not during life, but in nine autopsies,
and that he cites only eight other cases of endo- or pericarditis which
had been either published or reported to him. He admits too that there
had generally been in these cases, at some former period, an attack of
acute rheumatism. Besnier, Homolle, Malherbe, Vidal, and Colombel, in
their articles upon the disease under consideration, do not cite a
single case in which they have seen cardiac disease in rheumatoid
arthritis. On the other hand, McLeod, Garrod, Fuller, Flint, Senator,
and Pye-Smith either deny or ignore the occurrence of cardiac disease
as a manifestation or complication of this disease. My personal {84}
experience coincides with that of those authorities last cited, except
in one instance, and that is open to the objection that the patient's
father had had acute articular rheumatism, the mother was the subject
of chronic deforming arthritis, and the patient had experienced during
many winters an affection which began in the smaller joints and
permanently damaged them; when first seen by me he had chronic disease
of the aortic valves. He may have had true articular rheumatism as well
as rheumatoid arthritis. His father had experienced the one, his mother
the other. If those instances be excluded in which a former attack of
acute rheumatism might be adduced in explanation of the supervention of
cardiac disease, but few cases will remain to suggest that rheumatoid
arthritis may develop endo- or pericarditis; and when it is borne in
mind that in several ways the cardiac affections may have arisen as
mere coincidences of the rheumatoid affection, it is well to wait for
further evidence before accepting as proved the occurrence of cardiac
affections as local manifestations of rheumatoid arthritis. Garrod's
observation is still pertinent: "The form of the disease in which acute
cardiac inflammation has occurred may be rather that of true articular
rheumatism of a very subacute character."

[Footnote 224: _Loc. cit._, 172-175; Besnier, _loc. cit._, 699.]

Nor is the evidence at all satisfactory in favor of any special
tendency to the following affections, much less of their being local
manifestations of rheumatoid arthritis: viz. pleuritis (McLeod,
Fuller), asthma (Charcot), chronic laryngitis (Garrod), grave cerebral
or spinal disturbances (McLeod, Fuller, Vidal), paralysis agitans,
locomotor ataxia, sciatica, trifacial neuralgia, and albuminous
nephritis.[225]

[Footnote 225: To mention only some of the many sources of cardiac
disease other than rheumatism may be adduced scarlet and other fevers,
extension of inflammation from the pleura or lung and other sources of
local irritation, powerful or oft-repeated muscular efforts, Bright's
disease, senile degeneration, etc.]

Among the more frequent complications may be mentioned migraine,
certain cutaneous affections, more especially psoriasis, prurigo,
lichen, and some diseases of the eye, chiefly iritis, which is apt to
be relapsing, and sometimes episcleritis. It is remarkable that iritis
very seldom occurs as a complication of acute articular rheumatism.

The so-called rheumatic nodules occur also in chronic rheumatoid
arthritis. It is not yet established that they are peculiar to
rheumatism and to rheumatoid arthritis. Dr. Stephen Mackenzie has seen
them in one instance in tertiary syphilis, the patient not having had
arthritis, rheumatism, or chorea.

2d. The partial or oligo-articular form of rheumatoid arthritis, like
the general or polyarticular variety, is usually a primarily chronic
affection, insidious in its invasion and slow in its progress. It is
chiefly observed in old persons, especially men (senile arthritis),
affects frequently a single joint, and chiefly the hip, but
occasionally the knee, shoulder, or spinal column, either as a
consequence of special injury or of the wear and tear of life, or
exposure to cold and wet, or even of what seemed to be simple acute or
subacute articular rheumatism or gonorrhoeal rheumatism. When not the
result of injury, two or three joints may suffer, both hips or knees,
or hip and some of the vertebræ, hip, knee, and ankle of the same limb,
and so on. Even in those cases in which the disease for a long time is
confined to a single joint and may have been caused by an injury,[226]
other joints, finally, are apt to become {85} affected, often in a
symmetrical order. So that it may seem almost general, or at least
polyarticular, just as the converse sometimes happens in the general
rheumatoid arthritis of long standing, where the disease becomes
greatly aggravated in one articulation and produces great deformity and
destruction of it, the others remaining as they were.

[Footnote 226: See Ord's case, II., _Brit. Med. Journal_, i., 1880,
158.]

The symptoms of this partial chronic form are very much those of the
general form already described, but there is usually in the early
stages less heat, tenderness, and swelling of the affected joint; the
pain is less acute, but more abiding, and, with the exception of more
or less stiffness or impeded movement in the joint, it may be the only
sign of disease present, so that at this stage of the affection it may
be taken for simple chronic articular rheumatism. But the disease
persists; the voluntary movements become more painful and difficult;
slight exercise of the joint is followed promptly by fatigue and
aggravation of the pain, and yet the articular surfaces may be pressed
together, and flexion and extension be practised, without causing much
suffering. Slowly and continuously alterations take place in the
affected articulation; with but little heat or redness it enlarges
steadily, the soft parts becoming infiltrated and thickened, or
effusion taking place into the capsule; the articular surfaces become
irregularly depressed by the growth of osteo-cartilaginous rings,
osseous nodosities, and stalactiform processes upon them, and these
irregularities, together with one or several loose bodies, may be felt
in the joint. The enlargement of the articulation becomes more
apparent, owing to the wasting of the muscles of the limb; its
movements become more and more restricted and difficult, although
perhaps not more painful, and are attended with creakings and gratings
perceptible to the ear and hand; and at last nearly all movement of the
joint may be prevented by the alterations in the shape of the
epiphyses, or by the interlocking of the osseous outgrowths, or in rare
cases by actual union of the bones. This form constitutes, par
excellence, arthritis deformans. In many instances there is little
effusion throughout the process, notwithstanding the grave deformity in
progress; hence the term dry arthritis. Even the partial form is
sometimes more active in its invasion, as when it very rarely succeeds
acute or subacute articular or gonorrhoeal rheumatism, or, more
frequently, follows an injury.

The duration of the partial form is usually very protracted; it may be
ten or twenty years. Exacerbations of the disease occur from time to
time, in the intervals of which the patient may be free from pain,
although the affected joints are seriously crippled.

The affection is not in itself fatal; the patient may attain an
advanced age and die of some intercurrent disease, such as dysentery,
pneumonia, cerebral hemorrhage, or other affection incident to old age.

A description of the features presented by partial rheumatoid arthritis
affecting the hip (morbus coxa senilis), the shoulder, and other joints
rather appertains to works on surgery, and only a glance at the
evidences of the disease in the vertebral column (spondylitis
deformans) will here be given. When the cervical vertebræ are
implicated the power of rotating the head from side to side is usually
preserved and is attended with a crackling noise, while the rest of the
cervical region is stiff and the head cannot be bent forward; when the
dorsal or lumbar vertebræ suffer the back becomes bent, the patient
stoops greatly and cannot stand {86} erect, and his body is shortened
and more or less twisted. A careful examination will discover not only
the great rigidity of the spine, and as it were fusion en masse of its
joints, but in these persons the bony outgrowths may be felt.
Occasionally the alteration in the vertebræ by compressing the cord or
its membranes, or the spinal nerves and ganglia, may produce neuralgic
pains in the cervical, dorsal, lumbar, or sciatic nerves, wasting of
the muscles, more or less paralysis, and even vasomotor disturbances.

3d. Heberden's nodosities are certainly sometimes the effect of
rheumatoid arthritis, implicating chiefly, often solely, the distal
joints of the fingers, where it slowly forms two little hard nodules
about the size of dried peas upon the side of the articulations. These
are notably enlarged and their movements impaired, but pain is seldom
experienced, and were it not for deviation of the end of the finger to
one side or the knob-like excrescences upon the joints--appearances
which much disfigure the hand--patients would not speak of the
affection. In many cases these alterations likewise involve, but in a
minimum degree, the first phalangeal articulations, and less frequently
the metacarpo-phalangeal, and even some of the larger joints--the
wrist, knee, or hip, etc. Like the other varieties of rheumatoid
arthritis, this form occasionally has a more active invasion than is
above mentioned, and may be attended by local pain, heat, and redness,
or such symptoms may occur as exacerbations of the chronic disease.

Gout may precede these nodosities, or, as in the case of
Charcot's,[227] the latter may precede the former by several years.
Finally, Charcot remarks that Heberden's nodosities are "often
accompanied by asthma, migraine, neuralgia, especially of the sciatic
nerve, and muscular rheumatism, and that these manifestations may
alternate with the exacerbations of the disease."

[Footnote 227: _Loc. cit._, 198.]

MORBID ANATOMY.--Every component tissue of the articulations exhibits
signs of a chronic inflammatory process. In the chronic form affecting
the larger joints the synovial membrane is found more or less
congested, opaque, and thickened; at the point of its reflection upon
the bones its fringes are thickened and injected and their villosities
greatly increased in number, length, and thickness, and in extreme
instances have been aptly compared to the wool on a sheep's back. The
cartilage-cells normally existing in the synovial fringes likewise
proliferate and develop into cartilaginous growths, many of which
become infiltrated with lime salts, or even ossified, and in this way
originate some of the foreign bodies, pedunculated or sessile, which
are found in the joints. These may be attached to the synovial fringes,
or imbedded in the membrane itself, or set free by rupture of their
pedicles. In some examples these neoplasms resemble in size small
melon-seeds; in others they form irregular masses, many of which are as
large as hazel-nuts.

At the outset there is frequently an increase of synovial fluid, richer
in mucine than natural, which lessens considerably in the later stages
and becomes a turbid, viscid fluid of a dirty white or reddish-yellow
color, containing no pus, but degenerating epithelium and fragments of
villosities and cartilage. In many cases, more especially of the
partial {87} form, very little effusion into the articulations takes
place (arthrite sèche).

The inflammatory irritation excites proliferating and degenerating
processes in the cells and basis-substance of the cartilage covering
the bones, and the changes described in connection with acute rheumatic
arthritis ensue. Those parts of the cartilage covering the bones which
suffer pressure in locomotion fibrillate on their surface, and either
undergo mucous degeneration, resulting in ulceration and complete
absorption, or are thinned and worn away by attrition. In either way
the ends of the bones become laid bare. Those portions of the cartilage
at the periphery of the joints which escape compression in the erect
posture likewise proliferate, but, according to Cornil and Ranvier, in
consequence of being covered by the synovial membrane the proliferating
elements are retained in situ, instead of escaping into the articular
cavity, and develop into actual cartilage, and may ultimately ossify.
In this way irregular masses of cartilage (enchondromata) and bone
(osteophytes) form around the heads of the bones, enlarging them
considerably, altering their shape, encroaching upon the articular
cavity as well as extending up the shafts of the bones, and displacing
the capsules of the articulations. Similar productions of cartilage
sometimes form in the thickened capsules and ligaments, especially in
very protracted cases, or these parts become infiltrated with lime
salts.

While these processes are going on at the periphery and the centre of
the cartilages, in its deeper layers the proliferating cells are
undergoing ossification and rendering the ends of the bones very dense
and compact, so that under the attrition to which they are exposed by
the articular movements they acquire the smoothness, polish, and white
aspect of ivory (eburnated). It is probable that the articular ends of
the bones participate in this proliferation and development of bone,
which increases their compactness and is followed by eburnation. That
the bone itself does sometimes play a part in the hyperostosis which is
in progress is shown by an increase of an inch in the length of the
right ramus of the maxilla over that of the left in Adams's first
plate.[228] Forster's[229] and Ziegler's[230] later investigations
confirm this view. Nor is the periosteum exempted from the
proliferating process which may have long existed in the several
articular tissues, as is shown by the considerable enlargement of the
diameter of the shaft of the long bones and by the osteophytes which
form on the exterior of the vertebræ and often unite several of them
together by a series of osseous splints, interfering with the mobility
of the spine. Notwithstanding this development of cartilage and bone
upon the exterior of the articular extremities, the interior,
especially in old people or in very chronic examples of the general
form of the disease, or rarely in the partial form, undergoes
degeneration and atrophy. The spongy substance becomes rarefied,
thinned, and friable (osteoporosis), so that it has been easily cut or
crushed, and it is frequently loaded with fat. True ankylosis of the
diseased joints is rare, except in the very small articulations when
kept at rest; even under this condition fibrous ankylosis is not of
frequent occurrence.

[Footnote 228: _Illustrations of the Effects of Rheumatic Gout_,
London, 1857.]

[Footnote 229: Forster, _Handbuch der Path. Anat._, p. 1000.]

[Footnote 230: _Virchow's Archiv_, 1877.]

Finally, the interarticular fibro-cartilages and ligaments and the long
{88} tendon of the biceps degenerate and are absorbed. The muscles in
protracted cases suffer simple atrophy, but are sometimes the seat of
an interstitial accumulation of fat. Thus far, no lesions of the nerves
supplying the diseased joints nor of the spinal cord have been
discovered.

ETIOLOGY.--The causation of rheumatoid arthritis is involved in much
obscurity--in part, because sufficient attention has not been paid to
its clinical varieties. We will examine first the general progressive
form which is the more common.

In women it prevails during the child-bearing period. It is probably
oftenest developed between twenty and thirty, and continues to occur
frequently up to the period of the menopause, fifty, after which it
develops comparatively seldom. Of Ord's 33 cases, 10 were between
twenty and thirty years; 11 between thirty and forty; 9 between forty
and fifty; and 3 between fifty and sixty.[231] Children are not exempt.
E. C. Seguin saw three children of the same family suffering from the
disease at ages from two and a half to four years.[232] Moncorvo[233]
met with an example at two years and a half, Laborde at four, and
Charcot at ten. It occasionally begins in both sexes after sixty.

[Footnote 231: _Brit. Med. Jour._, 1880, 156.]

[Footnote 232: _The Med. Record_, London, 1877, 797.]

[Footnote 233: _Du Rheumatisme Chronique Noueux des Enfans_, Paris,
1880.]

It is pre-eminently a disease of females up at least to fifty; after
that it is not infrequent in men, and is then often only partial, at
least at first. The most frequent progressive form, however, does often
occur even in boys.

It is probably more frequently observed in cold and damp climates than
in those of opposite qualities, for cold is regarded as its most common
cause. However, it is met with in India and other hot climates. Besnier
asserts it is almost unknown in the tropics, but new investigations are
needed on this point.

Direct hereditary predisposition exercises but little influence,
according to Garrod, and we certainly often see the disease confined to
a single member of a large family, although Seguin saw three young
children of one family affected with it, their parents being free from
any disease. Trastour three times saw the children of women who were
afflicted with nodular rheumatism already suffering from articular
rheumatism; and Charcot once saw the grandmother, the mother, and the
granddaughter successively attacked. At present I have a patient whose
mother at fifty-five and maternal grandmother at sixty became subjects
of a crippling polyarticular affection; another of my patients informed
me that his mother and a young sister were like himself victims of the
disease. This direct transmission appears to be rare, judging from my
own experience and from the few instances of it mentioned by writers.
But very many authorities maintain that simple acute and chronic
rheumatism and gout in the parents predispose to rheumatoid arthritis
in the offspring (Charcot, Trastour, Besnier). Now, the facts given in
support of this opinion are not numerous. Trastour found that out of 45
cases of nodular rheumatism the father or mother were rheumatic in 10
instances, but the form of the rheumatic affection is not stated.
Charcot, Besnier, and Homolle, although believers in the doctrine, do
not cite an example in proof. However, in Pye-Smith's 27 cases of
osteo-arthritis, five stated that rheumatism had occurred in their
families. Thus, two fathers {89} had had rheumatic fever, and one was
rheumatic, and two sisters of different families had had rheumatic
fever. Besides, the father of a sixth and the grandmother of a seventh
had had gout.[234]

[Footnote 234: _Guy's Hospital Reports_, 3d Series, xix. 348.]

The evidence in favor of the doctrine that true articular rheumatism
transmits an hereditary tendency to rheumatoid arthritis does not
appear to be conclusive, although it is highly thought of by those who
regard the latter disease as a variety of rheumatism. Some
considerations of an opposing character deserve mention. Acute
articular rheumatism has very rarely passed continuously into
rheumatoid arthritis, and very rarely has been followed at short
interval by that disease; and in such exceptional cases the antecedent
affection may have been really the acute form of rheumatoid arthritis,
which closely resembles acute articular rheumatism. Trastour,[235]
Vidal,[236] Charcot,[237] and others admit that acute rheumatism can
hardly be placed amongst the antecedents of the rheumatoid affection.
Garrod[238] with some others states that now and then acute rheumatism
acts as an exciting cause of it, which appears to have been Fuller's
view;[239] he had repeatedly known it to commence apparently as a
sequel of acute rheumatism. However, Ord met with a case in which the
lesions of rheumatoid arthritis were present in a typical form in a
patient who had mitral disease as a result of acute rheumatism, the
arthritis having begun as a continuation of the acute attack.[240]

[Footnote 235: _Thèse de Paris_, 1853, p. 41.]

[Footnote 236: _Ibid._, 1855, p. 9.]

[Footnote 237: _Leçons Cliniques_, p. 214.]

[Footnote 238: Reynolds's _Syst. Med._, 1870, i. 920.]

[Footnote 239: _Lib. cit._, 333.]

[Footnote 240: _Brit. Med. Jour._, 1880, i., 158.]

That so common an affection as articular rheumatism should occur in the
family or personal history of a patient the subject of the rheumatoid
arthritis is not improbable; nasal catarrh and many other very common
diseases must be frequent antecedents of the rheumatoid affection, yet
are not causes of it. Much the same remarks apply to the view that gout
in the parents may transmit a tendency to rheumatoid arthritis in the
offspring. The experience of English physicians in this matter is
hardly reliable, owing to the great prevalence of gout in England. In
Canada and many parts of the United States, however, while gout is a
rare disease, rheumatoid arthritis is a common one, and the writer has
not found an intimate relationship to obtain between the two
affections. It is not intended to deny that when the children of
rheumatic or gouty parents fail in health owing to their inherited
constitutional disease, they become liable to rheumatoid arthritis, for
feeble health predisposes to that affection.

Finally, many of the difficulties connected with this subject are
reasonably met by Hutchinson's[241] doctrine that there exists a state
of tissue-health which is transmissible by inheritance, which involves
liability to inflammations of joints and fibrous structures, and upon
this arthritic diathesis as a foundation may be built up, under the
influence of special causes, a tendency to gout, rheumatism, or any one
of their various modifications or combinations.

[Footnote 241: _Trans. International Med. Congress_, ii. 95; Guéneau de
Mussy's chap., "De la Diathèse Arthritique," _Clin. Méd._, 1874, t. i.
317-338.]

Hutchinson has demonstrated that gout is often followed by rheumatoid
arthritis, the lesions characteristic of both affections coexisting in
the same joint. Charcot and Cornil had previously observed the same
{90} thing.[242] Acute and perhaps chronic rheumarthritis have
sometimes preceded rheumatoid arthritis. If a predisposition, inherited
or acquired, to rheumatoid arthritis exist, the occurrence of gouty or
rheumatic irritation in the joints may suffice to induce the peculiar
form of disturbance characteristic of the rheumatoid affection, just as
injuries sometimes develop the partial form.

[Footnote 242: _Mémoires de la Société de Biologie_, 1864.]

There is a group of conditions affecting the sexual functions and
organs of women which appear to be specially connected with the general
peripheral form of rheumatoid arthritis. The disease follows pregnancy,
and specially frequent pregnancies, protracted lactation, and various
disorders of menstruation. The latter influence obtained in ten out of
eleven instances of the disease met with in girls under eighteen by
Fuller.[243] The frequency of the disease about the period of the
menopause has been already mentioned. Todd noticed its coincidence with
dysmenorrhoea. Ord in an able and original paper[244] has lately dwelt
upon ovario-uterine disorder or irritation as a frequent active cause
of the disease, having in his opinion met with 33 instances of the
kind. The relationship between these various conditions of the
functions and organs of generation and rheumatoid arthritis cannot be
regarded as settled. Garrod supposed that such conditions, by causing
debility, predisposed to the articular disease. Todd, an ardent
humoralist, held the nexus between the two to be unhealthy secretions
of the uterus, leading to blood impurity; while Ord has ably defended
Remak's view that a direct influence of the nervous system is the real
link of relationship. It seems necessary to remark that mere
coincidence may play a large rôle in the explanation of many of these
cases. In 17 at least of Ord's 33 cases the conditions stated by that
author cannot safely be adduced as anything more; and it is probable
that they would be found present in much the same proportion in any
other chronic painful disease of women.

[Footnote 243: _Loc. cit._, 335.]

[Footnote 244: _Brit. Med. Jour._, i., 1880, 151-153.]

Scrofula and phthisis are regarded by Charcot, Cornil, and Garrod as
frequent antecedents of rheumatoid arthritis: the first had several
times seen white swelling in youth, followed by nodular rheumatism in
later life;[245] and Fuller found that 23 out of 119 victims of
rheumatic gout had lost a parent or one or more brothers and sisters by
consumption.[246] Chlorosis has several times preceded rheumatoid
arthritis. When the prevalence of scrofula, phthisis, and chlorosis is
borne in mind, it will not appear strange that they should frequently
be found amongst the antecedents of rheumatoid arthritis, without
inferring any other relationship between them. Gonorrhoeal rheumatism
has also occasionally preceded rheumatoid arthritis, but Ord and
Hutchinson are probably correct in regarding that affection as a
variety of rheumatoid arthritis.[247]

[Footnote 245: _Loc. cit._, p. 208, foot-note.]

[Footnote 246: _Loc. cit._, p. 334, foot-note.]

[Footnote 247: _Trans. International Med. Congress_, vol. ii. p. 92;
_Brit. Med. Jour._, 1881, p. 158.]

Cold, especially when prolonged and associated with dampness, is
commonly held to be the most common cause of general rheumatoid
arthritis. A protracted residence in low, damp dwellings, deprived of
the sun's rays and of a free circulation of air, is a condition thought
most favorable to the provocation of this disease, perhaps years after
the condition has been done away with.

{91} Poverty and all that it implies are at least frequent antecedents
of the disease (hence one of its epithets, arthritis pauperum), as are
other debilitating influences, such as night-watching, insufficient
food, mental worry, grief, anxiety, etc. Be it remembered, however,
that the disease is frequently observed in the well-to-do, who live in
dry climates and warm houses, are well fed, and want for nothing; so
that the external conditions first mentioned are not essential causes
of the disease, and many of them may act merely as adjuvants.

Direct injury of a joint from a blow, a fracture, a whitlow, etc. may
sometimes induce a local rheumatoid arthritis, which may subsequently
become multiple and involve several articulations more or less
symmetrically.[248]

[Footnote 248: Vide Charcot's and Ord's cases, _loc. cit._]

The partial form presents some peculiarities of causation--thus: it
occurs chiefly in advanced life (senile arthritis), much less
frequently in middle life, very exceptionally in the very young. Men
are much more liable to it than women. It is chiefly this variety which
follows injuries, blows, dislocations, pressure, etc., and the disease
may then be limited to the injured joint and be monoarticular, or it
may extend and become polyarticular, or rarely, as in Ord's case, even
general. This monoarticular form appears to be sometimes induced by
other local irritations of the articular structures than those
following traumatic influences; and as foreign growths in joints and
gouty irritation may respectively induce the lesions indicative of
rheumatoid arthritis, so, it is probable, may simple chronic
rheumatism; and this may be the true relationship existing between
these several affections. It is doubtful at present whether purely
local irritation or injury of a joint can originate the alterations
belonging to rheumatoid arthritis--that is, in the absence of all
predisposition to that disease or of the arthritic diathesis. Cold and
dampness are generally admitted to be causes of the partial form, but
the evidence on this point is not altogether satisfactory. It may be
that chronic articular rheumatism is induced by the prolonged operation
of damp cold, and that the prolonged rheumatic irritation, aggravated
by constant use of the joint and by occasional violence, ultimately
superinduces the profounder alterations characteristic of arthritis
deformans. It appears highly probable that if the predisposition exist,
any long-abiding irritation of a joint, whether the result of violence
or disease, may ultimately originate the alterations of the cartilages
and bones which obtain in rheumatoid arthritis.

As regards the etiology of Heberden's nodosities, and their relation to
other affections of the joints, the following summary must suffice:
They obtain chiefly in advanced life, but do occur rarely in the young;
they are probably somewhat more frequent in women than in men; although
more frequently seen in the upper classes, the poor are not exempt from
them, no doubt because they are specially exposed to slight but
oft-recurring injuries of their digits, such traumatism being an
exciting cause of the disease, especially when confined to a single
joint. The affection is sometimes hereditary; both it and the general
or the partial forms of rheumatoid arthritis may coexist in the same
family and even in the same person. The alterations in the joints are
identical with those found in the general variety of rheumatoid
arthritis, and exist without {92} deposits of urate of soda (Charcot).
It resembles the general form of the disease just mentioned in its
tendency to involve many symmetrical articulations at the same time,
and the partial form in the rarity with which it extends beyond the
joints first attacked. While Heberden's nodosities, as Haygarth taught,
do occur independently of gout and the gouty habit, I believe with
Begbie[249] and Duckworth[250] that in some persons they are evidences
of gout or the gouty diathesis.

[Footnote 249: _Contributions to Practical Med._, 1802, p. 29.]

[Footnote 250: "On Unequivocal Gouty Diseases," _St. Bartholomew's
Hospital Reports_, vol. xvi., 1880, p. 190.]

Quite recently a woman aged forty-eight consulted me with these
nodosities beginning upon the last joint of the fingers, while she was
the subject of vesico-renal irritation and was passing free uric acid
in the urine. Hutchinson has twice seen them in combination with a
peculiar insidious and painless inflammation of the iris and vitreous
body, which occurs in the children of the gouty, yet such children have
no deposits of lithates in their joints, nor any lithiasis, nor acute
paroxysms of true gout, and he considers that "the last joint arthritis
is to be regarded as in part gouty, and in part a kind of articular
chilblain."[251]

[Footnote 251: _Trans. International Med. Cong._, ii. p. 96.]

Lastly, in some instances they are no doubt the hybrid offspring of an
inherited tendency to both gout and rheumatoid arthritis.

No more important principle in pathology exists than has been of late
years insisted upon, especially by Jonathan Hutchinson and in his
recent lecture by Sir James Paget[252]--to wit, that "by inherited
dispositions, accumulating and combining or converging in definite
proportions, new diseases may be developed and old ones be variously
modified."

[Footnote 252: _Lancet_, ii., 1882, 1017-1021.]

The pathogenesis of rheumatoid arthritis is the subject of differences
of opinion very like those existing in regard to acute articular
rheumatism. The weight of evidence is in favor of its diathetic
relationship to rheumatism; and the doctrine of an arthritic diathesis
and of the operation of the causes of the disease through the nervous
system appears to be specially applicable to it, with less difficulty
than to acute rheumatism, and the probability of a specific germ being
its true cause is very remote. What seems to be necessary in addition
to the preceding is, that the causes shall be more persisting and
oft-recurring, so as to maintain a prolonged local irritation of the
articular tissues, or that the neuro-arthritic diathesis shall be
highly developed. Under these conditions the prolonged or oft-repeated
application of cold and damp to the peripheral nerves, severe or
oft-repeated slight injuries to joints, urethral or ovario-uterine
irritation, chronic gout or rheumatism, or even, exceptionally, an
attack of the acute form of these diseases, may originate rheumatoid
arthritis; and all wearing influences, such as anæmia, excessive
menstruation, prolonged lactation, innutrition, failing health, mental
anxiety, or shock, etc., act as adjuvants in the development,
aggravation, and maintenance of the articular disease.

DIAGNOSIS.--It is perhaps not possible to distinguish with certainty
either the acute or the chronic form of rheumatoid arthritis from
subacute or chronic rheumarthritis respectively before the
characteristic deformities of the former affections have appeared.
Acute rheumatoid arthritis, which is comparatively rare, may be said to
exist, rather than subacute {93} articular rheumatism, if the disease
affect early and chiefly the smaller joints of the hands and feet alone
or along with some of the larger articulations, especially the
sterno-clavicular or the temporo-maxillary; if the effusion into the
joints be abundant; if inflammation persist in the articulations first
involved, notwithstanding the invasion of other joints; if the heart
escape; if the patient be a female who is constitutionally delicate, or
has borne children rapidly, or is the subject of disordered
menstruation, or has been attacked soon after childbirth or during
lactation;--finally, if, on cessation of the attack, one or more of the
joints remain swollen and permanently enlarged and impaired in
function. The coexistence of iritis, or a history of a previous attack
of that disease not attributable to syphilis or gout, would strengthen
the above view.

Precisely the same considerations serve to distinguish chronic general
or polyarticular rheumatoid arthritis from chronic articular
rheumatism, with the following qualifications: endo- or pericarditis is
not of frequent occurrence in chronic rheumatism, so that this
distinction is not available, and chronic rheumarthritis of long
standing does sometimes impair the movements of the joints, and even
produce slight alterations in them. However, it does not, as a rule,
involve so many joints as rheumatoid arthritis; it is less symmetrical
in its distribution, and much less prone to implicate the
sterno-clavicular, the temporo-maxillary, or the vertebral
articulations. Nor does it cause removal of the articular cartilage,
enlargement of the heads of the bones, and the formation of osteophytes
around them, and of loose bodies in the articulations, together with
marked deformities and luxations of the joints. A history of a remote
or recent attack of acute rheumarthritis or of chorea, or the presence
of chronic valvular disease, would strongly indicate the simple
rheumatic nature of the case.

The partial form of rheumatoid arthritis can with even less certainty
than the general be distinguished from chronic articular rheumatism
before the characteristic alterations of the joints have been
developed, more especially as it is sometimes a consequence of gouty
irritation and probably of chronic rheumatism. Chronic arthritis
following a traumatic cause, and persisting obstinately in the injured
joint is probably rheumatoid, if not strumous, gouty, or periarthritic.

But before definitely deciding it will be prudent to await the
development of some of the characteristic alterations of structure
appertaining to rheumatoid arthritis. An affection of the shoulder
frequently occurs which resembles in many respects rheumatoid
arthritis, and has been well described by Simon Duplay[253] and W.
Pepper.[254] It usually follows an injury, such as contusion, sprain,
etc., of the joint, but may be spontaneous; it is unattended by
swelling or deformity. Its early symptoms are pain on pressure of the
shoulder a little below the outer border of the acromion, and
especially behind it and at the coracoid process, also about the
insertion of the deltoid and below the acromion during movements of the
joint, especially when the arm is raised from the side or rotated
inwardly; early restriction of these movements, which increases till a
fibrous ankylosis becomes established and scapula and humerus move
together as one piece, motion between those bones no longer existing,
and forcible attempts to produce it giving great pain, and sometimes
producing {94} crepitus in or about the articulation; sometimes early
numbness and pain down the member to the hand in the course of the
ulnar, internal cutaneous, or the radial nerve; vicious and painful
semiflexion of the elbow; after a time wasting of the group of muscles
which move the shoulder-joint. Although usually monoarticular and of
traumatic origin, I have seen it affect first one and then the other
shoulder in the absence of any known injury, and beginning like a
neuritis or a neuralgia of the scapulo-humeral nerves. Duplay, however,
regards it as a periarthritis. It may be distinguished from the
rheumatoid arthritis by the absence of effusion into or enlargement of
the articulation, and of deformity of the bones; by the early
restriction of the movements and the rapid development of adhesions
which fix the articulation; and by the curability of the disease.

[Footnote 253: _Archives Générales de Méd._, Nov., 1872, pp. 512-542.]

[Footnote 254: _Archives of Med._, Oct., 1880.]

The articular affection of locomotor ataxia sometimes closely resembles
monoarticular rheumatoid arthritis,[255] but may be distinguished by
its sudden invasion, often without pain or fever; the prompt
development of a general and often enormous tumefaction of the entire
member, with copious effusion into the joint; the early destruction of
the articular cartilages, the rapid wearing away of the heads of the
bones, and the proneness to spontaneous fracture of their brittle
shafts; the prompt absorption of the articular effusion, followed by a
relaxed state of the ligaments and a facility of dislocation; the early
occurrence of the articular affection, when motor inco-ordination is
scarcely developed, and its frequent association with the crises of
ataxia or the presence of some of the other symptoms of that disease.
The importance of these facts will be especially evident in those
examples of ataxic articular disease in which, at an advanced stage,
eburnation and deformity of the articular surfaces, with the formation
of loose bodies and osteophytes, are observed, just as they are in
arthritis deformans.

[Footnote 255: Charcot's _Lectures on Diseases of the Nervous System_,
Syd. Soc., 1877; _Archives de Physiologie_, t. i., p. 161, 1868;
_ibid._, xi., 1869.]

Articular disease closely allied to what occurs in locomotor ataxia is
now and then observed in the early stages of progressive muscular
atrophy,[256] but while the large joints, more particularly the knee
and the shoulder, suffer in the former affection, the phalangeal
chiefly and the larger articulations more rarely are attacked in the
latter. Of course the peculiar symptoms of progressive muscular atrophy
coexisting with those of the articular affection would serve to
distinguish the latter from rheumatoid arthritis.

[Footnote 256: Remak, _Allgem. Med. Central. Zeitung_, March, 1862;
Rosenthal, _Clinical Treatise on Diseases of the Nervous System_,
translated by L. Putzel, M.D., 1879, p. 286.]

It is often very difficult to say whether a given case is one of
chronic rheumatoid arthritis or of chronic gout; and there is no doubt
that in England, where gout prevails, it is not unfrequently associated
with rheumatoid arthritis, sometimes preceding and even causing it,
much more often following it, for the one does not exclude the other.

While rheumatoid arthritis most frequently begins in the hand, and is
usually symmetrical and bilateral, gout commonly begins in the lower
extremities, and especially in the metatarsal joint of the great toe,
and of one foot only. Chronic gout is far more frequently preceded by
attacks of acute gout than chronic rheumatoid arthritis is by the acute
form of that affection; a history of inherited predisposition, of
indulgence in the {95} use of wine, ale, porter, and of animal food, of
deficient bodily exercise, with perhaps great mental occupation or
anxiety, of recurring gouty dyspepsia or of a tendency to lithiasis,
would indicate gout, while the absence of these and a history of
frequent exposure to cold and wet, of injury to the joint, of previous
exhausting disease or drain, of impaired health, debility, or poverty,
would strongly imply rheumatoid arthritis. Gout is especially observed
in males over thirty, and very rarely in children; general rheumatoid
arthritis is chiefly a disease of females during menstrual life, and
occasionally occurs in children of either sex.

The partial form is, like gout, chiefly a disease of men, but occurs
generally at a more advanced age than gout. Even chronic gout is more
or less paroxysmal, with distinct intermissions; chronic rheumatoid
arthritis is more or less abiding and progressive, with only remissions
in its course and severity; the former is frequently associated with
chronic renal disease, the latter is not. The urate-of-soda deposits
about the articulations in gout appear as more or less round or ovoid
swellings in the close vicinity of the joints, but not observing their
exact level or their general form; softish when recent, they never
acquire a bony hardness, and are nearly always capable of slight
lateral movement. The skin covering them is frequently stretched and
glossy, and may exhibit white spots of urate of soda. The articular
nodosities in chronic rheumatoid arthritis are actual osseous
enlargements of, or outgrowths from, the articular surfaces, forming
part of them, immovable and conserving more or less their form. The
integument covering the nodosities is not glossy or dotted with
chalk-like specks. The several types of deformity of the fingers
previously described, and mainly produced in rheumatoid arthritis by
muscular contractions and altered shape of the articular surfaces, are
not seen in gout. Finally, if chalk-like concretions are visible in the
ears, joints, or finger-ends, or if the blood contain uric acid, gout
is present. While rheumatoid arthritis and chronic gout occasionally
coexist in the same patient in England, in Canada, where the latter
disease is comparatively rare and the former quite common, the writer
does not remember to have observed such coexistence.

Besides the acute syphilitic disease of the joints already alluded to
as occurring in children (inherited), a chronic arthritis is observed
in the adult amongst the very late lesions of syphilis. It is usually
monoarticular, affects the larger joints, especially the knee, and may
originate either in the synovial membrane or in the bone and
periosteum. In syphilitic synovitis the history of the case, the
existence occasionally of soft gummy tumors in the periarticular
tissues and of hydrarthrosis, the trivial degree of pain and
tenderness, the insidious invasion and chronic course of the affection,
and its prompt relief by antisyphilitic remedies, will indicate the
nature of the case.

When it originates in the bone and periosteum, although the invasion
may be prompt and the pain at first severe, the latter usually
moderates greatly and becomes nocturnal, and the articular surfaces
present localized rather than general enlargement (hyperostosis); nodes
often coexist; effusion is moderate, unless the synovial membrane is
also involved, and full doses of iodide of potassium will soon afford
relief.

PROGNOSIS.--In the polyarticular form the course varies much more than
is commonly believed, and the disease must not be regarded as
necessarily {96} progressive and incurable. When it occurs in young
persons, and in children more especially, although it may suffer
exacerbations and remissions for a few years, yet arrest of the disease
and recovery of the functions of the joints, sometimes with very little
deformity, now and then take place under suitable management. Quite
recently a man of thirty-two consulted me about a vesical affection who
from the age of eight had suffered every winter for twenty years from
rheumatoid arthritis in his hands and feet, and finally in the knees.
Yet when seen by me he had been free from pain in his joints for three
years, and, although they were somewhat deformed, their movements were
remarkably free and painless.

Several of my younger patients while bearing children rapidly and
nursing them have had the disease in their hands or hands and wrists;
exacerbations have recurred during subsequent lactations, and yet the
disease has either become arrested or progressed very slowly and at
long intervals. It is admitted, however, that these are all exceptional
cases, and that the tendency both of polyarticular and of the
monoarticular forms is to progress, and, either steadily or at
intervals and by recurring attacks, to permanently deform the joints
and impair their movements. Even under these circumstances, however,
the patients may suffer little pain unless when forcible movements of
the articulations are attempted.

On the other hand, while the disease cannot be regarded as curable
under the employment of drugs, very much can frequently be done,
especially in the polyarticular form, to relieve the suffering and to
retard, if not arrest, the progress of the disease, and even to restore
sometimes very considerably the functions of the joints. Neither of
these forms of rheumatoid arthritis can be said to be dangerous to
life, and they often exist ten or twenty years and more without
seriously injuring the general health. Heberden's nodosities are
incurable, but they are little more than deformities.

TREATMENT.--The treatment of rheumatoid arthritis is, as a rule,
disappointing, and perhaps no affection requires more perseverence and
self-reliance on the part of the physician or more hopeful resolution
on that of the patient. Our first duty is to make an exhaustive search
as to the probable cause of the disease, as its removal is an important
step in the treatment of the affection, although such search is
frequently futile, and many of the alleged causes may, after all, be
mere antecedents or coincidences. However, inasmuch as the pathology of
the disease is very obscure, any abnormal condition of organ or
function that may be discovered should receive strict and prompt
attention, lest it should, either through disturbed innervation or
malassimilation or impaired nutrition or defective excretion, be the
predisposing or exciting cause of the disease. In women the most
careful inquiry should be made into the state of the ovario-uterine
organs and functions, and the least departure from their norm should be
at once treated. Deficient, excessive, or painful menstruation,
leucorrhoea, ovarian irritations, or pain, even displacements of the
uterus or ovary, should be corrected as soon as possible. Repeated
pregnancy and prolonged lactation, recurring mental anxiety and
physical fatigue, defects of diet, want of food, of sunlight, and of
good air, residence in damp dwellings, occupations involving exposure
to cold and wet, are conditions supplying important indications which
too often are {97} beyond the control of the physician, although they
peremptorily require his attention. The general form is often met with
in anæmic persons and in those of impaired health and vigor, and
probably very rarely occurs under opposite circumstances; and there is
a consensus of opinion that a lowering system of treatment is
contraindicated in rheumatoid arthritis.

Having efficiently set about correcting or removing these various
predisposing or determining causes of the disease, we next direct our
care to the disease itself. The remedies which had been found most
useful in rheumatoid arthritis before the introduction of salicylic
acid were cod-liver oil, quinia, iodine, iron, arsenic, and various
mineral waters, employed either externally or internally, usually in
both ways. Judging from my own late experience and from the results
obtained by Sée[257] and other French physicians, as communicated by
Jules Compagnon,[258] sodium salicylate, given in sufficient doses,
promises to be more generally useful in the more acute forms or in the
actively inflammatory periods and exacerbations of the disease than any
of those agents. Including Sée's cases, Compagnon has related 17
examples of rheumatoid arthritis, most of them of the general
progressive form, in which great improvement as regards pain,
stiffness, swelling, and even deformity, followed promptly the
employment of that salt, even after the failure of other remedies. It
proved signally useful recently in a rebellious chronic case of my own.
Pollock has lately published an instance in which 5 grains of
salicylate of quinia three times a day were in three or four days
followed by great relief.[259] The testimony already given of Dr. J. T.
Eskridge as to the great value of this salt in chronic rheumatism will
be held by some to be corroborative of its value in rheumatoid
arthritis. It is hardly necessary to say that it often fails in this
intractable disease, but it has frequently relieved the pain and
swelling and arrested the progress of it, at least for the time, even
when alkalies, iodine, arsenic, baths, etc. had failed.

[Footnote 257: _Bullétin de l'Académie de Méd._, Paris, t. v., 2d
Serie, 1877.]

[Footnote 258: _De l'Utilite du Salicylate de Soude dans le Traitement
du Rheumatisme_, par Jules Compagnon, Paris, 1880.]

[Footnote 259: _The Lancet_, ii., 1882, 141.]

It is probable that less than 45 grains per diem of the sodium salt is
of little value in even the most chronic forms, and that the quantity
requires to be increased in proportion as the febrile symptoms are
active, so that a drachm and a half or two drachms may need to be
administered in the day to some persons. It should be given in divided
doses at intervals of two hours, and, what is of primary importance, it
should be continued for a long time, even after much improvement has
resulted, and should be resorted to from time to time, especially
during recurrences of the pain, heat, or swelling. It is of
consequence, especially in elderly patients, to ascertain that the
medicine is being promptly eliminated by the kidneys and to watch its
effect upon the heart. The administration along with it of a little old
rye whiskey or brandy will sometimes be necessary in feeble people. In
those rather common cases in which the skin is inactive and perhaps
harsh the salicylate often improves that important organ of oxidation
and elimination, and should it not do so the addition of the ammonium
carbonate may be tried, especially in feeble persons with weak hearts.

{98} Moreover, the other drugs which sometimes prove serviceable in
this disease may be given at the same time or alternately with the
salicylate, or instead of it if it is not found to be of use or is not
tolerated. In chronic cases a prolonged course of cod-liver oil, alone
or along with malt extract, often seems to be of real service,
especially when nutrition is much impaired or when the patient is the
subject of acquired or inherited struma. Iodide of potassium, in
combination with quinia or other tonic, will often prove signally
useful in chronic cases unaccompanied by pyrexia, in which the pains
are worst at night. It should be first tried in moderate doses (5 to 8
grains), and be continued for a long time with occasional
intermissions, and before discarding it from disappointment--which
often arises--15- to 20-grain doses may be given tentatively for a
short period. Milk or coffee or Vichy water are good vehicles for its
administration. Whether free iodine in the form of the tincture, so
highly spoken of by Laségue,[260] acts as well or better than the
iodide of potassium is doubtful. He gave it at meals, in doses
progressively increased from 10 drops to 5 or 6 grammes twice a day, in
sherry or sweetened water, and persevered with it for a long period.
Garrod has had many restorations to health in severe forms of this
disease from the persevering employment of the syrup of the iodide of
iron. The iron in these preparations may deserve as much commendation
as the iodine, for it has often proved signally useful in this disease,
not alone on account of the anæmia which so frequently attends it, but
through its beneficial influence upon the nutritive functions and the
circulation.

[Footnote 260: _Arch. Gén. de Méd._, 1856.]

The usual rules regulating the employment of iron are to be observed,
and the condition of the digestive organs will demand special attention
during its employment. Although the influence of arsenic upon
rheumatoid arthritis is not uniform, yet as it sometimes proves really
useful[261] it should be tried. Like iron, it may prove beneficial in
several ways--by improving the quality of the blood, promoting the
circulation in the superficial layers of the skin, or exerting some
influence upon either the nerve-centres or perhaps upon the vaso-motor
nerves of the cutaneous or articular tissues. The last-mentioned
suggestion is favored by the circumstance noted by Charcot--viz. that
the first effects of arsenic in nodular rheumatism are often
intensification of the articular pains, and sometimes the production of
redness and swelling where they did not exist before. That author found
arsenic without effect or injurious in very inveterate cases and when
the disease had appeared at an advanced age. Five to ten minims of
Fowler's solution, or of the solution of the arseniate of sodium, which
is perhaps less irritating than the former, should be given immediately
after meals, and its effects upon the gastric and hepatic functions
carefully watched. De Mussy has highly recommended arsenical baths
(drachm ss-ij of arseniate of soda to 30 gallons of water), but as the
arsenic is not absorbed by the unbroken skin, any improvement which may
follow its employment is probably owing to the temperature of the bath
or the bath itself.

[Footnote 261: As to the value of arsenic in rheumatoid arthritis, see
Bardsley's _Medical Reports_, London, 1807; Begbie, _Edin. Med. and
Surg. Jour._, 1858; Fuller, _lib. cit._, p. 362; Garrod, _lib. cit._,
3d ed., p. 534; Guéneau de Mussy, _Bull. de Thérapeutique_, t. lxvii.,
1864, p. 24; Charcot, _lib. cit._, p. 222.]

A similar remark has been made respecting the value of the various {99}
thermal mineral baths, natural and artificial, so much employed in this
disease.[262] It is neither the nature nor proportion of their mineral
ingredients, but the degree of temperature, which constitutes the
essential point in the action of a bath. This, if true, explains the
almost equal reputation of the many varieties of thermal mineral
springs in the treatment of rheumatoid arthritis and chronic
rheumatism. It is this that permits the physician to promise the poor
patient as much benefit from the employment of hot baths of simple
water as of those of New Zealand, Plombières, or Arkansas.

[Footnote 262: Vide Niemeyer, _Text-Book Pract. Med._, N.Y., 1867, p.
488; _Traitement du Rheum. par les bains à haute temperature_, par Ch.
Aug. Bouther, Paris, 1878.]

The time for a resort to hot baths in rheumatoid arthritis is when the
very violent pains have subsided sufficiently to allow of their
employment; and while they may be hopefully used in the most chronic
and advanced cases, the earlier they are employed the more curative
they are. The temperature of these hot baths need not, as a rule,
exceed 95 to 100° F., although some authorities approve of raising the
temperature to 110° or 112° while the patient is in the water. A series
of twenty to thirty such baths, taken every second day for ten to
twenty minutes, is sufficient for one trial, and often effects very
great improvement in the disease. The aggravation or return of pain in
the joints which often follows the employment of warm baths will cease
after the fifth or sixth bath. Garrod's experience of the Turkish bath
is not favorable; it very often does much mischief by causing debility,
and its excessive use has induced rheumatoid arthritis in persons
previously free from the disease.

Now, while it may be true that simple hot-water baths employed at home
are as good as mineral thermal baths taken at their source, it is
generally admitted that it is best to send persons who can afford the
expense to the springs themselves, where they may drink the waters as
well as employ them externally, and at the same time secure all the
advantages arising from change of habits, scene, and climate, from
restriction to a proper diet, and from the systematic employment of the
waters and baths under the direction of persons experienced in their
administration, etc. No reliable rules can be laid down for the
selection of the mineral waters best adapted to each case: the stronger
alkaline waters perhaps had better be used with great care, such as
those of Carlsbad, Vichy, Mont Doré, Weisbaden, and after a course of
thermal mineral baths at such places as Aix-les-Bains, Wildbad, Bath,
Aix-la-Chapelle, etc., Garrod advises resort to some place where the
air is bracing and the waters tonic or chalybeate, as Buxton, Spa,
Schwalbach, or St. Moritz. In this country good results are often
obtained at the Hot Springs of Arkansas and the Hot Sulphur and the
Lithia Springs of Virginia. The use internally and in the form of hot
baths of the mineral springs of Saratoga, of Michigan, of the Licks of
Kentucky, and of California, of St. Leon and St. Catherine's (Canada),
is frequently very beneficial. In the selection of the mineral waters
to be drunk, and of the temperature and other qualities of the baths to
be employed, careful attention must be paid to the condition of the
functions of the skin, liver, kidneys, and nervous system; but space
cannot be afforded here for the consideration of this extensive topic.
Moreover, it occasionally happens that after failure of {100} sulphur
or alkaline baths some other form may succeed, as the vapor or hot-air,
or tepid or very hot-water bath. If decided benefit follow the first
series of baths, recourse should be had from time to time to a fresh
series, even for several years, in obstinate cases. Mud and peat baths
are much valued in Germany, although they do not always agree with
weakly or aged people.

The local treatment is of equal importance with the general, and it is
not unfrequently more effective in restoring the functions of the
articulations. In that rare variety, acute rheumatoid arthritis,
attended with much pain and heat in the joints, perfect rest in bed is
called for, together with other measures adapted to subdue the
inflammation and allay the pain. Compresses wet with warm water,
rendered anodyne by the addition of laudanum or belladonna, or both,
and covered with oiled silk, suit some cases--light linseed poultices,
applied moderately warm and extending considerably beyond the limits of
the articulation and covered with gutta-percha or oiled silk, in
others. As the pain and local heat subside, the tincture of iodine may
be applied extensively, or blistering-fluid over limited areas above
and below the affected joints, but not on them until the inflammation
has very much abated and is becoming chronic. These simple methods
should be employed assiduously and be aided by appliances to secure
actual rest of the inflamed joints. In the chronic variety complete
rest is not needed unless during the acute exacerbations, but the
movements should be at first somewhat restrained and be regulated by
the effects produced. But the severe pain experienced during the
movements must be borne; it will subside promptly. Decided increase of
pain and heat in the part, lasting many hours, would indicate more
reserve in the use of the joints. It is frequently very difficult to
determine when and to what extent movement may be permitted in this
disease. No fixed rule can be laid down of universal application, but
it may be stated that in proportion as the local disease becomes
indolent and inactive may pressure and active movements of the joints
be resorted to, for they then have a beneficial influence in preventing
stiffness, contraction, and deformity. Indeed, in my opinion it is not
wise to delay these movements long even in subacute cases. The editor
of this work has especially insisted upon the importance of systematic
daily movements of the affected joints as the most essential part of
the treatment,[263] "combined with thorough massage of all the muscles
whose functional activity is impeded and impaired."

[Footnote 263: "Some Practical Remarks on Chronic Rheumatism," by Wm.
Pepper, M.D., _Archives of Medicine_, Oct., 1880.]

The abiding chronic inflammation indicated by local heat, swelling, and
inflammation of the affected tissues may be variously treated. The
joints may be thoroughly fomented with tolerably hot water or by means
of the local vapor bath for half an hour, morning and night, and then
be gently rubbed for ten or fifteen minutes with iodine or weak
mercurial ointment or with the compound camphor or acetic turpentine
liniment, or, if these are too stimulating, with some bland oil, such
as cod-liver or neats' foot or cocoa oil, after which should be applied
hot-water compresses or linseed poultices or a wrap of soft cotton wool
covered with oiled silk and secured by an elastic, moderately tight
roller. If these means prove inefficient and the inflammatory process
grow more {101} indolent, counter-irritants may be conjoined with or
substituted for them. Small fly blisters or strong iodine paint may be
applied close to the joints, or the ordinary iodine tincture may be
brushed over them, or the above ointments or liniments and one of the
bland oils may be more forcibly rubbed in. The prolonged rubbing of
these stiff, swollen joints with oil is not valued as much as it
deserves.

Compression of the thickened tissues by means of a thick envelope of
cotton wool and thin flannel or rubber bandage sometimes acts very
well, probably by reducing the amount of blood and interfering with
cell-growth or promoting cell-degeneration. Hot sand-baths to the
affected joints are sometimes useful.

These several measures should be perseveringly applied, and in
proportion as chronicity prevails the active and passive movements of
the articulations and massage of the muscles and adjacent tissues
should be daily and efficiently practised.

Electricity will often be found an important adjuvant in this as well
as in an earlier stage, not only in improving the nutrition of the
muscles, but in promoting absorption, allaying pain, and subduing
excitability of the peripheral structures, removing muscular
contractions, and probably modifying the local inflammatory processes.
It appears also in some cases to improve the general health. The
constant current is generally the most useful, and should have an
intensity of about ten to fifteen milliampères, and be applied daily
for ten or fifteen minutes. The positive pole, terminating in a large
flat moistened sponge, is applied to the spinal origin of the brachial
or lumbar plexus, according as the superior or inferior members suffer,
while the negative pole is immersed in a vessel of warm salt water in
which the hands or feet are placed. Some apply the negative electrode
to the joints and the positive to the limb higher up.[264] The faradic
current may also be employed on account of its action upon the muscles
and small vessels. In the advanced stage attended with marked
thickening of the articular and periarticular tissues, with
contractions of the muscles and greater or less impairment of movement,
the above measures are still our chief resources; but they may be
employed more vigorously. We have little fear now of lighting up
inflammation; we indeed desire to excite a more active circulation in
the part with a view of removing the congested state of the capillaries
and venules, so favorable to the development of fibroid growths. In
this stage especially vigorous active and passive movements of the
affected joints, and massage of the muscles which move them, and
gymnastics, are imperatively needed, and it is sometimes almost
marvellous what an amount of mobility and usefulness may thereby be
restored to apparently helplessly crippled and deformed articulations
and members. Persons who have not walked for years are frequently so
much improved as to be able to leave their sofa or bed, and with or
without crutches or mechanical aids walk about, while their abiding
pains depart, and this notwithstanding the permanent deformity of the
articular surfaces. (For the various mechanical appliances that are
sometimes necessary in this advanced stage works upon surgery may be
consulted.)

[Footnote 264: Homolle, _lib. cit._, p. 710.]

The hygienic measures to be observed are probably very much the {102}
same as those indicated in the article upon simple chronic articular
rheumatism--some of them at least--and are such as may be inferred from
a review of the exciting causes of rheumatoid arthritis. Be it
remembered also that acute and chronic articular rheumatism appear
amongst the causes of that disease. We are hardly justified in
promising arrest of the disease on removal to a warm, dry, and even
climate; yet wealthy patients need not be dissuaded from trying the
experiment. The use of flannel underclothing and the employment of
tepid or even moderately cool baths, followed by the use of the
flesh-brush or rough towel, are important means of protecting persons
predisposed to this disease. The ordinary hygienic laws adapted to
maintain a healthy state of all the functions, mental as well as
physical, are to be observed, for in this disease the influence of the
mind over the body is shown by the frequency with which rheumatoid
arthritis follows closely upon mental shocks, worry, etc.

The diet, it is generally admitted, should be of a nutritious
character, yet plain and digestible, and, unless specially required to
meet certain indications, should not include heavy wines or fermented
liquors. However, Garrod affirms that uncomplicated rheumatoid
arthritis is not aggravated by the use of porter, ale, or sound wines;
and his rule is to give sufficient of these alcoholic beverages to
support the tone of the whole system, but not enough to excite the
circulation and thereby produce subsequent reaction.

Finally, the above system of treatment must be persisted in year by
year with the object of securing arrest when cure has not been
effected.


Gonorrhoeal Rheumatism, or Gonorrhoeal Arthritis.

SYNONYMS.--Arthrite ou Arthropathie blennorrhagique,
Tripper-rheumatismus, Gonocele, Urethral Rheumatism, Urethral
Synovitis.

ETIOLOGY.--As its name implies, the cause, par excellence, of the
disease is gonorrhoea, as was perhaps first indicated by Selle[265] and
Swediaur,[266] although, no doubt, an affection apparently identical is
rarely observed associated with non-contagious urethral discharge and
with the urethral irritation incident to catheterism and to stricture.
I have seen it associated with a simple mucous urethral discharge in a
man of gouty habit, married and free from the suspicion of specific
infection. Such discharge has been attributed to gouty irritation, to
dietetic and venereal excesses, and to the contact of non-specific
vaginal secretion; and such origin is well established. More than one
observer has noticed a susceptibility to urethritis on the part of
persons who have had gonorrhoeal rheumatism. A gouty taint is
undoubtedly often present in urethral rheumatism. These non-gonorrhoeal
cases require more close investigation than they have received.[267]
Fournier has not met with them.[268]

[Footnote 265: Chr. Th. Selle, _Medicina Clinica, oder Handbuch der
Medicin_, Berlin, 1781.]

[Footnote 266: Swediaur, _London Med. Gaz._, 1781.]

[Footnote 267: See Elliotson, "Non-contagious Urethral Rheum.," _Med.
Times_, i. 60, p. 613.]

[Footnote 268: Fournier, _Nouv. Dict. de Méd. et de Chir._, t. v. p.
228.]

The stage of the gonorrhoea at which the articular affection may appear
varies very much. It frequently sets in from the sixth to the sixteenth
day of the discharge; it is common enough between the third and sixth
or twelfth weeks, and may be delayed as late as the twelfth month.
There {103} is no constant relation between the severity of the
urethral inflammation and the frequency with which, or the time at
which, the articular symptoms arise; and these, once established,
appear to be largely independent of the state of the urethra. On the
advent of the joint affection the discharge usually continues as it
was, although it often abates somewhat. Fresh attacks of gonorrhoea,
even when very mild, often develop new invasions of the articular
affection, as though an idiosyncrasy existed.

While the ordinary exciting causes of simple acute articular rheumatism
are not necessary to the production of gonorrhoeal rheumatism, they do
now and then act as adjuvants. Such are cold, fatigue, and injuries of
the joints, and a severe acute arthritis is not infrequently developed
during gonorrhoea under such circumstances. Other predisposing
influences probably exist, the absence of which in some measure
explains the infrequency of gonorrhoeal rheumatism as compared with the
prevalence of gonorrhoea. Besnier holds that constitutional rheumatism,
the arthritic habit, or l'héredité arthritique, is not infrequently
present in the victims of gonorrhoeal rheumatism as a predisposition;
Nolen[269] found an inherited rheumatic predisposition in 6 out of 88
cases, and that 4 others had had rheumatism before contracting
gonorrhoea; and Hutchinson maintains that it is the existence of the
arthritic diathesis which enables urethral inflammation to produce
gonorrhoeal rheumatism. He says: "From statistics that I have carefully
collected I have no hesitation in believing that the predisposing cause
of it usually is the inheritance of arthritic tendencies;" and adds,
"Very often the subject of gonorrhoeal rheumatism will give a family
history of gout." However, the disease often occurs in the absence of
any discoverable tendency, hereditary or acquired, to simple articular
rheumatism. On the other hand, persons have had one or several attacks
of gonorrhoea previously that did not give rise to rheumatism. Nolen's
table of 88 cases contains 12 instances of this kind. It is probable
that by reducing the resisting force of the organism, scrofula, the
so-called lymphatic diathesis, anæmia, and debility favor the
development of the disease.

[Footnote 269: "Rheumatismus gonorrhoicus," _Deutsches Archiv für klin.
Med._, Bd. xxxii., 1883.]

Gonorrhoeal rheumatism, like gonorrhoea, is proportionally as well as
actually much more frequent in men than in women (111 men, 7 women,
Nolen); and the greater proclivity of the former has been attributed to
the greater delicacy, sensibility, and complexity of the structures
involved in them than in women by gonorrhoea.

MORBID ANATOMY.--The lesions of gonorrhoeal rheumatism in the early
stage resemble closely those of acute articular rheumatism; and it is
probable, for opportunities of ascertaining by actual dissection are
very rare, that the synovial membrane chiefly suffers. In more advanced
stages the joints contain serous fluid in which fibrinous flakes and
numerous leucocytes are found; the cartilages may be eroded and
softened; and in some protracted cases even the bones may participate
in the inflammation, and the changes found in polyarticular rheumatoid
arthritis may be developed. Ultimately fibrous adhesions, resulting in
ankylosis, may occur. Suppuration very rarely takes place, and it is
probable that in such cases pyæmia is added to gonorrhoeal arthritis.

{104} SYMPTOMS.--Gonorrhoeal rheumatism may attack any of the joints;
it most commonly invades the larger at first, more especially the knee;
the ankle is next in order of frequency, and then succeeds the
shoulder, closely followed by the smaller joints of the hands and feet,
which are very seldom affected primarily and antecedently to the larger
joints. The temporo-maxillary, the sacro-iliac, the sterno-clavicular,
the intervertebral, do not escape gonorrhoeal rheumatism more than they
do rheumatoid or pyæmic arthritis.[270] The disease most frequently
invades several joints simultaneously or successively, but, soon
declining in many of them, it finally becomes localized in a few or
rarely in a single articulation. It is monoarticular from the first in
about 20 per cent. of cases, especially in the knees.

[Footnote 270: Vide Fournier, _Nouv. Dict. de Méd. et de Chir. Prat._,
t. v. p. 230: in 119 cases, knee, 83; ankle, 32; fingers and toes, 23;
hip, 16; wrist, 14; shoulder, 12; elbow, 11; temp.-maxillary, 6; etc.]

Gonorrhoeal rheumatism presents several clinical forms: First,
Arthralgic: pains of greater or less severity, sometimes increased by
movement, but unaccompanied by redness or swelling, affect one or
frequently several joints; they wander from joint to joint, are liable
to exacerbations, and sometimes resist treatment. This form occurs
either in a chronic state in the course of an old gonorrhoea, and
without other rheumatic symptoms, or as an acute affection along with
other rheumatic symptoms, as in the second form. Second: Rheumatic: in
this the symptoms are almost identical with those of subacute articular
rheumatism or the more active forms of polyarticular rheumatoid
arthritis. Several joints are usually implicated, perhaps suddenly,
either quite spontaneously or after chill, exertion, or strain, or
rheumatic-like pains having been felt for two or three days in the
soles, ankles, or loins, the painful joints become moderately swollen,
tender, and hot; pyrexia supervenes with its early chilliness, malaise,
and anorexia; the temperature is not high; the profuse acid sweating
and the very acid, high-colored urine of acute articular rheumatism are
not observed or but transiently and to a very slight degree. In a few
days the moderate febrile disturbance subsides, but the local
inflammation persists, and extends to other joints, without promptly
leaving those first invaded; while lingering in all it often fixes
itself in one or more joints, and is apt to produce a copious and
rebellious intra-articular effusion. Still, it very rarely involves as
many articulations as primary acute rheumatism. The periarticular
tissues usually are more involved than in subacute or even chronic
primary articular rheumatism. Hence the considerable swelling from
oedema on the back of the hand or foot, around the knee, behind the
elbow, and the copious effusion into the adjoining bursæ and tendinous
sheaths, and in the case more especially of the small joints of the
fingers and toes the fusiform enlargement and deformities resulting
from periostitis of the articular extremities. The pain, deformity,
pseudo-ankylosis, etc. produced by these periarticular processes are
very persistent and rebellious, and, although they do usually disappear
at last, occasionally the inflammatory irritation extends to the
cartilaginous and osseous structures, and rheumatoid arthritis with its
permanent deformities results. It is perhaps chiefly in this
polyarticular form of gonorrhoeal rheumatism that cerebral, spinal,
cardiac, pleural, and ocular complications most frequently occur. {105}
In the Third form, or Acute Gonorrhoeal Arthritis, after two or three
days of pain wandering from joint to joint, a single articulation
suddenly, and frequently about the middle of the night, becomes the
seat of atrocious and abiding pain, followed in a few hours by very
considerable swelling of the articulation, not due chiefly to articular
effusion, but to periarticular oedema and enlargement of the bones. The
pain and tenderness are most severe at the line of junction of the
articular surface; the swelling begins at that point, and extends
widely, especially over the dorsal aspects of the wrists and elbows,
the joints most liable to this form, although any articulation may
suffer. The joint is also hot, it may be pale, but is usually more or
less red, and occasionally presents the appearances of severe
phlegmonous inflammation, and excites a sensation of
pseudo-fluctuation.[271] The affection may resolve, or fibrous
ankylosis may ensue, or very rarely suppurative destruction of the
articulation may occur, although such issue has been denied (by
Fournier, Rollet, Voelker). It is remarkable that, like the other forms
of gonorrhoeal rheumatism, the acute inflammatory form is not
accompanied by a general febrile disturbance at all proportionate to
the severity of the local disease. A Fourth form occurs as a Chronic
Hydrarthrosis. Although occasionally accompanying the polyarticular
variety, it is frequently observed independently, and is then often
monoarticular, and affects especially the knee; however, both knees
sometimes are involved. The ankle- and elbow-joints suffer much less
commonly than the knee. The effusion into the articulation takes place
insidiously, although rapidly producing considerable enlargement of and
fluctuation in the joint, without local heat, redness, or tenderness,
and often with but little or no pain or pyrexia. It is not as often
associated with inflammation of the tendinous sheaths and bursæ or of
the eye as the polyarticular form, but it is apt to be very slow in
resolving, and may last for two or three months, a year, or several
years, and in scrofulous patients may degenerate into white swelling.
The formation of pus in the joint is very rare. It occurred twice in 96
cases tabulated by Nolen; hydrarthrosis obtained 12 times; and serous
synovitis 64 times; chronic rheumatism or arthritis deformans 5 times;
tumor albus once.[272] A Fifth form of gonorrhoeal rheumatism, like
other varieties of so-called secondary rheumatism, involves
predominantly the tendons and tendinous sheaths, the bursæ and
periosteum, sometimes without, but far more frequently in association
with, affection of the joints. Pain, sometimes severe and increased by
movement and pressure and aggravated at night, with local swelling and
tenderness, are the symptoms. In their fixity and persistence, their
tendency to relapse, and their chronic course these periarticular
affections resemble gonorrhoeal inflammation of the joints. Gonorrhoeal
bursitis is often severe enough to resemble phlegmon, but it does not
end in suppuration; it is most common in the bursæ covering the
patella, the olecranon, and especially in that under the tendo Achillis
and the deep one covering the inferior tuberosity of the os calcis; but
any of the bursæ may suffer from gonorrhoeal rheumatism. The periosteum
in the vicinity of the affected articulation and over the most
prominent parts of the bones is sometimes the seat of small
circumscribed firm nodes which {106} are painful and tender, and may
either resolve rapidly or very slowly (Fournier).

[Footnote 271: _De l'Arthrite aigue d'origine blennorrhagique_, par le
Dr. André Felix Bieur, Paris, 1881.]

[Footnote 272: _Loc. cit._, p. 133.]

Along chiefly with the third form of gonorrhoeal rheumatism, or
independently, the various muscles and nerves may be the seat of
myalgia and neuralgia. The sciatic nerve is specially liable. In the
same form are often met those ocular affections observed not
infrequently in rheumatoid arthritis and very rarely in acute articular
rheumatism--viz. conjunctivitis and iritis. Aqua capsulitis is more
common than the others, according to Fournier. The ocular affections
may precede, accompany, or alternate with the articular, and, not being
due to direct introduction of the urethral contagium into the eye, are
regarded as manifestations or localizations of gonorrhoeal rheumatism.
The varieties of erythema sometimes present in primary acute articular
rheumatism have been observed in gonorrhoeal rheumatism.

Much difference of opinion obtains as to whether inflammations of the
heart, lungs, and serous membranes occur as manifestations or
localizations of true gonorrhoeal rheumatism. Even those who, like
Besnier, contend for the rheumatic nature of gonorrhoeal rheumatism
admit that they are quite exceptional in that affection. Endocarditis
is probably more frequent than pericarditis, and the aortic are more
liable than the other valves to suffer. Gonorrhoeal endocarditis has
been observed without the articular affection, although it is
especially when several joints are involved and the pyrexia is well
marked in gonorrhoeal rheumatism that the above visceral complications
occur. While admitting that Morel,[273] Marty,[274] Pfuhl,[275] and
others have reported what appear to have been authentic cases of
gonorrhoeal endocarditis, I would remark that it must be almost
impossible at times to distinguish a polyarticular acute gonorrhoeal
rheumatism from ordinary acute articular rheumatism, and that in other
instances the possibility of pyæmia developing in gonorrhoea, and
producing both the articular and the visceral lesions, or the latter
only, cannot be denied. And the same remarks are applicable to the
cerebral and spinal disturbances that Vidart and others have recorded
as occurring in gonorrhoeal rheumatism.

[Footnote 273: _Rev. des Sciences Méd._]

[Footnote 274: _Archives générales de Méd._, Dec., 1876.]

[Footnote 275: _Deutsche Zeitschrift für pract. Med._, No. 50, 1878.]

The course, termination, duration, and prognosis need not be insisted
upon after what has gone before. The duration is very variable. Many
recover in four to eight weeks, many not for three to six months and
longer; relapses are of frequent occurrence; complete and tolerably
prompt recovery is not uncommon in first attacks and in young and
healthy subjects; rebellious persistency, and even deformity, with
impairment of the articular movements, and not infrequently even
fibrous ankylosis of one or many joints, sometimes including the
vertebral, may be observed. Indeed, the most formidable examples of
spondylitis are associated with gonorrhoeal rheumatism as its exciting
cause.[276] These unfavorable issues are most apt to follow repeated
attacks in unhealthy and especially scrofulous persons. Both rheumatoid
arthritis and strumous articular disease have appeared as sequels of
gonorrhoeal rheumatism. Life is not endangered, except in very rare
instances in which cardiac or cerebral {107} complications obtain; and
to stiffened enlarged joints the functions may often be restored by
efficient treatment.

[Footnote 276: Brodfurst cites two such cases: Reynolds's _System of
Med._, i. 980. So does Nolen in an elaborate article upon rheumatismus
gonorrhoicus in _Deutsches Archiv für klin. Med._, Bd. xxxii., 1883. I
had not seen it before this paper was written.]

DIAGNOSIS.--In some instances no doubt what appears to be ordinary
gonorrhoeal rheumatism, owing to the coexistence of urethral discharge
and articular inflammation, is really pyæmic arthritis. The
intermediate link in the causation may be suppuration in the prostate
or its veins or in the testicle or the penis or in its dorsal vein, or
the urethral pus may undergo changes and become septic and be absorbed.
In other instances it is highly probable that true primary acute
articular rheumatism sometimes occurs coincidentally with gonorrhoea.
If in addition to the presence or recent existence of gonorrhoea the
case present several of the following features, gonorrhoeal rheumatism
may be said to exist: moderate or mild pyrexia and articular pain; the
number of joints attacked being few, with a tendency to concentration
in one, either from the first or secondarily; no migration from one
joint to another; no delitescence, but marked chronicity and indolence,
with a tendency to hydrarthrosis and to implication of the synovial
sheaths and bursæ; an absence of cardiac complications; the frequent
and often early coincidence of special ophthalmic affections.

TREATMENT.--The patient should be confined to bed, so as to secure rest
to the inflamed articulations, and when severe arthritis (third form)
exists an efficient splint is peremptorily required, and its
application is often followed by prompt relief to the pain. It should
be retained until not only all pain, but all tenderness on pressing the
articulation, has disappeared. In short, the principles and details of
local treatment suited to gonorrhoeal rheumatism are the same as those
recommended for rheumatoid arthritis, which it so closely resembles;
and the reader is referred to that article for information. Although
there is a greater proclivity to copious effusion into the joints in
gonorrhoeal rheumatism than in rheumatoid arthritis, there is less to
those deeper lesions which affect the bones, and complete recovery is
usually more certain and more prompt in the former than in the latter.
Measures to prevent stiffness and even ankylosis of the articulations
are often an urgent indication. In the general treatment, also, almost
the same remedies are indicated as have been recommended for rheumatoid
arthritis. The salicylate of sodium, given freely, is sometimes
signally useful, more especially when several joints are acutely
inflamed. In the more chronic stages, when much articular effusion
exists, a prolonged course of potassium iodide is occasionally
beneficial. The local measures, however, simultaneously employed,
doubtless co-operate efficiently. Iron and quinia will frequently be
demanded by general debility, anæmia, and impaired nutrition; and the
same may be said of cod-liver oil, extract of malt, etc. The
circumstances under which the various baths are likely to be useful
have been mentioned in connection with the treatment of rheumatoid
arthritis.

The gonorrhoea should be treated in the same way that it ought to be if
no arthritis existed. The rest, the moderate diet, and even the
salicylate of sodium, favor its removal, but the frequent employment of
mild astringent injections should not be omitted.



{108}

GOUT.

BY W. H. DRAPER, M.D.


DEFINITION.--Gout, as a disease, in the traditional acceptation of the
term, is a specific arthritis, characterized by the deposit of the
salts of uric acid in the affected joints. Gout, as a diathesis, is a
blood crasis in which there is an accumulation in the blood serum of
the uric acid salts, the consequence either of the increased formation
or of the defective excretion of these products of proteid
metamorphosis. The manifold irritations of the different tissues, and
the accompanying subjective and objective symptoms provoked by this
dyscrasia, are termed gouty.

SYNONYMS.--(_a_) _Eng._, Gout; _Lat._, Gutta; _Fr._, Goutte; _Sp._,
Gota; _Ger._, Gicht--derived from the nomenclature of humoral pathology
and descriptive of the distillation (goutte à goutte) of the poisonous
humor into the joints--arthritis uratica. (_b_) Gouty diathesis;
constitutional gout; irregular gout.

CLASSIFICATION.--(_a_) Gout as a specific form of articular
inflammation is classified according to its location--cheiragra,
onagra, podagra, gonagra, etc. (_b_) Gout as a constitutional disease
is classified, 1st, according to the structures affected--_e.g._
articular gout; tegumentary gout, embracing mucous as well as cutaneous
affections of gouty origin; nervous gout; parenchymatous or visceral
gout; 2d, according to the degree of the inflammatory process--acute,
subacute, and chronic; 3d, according to certain irregularities
manifested in the development and progress of gouty lesions as
metastatic, retrocedent, and suppressed gout. This classification of
constitutional gout is based upon the well-recognized clinical
observation in the history of gouty persons and gouty families, that
the characteristic lesions of the joint-structures are often correlated
with lesions of the skin, mucous and serous membranes, vessels, nerves,
and parenchymatous organs, which are marked by the same blood dyscrasia
that exists in articular gout, and which are most successfully treated
by the same measures which experience has suggested in the management
of the arthritic disease.

Musgrave in his work[1] treats of a great number of varieties of gout,
as follows: De arthritide anomala; de colica arthritica; de diarrhoea
arthritica; de dysenteria arthritica; de abscesse intestinorum
arthritica; de melancholia arthritica; de syncope arthritica; de
calculo renum arthritico; de asthmate arthritico; de catarrho, tussi,
et peripneumonia arthritica; de phthise arthritica; de angina
arthritica; de capito dolore et {109} vertigine arthritica; de
apoplexia arthritica; de paralysi arthritica; de doloribus in corpore
vagis, fixis; de ophthalmia, de erysipelate et achoribus arthriticis;
etc.

[Footnote 1: _De Arthritide Anomala, sive Interna, Dissertatio_,
Geneva, 1715.]

HISTORY.--The records of medicine furnish simple evidence of the
prevalence of gout in all ancient as well as in modern civilized
communities. Its origin in the perversion of physiological functions
was as clearly recognized by the prophets of the old testament of the
medical art as it is by the founders of the gospel of modern science.
The refined processes of animal chemistry have simply revealed the
materies morbi which was foreshadowed in the "peccant matters" of the
humoralists, which were supposed to be distilled into the joints and
other structures, provoking inflammation and tophous deposits. This is
the most notable and interesting fact in the history of gout, that it
has from the earliest times been regarded as a specific form of
arthritis and dependent upon the circulation in the blood of peccant
matter. It was not, however, until the latter part of the eighteenth
century, when Murray Forbes, and a few years later Wollaston, called
attention to the fact that uric acid was the chief ingredient in
urinary calculi and in tophous deposits, that our knowledge of the
pathology of gout may be said to have had its beginning. The
demonstration by Garrod, in 1848, of the presence of lithate of soda in
the blood of gouty persons, also marks an era in the history of the
pathology of gout.

While the humoralistic theory of gout has prevailed almost to the
exclusion of all others, it is historically interesting to note that
the views of the solidists, as represented by Cullen, who maintained
that "gout was an affection of the nervous system in which the primary
moving powers of the whole system are lodged," have been recently
revived and are attracting considerable attention.

ETIOLOGY: PREDISPOSING CAUSES.--Heredity may be regarded as the most
prominent of the predisposing causes of gout. Statistics of arthritic
gout show this tendency in a varying but always large proportion of
cases. Scudamore observed it in nearly 60 per cent. of his cases;
Garrod, in 50 per cent. of his hospital cases and, in a much larger
proportion, in his private practice; Gairdner found it in 140 out of
156 cases. If all the manifestations of the gouty vice were taken into
consideration in determining the influence of heredity, it would
doubtless be shown in a still larger percentage of cases.

It is generally supposed that there is a greater frequency of
inheritance from the male ancestors and in the male descendants. This
may be explained by the fact that men are more exposed to the other
predisposing and to the exciting causes of gout. My own experience
leads me to suspect that if we took into consideration the irregular
manifestations of this morbid inheritance, we should find it as
frequently in the female, both in the ascending and descending line; of
the greater frequency of acute articular gout, however, in the male,
there can be no question. While it is true that acute attacks are
comparatively rare in women, both before and after the menopause, it is
undeniable that the subacute and chronic forms of gouty arthritis are
by no means rare in them, both before and after the cessation of
menstruation. The Hippocratic proposition that women enjoy immunity
from gout by reason of the menstrual flux can hardly be entitled to
much consideration in view {110} of the fact that they are commonly
less exposed to the exciting causes of the disease, and that when they
subject themselves to the same vicious habits which entail the disease
in men they suffer like men.

Statistics as to the age at which articular gout is most often
developed show that the larger proportion of cases occurs in the decade
from thirty or forty. It is rare before twenty, and the frequency
diminishes rapidly after sixty. Some well-authenticated cases have been
observed before puberty in children in whom the hereditary taint was
strongly developed. Gairdner claims to have seen several cases in
infants at the breast. Trousseau saw a case in a boy aged six, and
Garrod in a youth of sixteen. At the other extreme Garrod reports a
first attack at the age of eighty, and another in the ninetieth year.
The cases at the extremes of age are certainly rare, and other causes
of arthritic inflammation might easily be invoked to explain them. It
is a significant fact that the largest proportion of attacks of acute
articular gout occurs after the period of complete development is ended
and before the period of degenerative changes has begun, when the
necessities of growth have ceased and food is required only for the
nutrition of the tissues, the maintenance of vital energies, and the
demands of work.

Much stress was laid by the earlier writers on the effect of
temperament as a predisposing cause of gout. The vague ideas involved
in the classification of mankind according to temperament may be said
to have lost their influence in the scientific conceptions of modern
pathology. Gout is observed in persons exhibiting the most diverse
peculiarities in physical conformation and physical disposition. The
true interpretation of the facts in regard to the relations of
temperament to gout, so far as those relations exist, would seem to be
that the conditions which give rise to gout are responsible also for
the physical and moral idiosyncrasies of gouty subjects.

A vicious hygiene may be regarded as one of the chief predisposing
causes of gout. The disease is essentially one of advanced
civilization, and is alike the product of the luxury and the misery
which a high civilization entails. It is a common error to suppose that
gout is the consequence only of luxurious living. If the essential
cause of the disease is the circulation of imperfectly oxidized plasma,
then there are two ways in which this defective oxidation may be
brought about: either there is an excess of food ingested beyond the
capacity of the individual, under the most favorable conditions, to
consume, or the conditions of oxidation may be so impaired that the
complete combustion of even a moderate supply of food is impossible.
Perfect oxidation requires an even balance between the amount of food
ingested and the oxygen inhaled. A consideration of this axiom explains
several circumstances in the history of gout. As has been remarked, the
disease is rare during the period of growth and development, when the
processes of nutrition are active and the consumption of food in
excessive quantities is rendered possible by the large demands for the
needs of the growing body and for the development of active energy. It
is common in adult life when the processes of nutrition are less
active, when growth is complete, and when the supply of food must be
regulated according to the amount of energy to be developed. It must
also be observed that while the disease is most frequently caused by
excesses in the consumption of {111} food, it is also often the
consequence of an insufficient supply of pure air; hence we find it
often among those who cannot be accused of gluttony, but whose
occupations or poverty compel them to live and work in a vitiated
atmosphere.

The influence of alcoholic liquors in the production of gouty dyscrasia
is generally acknowledged. There seems to be a striking difference,
however, in the effects of the distilled and fermented preparations of
alcohol in this respect. Gout is certainly more prevalent in countries
where large amounts of fermented liquors are used than in those where
distilled spirits are chiefly consumed. The disease is more prevalent,
for example, in England than in Scotland or Ireland, especially among
the lower classes; it is said also that it is rare in Russia and
Poland, where spirits are more exclusively used. There is a difference
also in the predisposing influence of the different varieties of
fermented liquors in the production of gouty dyscrasia. The heavier
wines, sherry, madeira, and port, are known to be more mischievous in
this respect than the lighter wines of France and Germany, though there
is abundant clinical evidence of the fact that even these wines, and
especially the richer clarets. Burgundies, and Rhine wines, frequently
give rise to acute gout and the gouty habit. There can be no question
as to the pernicious effects of the malt liquors as gout-producers. The
great frequency of gouty diseases particularly among the lower classes
who consume these beverages in large quantities is undeniable. This is
true especially of the stronger English and Scotch ales, and to a less
degree of the lighter English, American, and German beers. The effect
of cider and perry as gout-producers is also well recognized. It has
been observed in certain districts of England where cider is largely
consumed, and, though acute articular gout is said not to be a common
disease in New England, where cider has always been much used, there
can be no question that it often leads to the development of the
irregular forms of gout. As one of the forms of fermented alcoholic
beverages containing, in its fresh state especially, a large amount of
sugar, it favors the production of the acid dyspepsia which is a common
antecedent in the formation of a gouty dyscrasia.

In 1854, Garrod called attention to the fact that a considerable
proportion of the gouty patients in hospital practice--at least 30 per
cent.--was represented by painters and other workers in lead. This
statement has since been confirmed by other observers, and the
association of the characteristic symptoms of this form of metallic
poisoning, such as the blue line on the gums, colic, and the different
forms of paralysis, with both articular and visceral gout, especially
the contracted kidney, is certainly frequent. The relation, however, of
saturnine poisoning to gout in this association is not easy to
determine, Garrod himself pointing out that while the women in the
lead-works frequently had the colic, they but rarely had gout. The
difference in susceptibility of different individuals to all forms of
metallic poisoning is well recognized. It is more strikingly observed
perhaps in mercurial and arsenical poisoning than in that of lead. It
is well known that the internal use of lead as an astringent in cases
of hemorrhage and intestinal catarrh is occasionally, though very
rarely, followed by the evidences of lead-poisoning. This difference in
susceptibility is perhaps explicable on the theory that persons
inclined to gout have less power in eliminating the {112} metal than
those who are not gouty, so that it is possible that plumbism is the
effect rather than the cause of gout, as has been commonly supposed.

Tanquerel des Planches found none of those changes in the kidneys as
the result of plumbism such as are frequently met with in gout, and
Rosenstein, who was able to produce saturnine epilepsy in dogs, found
no renal changes to have occurred. Charcot and Gombault in recent
experiments of feeding guinea-pigs with lead found changes in the
kidneys similar to those produced by tying the ureters.

EXCITING CAUSES.--Paroxysms of acute or subacute gouty inflammation of
the joints, skin, or mucous membranes, as well as the neuroses of gouty
origin, are excited by a variety of causes: errors in diet, both as to
quantity and as to specific articles; excesses in the use of fermented
liquors--even moderate indulgence, in persons with strong gouty
tendencies--are perhaps the most common exciting causes. Sudden changes
in temperature, and especially sudden changes in barometrical pressure,
sometimes excite and often aggravate the sufferings of gouty persons.
Blows, contusions, and mechanical strain frequently determine arthritic
attacks; the large proportion of paroxysms affecting the
metatarso-phalangeal joint of the great toe is explained by the fact
that this joint is more exposed than any other to strain and injury.
Finally, nervous exhaustion, from any cause, from overwork or sexual
excesses, from grief, anger, or shock, may provoke any of the
inflammatory or neurotic consequences of this disease.

PATHOLOGY.--It would be impossible in the limits of this article to
review the many theories that have prevailed in regard to the pathology
of gout, or even to discuss fully those that may be said to divide
professional opinion at the present day. Since the discovery, by
Garrod, of the salts of uric acid in the blood-serum of gouty patients,
the humoral pathology of gout has certainly had the largest number of
adherents.

The lithæmic pathology may be said to be based primarily upon the
chemical theory of digestion or food-transformation. This theory
proceeds upon the idea that every atom of albuminous or carbonaceous
food that enters the body, whether it goes to the construction of
tissue or is destined for the direct conversion of potential into
active energy, is finally eliminated, for the most part, as urea,
carbonic acid, and water. This transformation, of course, is supposed
to be effected by a process of oxidation, but neither the exact mode of
transformation nor the share which the different organs and tissues
take in its accomplishment can be said to be certainly known. Recent
investigations seem to indicate that the liver is chiefly concerned,
not only in the metamorphosis of the carbohydrates, but also in the
formation of urea, so that the arrest in the conversion of starches and
sugars which results in glycosuria, and the check in the metabolism of
the proteids which give rise to lithæmia, may both have their origin in
hepatic derangement. The not infrequent association of glycosuria and
lithæmia in the same patient, and the frequent alternation of gout and
saccharine diabetes in gouty families, are significant facts in support
of the common origin of these diseases.

The purely chemical theory of gout and diabetes, that they are diseases
of suboxidation--a theory most ably advocated by Bence Jones[2]--has
{113} much to commend it from the valuable suggestions which it affords
in the clinical management of these maladies; but it must be
acknowledged that while a defective oxidation seems to be an essential
factor in the production of gout and diabetes, it is impossible to
reduce the process to the simplicity of a chemical equation. It cannot
be claimed that the complex chain of transformations which organic
chemistry has demonstrated in the destructive metamorphosis of albumen
and starch in the laboratory is represented in the vital chemistry of
the body. All that can be said in the present state of knowledge is,
that the metabolism of food is in its nature a chemical analysis,
modified and regulated by vital force, and resulting in the building up
of tissues and in the conversion of potential into active energy.
Imperfect blood-elaboration must depend upon much besides a disturbance
of the balance between the amount of food ingested and the oxygen
inhaled, though this must unquestionably be an important factor in its
production. Heredity and the mysterious influence of the nervous system
complicate the problem of the malnutrition which leads to gout, in such
a way that while the general proposition may be maintained that gout is
a disease in which suboxidation occurs, it is not possible to affirm
whether suboxidation is the essence of the disease or only one of its
phenomena.

[Footnote 2: _Lectures on Some of the Applications of Chemistry and
Mechanics to Pathology and Therapeutics_, H. Bence Jones, London,
1867.]

It is probable, however, that the pathogenesis of the gouty dyscrasia
involves a much more complex process than the simple accumulation of
uric acid salts in the blood. Uric acid, like urea, is one of the
normal results of the metamorphosis of the albuminous foods and
tissues. In birds and reptiles it takes the place of urea as the final
issue of this metabolism. It has been supposed, as one atom of uric
acid can be split by oxidation into two atoms of urea and one of
mesoxalic acid, that uric acid was the penultimate of urea, the result
of a lower degree of oxidation. It is by no means certain, however,
that it is a necessary antecedent of urea. In birds, who consume by
their rapid breathing an enormous proportion of oxygen, as well as in
the slow-breathing reptilia, the nitrogenous excrements are in the form
of urates; and under such divergent conditions it is impossible to
explain the variations in the proteid metabolism by varying degrees of
oxidation. The only reason that can be assigned for the elimination of
the nitrogenous waste in some animals in the form of urea and in others
in that of urates is the teleological one that the urea is destined for
a fluid and the urates for a solid excretion.

But apart from these physiological objections to the theory that uric
acid is necessarily the offending substance in gout, it is well known
that uric acid salts accumulate in the blood in febrile diseases, in
disorders of digestion, and in anæmia--notably in splenic anæmia--and
do not produce either the symptoms or lesions of gout. Todd maintained
that gout might occur without an excess of uric acid in the blood; and
it is certain that in the atonic and irregular forms of the disease
uric acid may not be found in excess in the blood or appear in excess
in the urine. Another significant circumstance in the history of gouty
persons tending to show that uric acid may be, after all, only an
epiphenomenon in the disease, and not its exciting cause, is that the
power of digesting farinaceous and saccharine foods in this disease is
markedly diminished. To such a degree is this true that sufferers from
the gouty dyscrasia are most {114} promptly relieved of their symptoms
of primary indigestion by restricting their diet very largely to
albuminous foods; and not only does such a diet diminish the dyspeptic
symptoms, but I am persuaded by a considerable experience that it is
one of the surest prophylactics against the recurrence of gouty
lesions. It is well known that the fermented preparations of alcohol
are among the most frequent exciting causes of acute gout, and cases
are by no means infrequent in which indulgence in sweet foods and in
fruits will provoke many of the well-recognized local lesions of the
disease.

The explanation of this anomaly in the uric acid pathology of gout may
possibly be found in the suggestion of Garrod, that the deposition of
the urates is caused by their insolubility, and, as this insolubility
is increased by the diminished alkalinity of the serum, that the
evolution of the acids in the digestion of the carbohydrates so
diminishes the normal alkaline state of the blood that the uric acid
salts are more readily precipitated. But even if we accept this
explanation, the fact remains that as efficient factors in the
production of the gouty diathesis the carbonaceous foods may play as
large and perhaps a larger part than the albuminous foods. It would
seem, therefore, in view of the conflicting evidence in regard to the
theory of the uric acid origin of gout, that the chemical pathology of
this dyscrasia is still involved in considerable obscurity.

The recent advances in neuropathology have revived of late years the
views of Cullen on the pathology of gout. Dyce Duckworth[3] has lately
advocated the theory that gout is a trophoneurosis. This theory grows
out of the recognition of the protean manifestations of this disease,
and especially of the neurotic element which is so prominently
developed in its evolution. The frequency of purely nervous symptoms in
gouty persons is a fact which is daily brought to the notice of those
who have much opportunity to study the disease. These symptoms may be
said to affect all the functions of the nervous system; among these we
may mention psychical disturbances, such as hypochondriasis and
hysteria; derangements of sensation, such as neuralgias and
dysæsthesias of every variety; and spasms of voluntary and involuntary
muscles, such as cramps, grinding of the teeth, asthma, and vesical
tenesmus. Another fact which arrests attention in the history of gouty
persons is the frequency with which purely nervous influences determine
attacks of gout; the effect of nervous exhaustion, whether provoked by
overwork or mental anxiety, or the more explosive discharges of
nerve-force in rage and great emotional excitement of any kind, is well
recognized as a frequent precursor of gouty lesions. The influence of
certain diseases of the nervous centres also, such as cerebro-spinal
meningitis, Pott's disease, and tabes dorsalis, in determining
arthropathies and lesions of the skin and mucous membranes, furnishes a
striking analogical argument in favor of the possible nervous origin of
the lesions in gout. The recognition of these facts, however, does not
necessarily militate against the commonly accepted humoral pathology of
gout. The healthy action of the nervous centres must depend primarily
upon a normal nutrition, and a normal nutrition depends on healthy
blood-elaboration. That perverted innervation may be an important
factor {115} in the development of malnutrition through the accident of
inheritance is doubtless true, but in the acquired disease it seems
more probable that the lithæmic condition is the primary source of
disturbed innervation. It may be that gouty lesions are determined as
reflex phenomena through the medium of the trophic centres--if such
centres there be--rather than by the direct irritation of the affected
tissues by the gouty blood; and it is not unreasonable to suppose that
nervous exhaustion from any cause may produce in these centres greater
reflex excitability.[4]

[Footnote 3: _Brit. Med. Jour._, March 26, 1881.]

[Footnote 4: Edward Liveing, in his work _On Megrim, Sick Headache, and
Some Allied Disorders_, p. 404, thus expresses his conviction as to the
neurotic theory of gout: "The view which is commonly entertained is,
that the excessive generation or retention of uric acid in the system,
which is regarded as the fundamental fact in the pathology of gout,
exerts a toxic influence upon the nervous centres, while the particular
character of the disorder is determined by the territory involved. This
limited operation of a cause so general in its nature is a real
obstacle to this view; on the other hand, there is much in the history
of gout--its hereditary character, limitation to particular ages and
sexes, periodicity, explosive character, sudden translations, and
remarkable metamorphic relations with nervous disorders--which seems to
stamp the malady as a pure neurosis; and even the fit itself, with its
sudden nocturnal invasion, the late Dr. Todd was accustomed to compare
to one of epilepsy or of asthma."]

PATHOLOGICAL ANATOMY.--Blood-Changes.--Garrod's demonstration of the
excess of uric acid in the blood of gouty persons constitutes the chief
recognized hæmic change in this disease. That this is a constant
change, and one that is essential to the existence of gout, cannot be
said to be proved. The presence of uric acid in the blood is not always
productive of gout, since it has often been found in the blood of
healthy persons, and its temporary excess during pyrexia, and
especially in the fevers and other morbid states in which spleen is
congested, has already been noted. The excess of uric acid, however, in
gouty blood may reach, according to Garrod, as much as 0.11 grain in
1000 grains of serum. It is probable that other excrementitious
substances exist in the blood in gout which bear a closer etiological
relation to this disease than uric acid, but they have not been
demonstrated. The other blood-changes which are noted by Garrod--the
diminished specific gravity of the serum from loss of albumen, the
diminished alkalinity, and the increase of the fibrin in the
inflammatory forms of the disease--are probably inconstant. In chronic
gout the objective signs of anæmia which are often present would
indicate a marked diminution in the red blood-corpuscles.

The tissues which are the chief seat of gouty lesions are the
connective tissues. In the evolution of the disease the joints, where
the connective tissue is most dense and the least vascular, suffer
earliest; at a later period the connective tissue of the blood-vessels,
nerves, and viscera becomes subject to gouty changes.

According to Garrod, the exudations in articular gout are rich in the
urates of soda, lime, magnesia, and ammonia; they also contain some
phosphate of lime and traces of organic matter. The watery portion is
absorbed and the salts are deposited in crystalline forms. The location
of these deposits varies: they are found on the synovial surfaces, in
the cartilage-cells, and in the intercellular substance; in the
tendons, ligaments, and bursæ, and in the subcutaneous connective
tissue. The urate of soda occurs not on the free surface of the
cartilage, and replacing {116} the latter, as was formerly generally
supposed, but as an infiltration into the substance of that tissue; and
Garrod found that there is always a thin layer of unaffected cartilage
lying between the deposit and the free articular surface--an
observation which has been confirmed by Budd and quite recently by
Ebstein.[5]

[Footnote 5: W. Ebstein, _Die Natur und Behandlung der Gicht_,
Wiesbaden, 1882.]

Very important are the recent investigations of the latter. After
making numerous observations on the cartilages and other affected
tissues of gouty subjects, besides studying the disease artificially
produced in fowls, he has shown that those portions of cartilage and
other tissues in which the deposit occurs are in a state of necrosis,
as is evident from the fact that when the urates are dissolved out by
warm water the area in which the deposit occurred, though apparently
normal to the eye, refuses to be stained with aniline dyes, and lies
plainly visible as a light spot in the midst of stained tissue. Since
the work of Weigert we know that this is a sure sign of that peculiar
form of death of a tissue to which the name of coagulation necrosis has
been given. Ebstein regards this necrosis as primary and the deposition
of the uratic salt as secondary. According to him, the urates
circulating in the blood give rise to necrosis in parts where the
circulation is sluggish (as the articular cartilages, the ears, and the
extremities generally), and where, consequently, they remain a greater
length of time in contact with the tissues. The necrotic portion has,
however, an acid reaction, which causes a deposition, from the soluble
neutral salt, of an acid urate in a crystalline form. Ebstein claims
that this necrotic area, in which there is deposited a crystalline
urate of soda, and around which there is a secondary inflammatory zone,
is characteristic solely of gout. "I have never seen," he says, "in
gout a crystalline deposit of urates occurring in normal tissue."

In addition to these so-called specific changes we find a hyperplasia
of the connective tissue in the fibrous structures of the affected
joints. The thickening thus induced, with the contraction of the new
tissue and the atrophic changes resulting from pressure and disuse, are
the causes of the deformities, subluxations, and impaired movements of
gouty joints. Occasionally, the local irritation provoked by the
pressure of the tophous deposits results in abscesses from which a
mixture of pus and pasty urates may be discharged. These abscesses in
feeble and anæmic subjects are sometimes difficult to heal. More
frequently the skin undergoes gradual absorption and the chalk-like
deposits are exposed.

The frequency with which the metatarso-phalangeal joint of the great
toes is affected in gouty persons has always been noted. In Scudamore's
tables the proportion of the first attacks in this joint was 72 per
cent., and in 66 per cent. one or both great joints were affected to
the exclusion of other joints. This frequency is due to the fact that
this joint is the most vulnerable one in the body, bearing as it does
the weight of the body and being exposed to most frequent shock. The
phalangeal joints of the hands and the wrist-joints are also often the
seat of acute gout, though these joints are more frequently affected by
the subacute form of the disease. The larger joints may also be the
seat of true gouty inflammation; indeed, no joint, not even the
intervertebral, can be said to enjoy immunity, and the hip and shoulder
are occasionally attacked to {117} the exclusion of others. The
cartilages of the ear and the arytenoid cartilages are sometimes the
seat of gouty deposits.

The great frequency of arterial sclerosis, and the subsequent fatty and
chalky metamorphosis in persons who have suffered from chronic gout,
are well recognized. Next to syphilis, gout seems to be the most common
cause of these arterial changes. The influence of these lesions in the
arteries and capillaries in determining cardiac hypertrophy and
cerebral hemorrhage is often seen in the accidents which terminate the
lives of gouty patients.

In the heart a gouty endocarditis is of not uncommon occurrence,
according to Ebstein, who cites Lancereaux as having found uric acid in
concretions on the valves. Garrod, however, after examining a number of
cases in which cardiac disease existed with gout, states that in his
opinion the valvular changes are not due to a gouty deposit, he never
having been able to demonstrate the presence of uric acid in them.

Some years ago Sir James Paget called attention to the frequency of
adhesive phlebitis as a gouty lesion. This is observed in connection
with articular gout, but may also occur independently of joint-lesion.
It is observed most frequently in the lower limbs, is generally
symmetrical, and shows a disposition to metastasis.

Neuritis and sclerotic lesions of the nerve-centres are not uncommon in
the history of acquired and inherited gout. The neuralgias and other
temporary dysæsthesias which constitute a considerable category in the
symptoms of gouty persons are doubtless due to transient central and
peripheral lesions.

The so-called gouty kidney is the most striking illustration of the
effect of the gouty dyscrasia in the production of a characteristic
visceral lesion. The changes which occur in the kidney as a result of
gout are--a contraction of the organ, the result of interstitial
inflammatory processes, and a deposition of uratic salts, occurring
mainly in the papillary portion. The views as to the exact locality
where these deposits occur still differ considerably. Garrod is of
opinion that it occurs in the fibrous interstitial tissue. Virchow, on
the other hand, regards the lumen of the tubuli as the seat of the
deposit, and in this he is supported by Charcot and Cornil and Ranvier,
Lancereaux and Wagner. Dickinson inclines to the view of Garrod, and
believes that it is the deposition of the urates in the interstitial
tissue which gives rise to the chronic inflammation which results in
cirrhosis of the kidney--the granular kidney of gout. Ebstein seems to
think that the interstitial connective tissue, having previously
undergone a state of necrosis, as in cartilage and other connective
tissues, is the seat of the deposit. As in cartilage, he regards this
necrotic state as typical of gouty deposits. About the necrotic area in
which the deposit has occurred a secondary inflammation takes place,
leading ultimately to contraction of the new fibrous tissue formed. He
calls attention to the fact that (1) the kidneys may be perfectly sound
in gout; (2) the kidneys may be the seat of chronic interstitial
inflammatory changes, with cirrhosis, without any urate deposits of any
kind being demonstrable; (3) there may be chronic interstitial
nephritis, with crystallized urates in the urinary tubules.

As regards changes in the liver, few satisfactory accounts exist. {118}
Portal originally called attention to the fact that in gout and
rheumatism indurations of the liver caused by the deposit of a
phosphatic earth occurred, and Charcot has recently referred to the
fact. Ebstein cites a case in which in a gouty patient he was able to
make a diagnosis of moderate hypertrophic hepatic cirrhosis, but so far
he had not been able to confirm it by post-mortem examination.[6]

[Footnote 6: Gout in Animals.--Of the occurrence of gout in animals not
many reliable reports exist; Ebstein has collected a few. Thus, he
cites a case where in an old hunting-dog uratic concretions were found
in the articular ligaments and in the periosteum of the epiphyses of
many joints, but especially those connecting the ribs with their
cartilages. In the toes of falcons and of parrots kept in confinement
deposits of urates have been observed, and in an alligator dying in
captivity deposits were found in the muscles as well as the joints
which consisted of free uric acid together with sodium urate.

Experimentally, Ebstein was able to produce gouty lesions having all
the characteristics of those occurring spontaneously in man by
injecting subcutaneously small quantities of the neutral chromate of
potash into the blood of cocks for a considerable period of time. By
this method changes in the epithelial elements of the kidney were
produced, preventing the elimination of the urates from the blood and
causing their consequent accumulation in the system. He obtained in
this way typical deposits of urates in the joints, tendons, muscular
sheaths, heart, and other organs, while the birds emaciated and finally
died. But these experiments, which are extremely valuable and
interesting, still need confirmation.

The experiment of tying the ureters of fowls is an old one. Galvani who
was perhaps the first to perform it, employed it in his investigations
on the kidney, and since then Zalesky, Pawlinoff, Von Schroeder,
Colosanti, and others have made use of it in their experimented studies
on the site of origin of uric acid. As a result of this operation
deposits of urates occur in various organs. Ebstein, however, does not
regard them as analogous to the gouty deposit in human beings, as they
lack the feature of necrosis, which, as mentioned above, he considers
as alone characteristic of the true gouty lesion.]

SYMPTOMATOLOGY.--The development of true gouty lesions, whether of the
acute or subacute form, is usually preceded by a period, more or less
protracted, in which characteristic derangements of the health present
themselves. These derangements may be conveniently classified as
disturbances of primary digestion and as manifestations of
malnutrition.

The disturbances of primary digestion are repeated attacks of flatulent
dyspepsia, with pyrosis, colicky pains, alternate constipation and
diarrhoea, and a scanty, high-colored, and heavy urine with uratic
sediments. This dyspepsia may be accompanied with a variety of reflex
nervous symptoms, such as pain in the nape of the neck and occiput,
insomnia, palpitation, sighing respiration, singultus, and nausea.
These symptoms are commonly described as due to biliousness, and are
provoked by excesses in diet, and not unfrequently by moderate
indulgence in certain common articles of food, such as sweets, fruits,
farinaceous foods, and the fermented preparations of alcohol.

Derangements of nutrition are shown by a disposition to erythematous
and catarrhal affections of the skin and mucous membranes, to
affections of the sebaceous glands, and to premature falling of the
hair. There is often a more or less marked tendency to obesity.
Accompanying these derangements there may be a loss of energy, both
physical and mental, manifesting itself in indolence and fatigue on
slight exertion, in irritability of temper, with diminished
intellectual activity and hypochondriasis. Neither the primary
indigestion nor the nutritive derangements invariably precede the
development of acute gouty lesions, nor are they necessarily followed
when they exist by the articular signs {119} of gout; but they are so
commonly associated with the evolution of what are regarded as the
specific lesions of gout that they may fairly be described as
constituting its prodromal period.

ACUTE ARTICULAR GOUT.--A typical attack of acute gout is usually
sudden. It seizes its victim without warning, and often rouses him from
sleep with a vicious agonizing pain in the joint assailed. Examination
will reveal a slight redness, heat, and puffiness of the part affected
altogether disproportioned to the intensity of the pain; the tenderness
is exquisite, and the torture is often aggravated by the occurrence of
reflex spasms of neighboring muscles. There is usually moderate fever,
and if the surface be exposed there may be a chill. Sleep is impossible
and the restlessness uncontrollable. As the morning advances slight
perspiration occurs, and sleep may become possible. With the abatement
of pain there is coincident increase in the signs of inflammation: the
joint swells, the skin becomes red and oedematous around the joint, and
the superficial veins are distended. But, though the pain subsides with
the occurrence of swelling, and usually in proportion to its degree,
the tenderness and pain on any attempt to move the joint continue to be
extreme. The day is passed in comparative ease, but the evening
generally brings an exacerbation of pain and fever, and the night
another paroxysm of agony--not as severe as the first, but severe
enough to make the daylight a benison. The progress of the disease
after the second day, provided it is confined to one joint, is usually
marked by a steady and regular decline in the severity of the symptoms.
If the attack is confined to a single joint, a week may elapse before
the inflammatory signs subside, and it may be a fortnight before
pressure can be borne or the mobility of the joint is restored.
Occasionally the sufferings of an acute attack of gout may be
protracted by successive seizures for several weeks. The fever during
the attack is distinctly remittent, the evening exacerbation rarely
exceeding 103° F.

The urinary symptoms before, during, and after an acute paroxysm of
gout are interesting and important in their bearing upon the uric-acid
theory of the disease. Garrod's statements upon this point are
generally accepted, and have been confirmed by other observers. He says
that previous to the attack the amount of uric acid in the urine is
below the average--that during the paroxysm the proportion grows
smaller, and only rises to the normal standard with the termination of
the seizure. The reaction of the urine is strongly acid during the
paroxysm. This is due probably to the increased excretion of acid
phosphates. The quantity of the urine is generally diminished, the
specific gravity increased, and the color deepened.

Attacks of acute gout are generally followed by improved health and
capacity for physical and mental work and enjoyment. The blood seems to
be purified, the processes of digestion and assimilation are once more
normally performed, the equilibrium of the nervous centres is restored,
and the evolution of all the vital energies proceeds with ease and
vigor. This state of well-being may continue for a year or two years,
or even a longer period, after the first attack, the immunity varying
according to the intensity of the inheritance or the habits of life.
The subsequent attacks are apt to occur at increasingly shorter
intervals, and, as a rule, the acuteness of them tends to diminish.
Gradually the dyscrasia becomes more {120} profound, and the
constitutional symptoms and structural changes which belong to the
atonic and irregular forms of the disease are developed.

ATONIC GOUT.--Though subacute, irregular, or atonic gout is often the
sequence of repeated attacks of the acute disease, it is not
necessarily preceded by them, nor is acute gout invariably followed by
a marked gouty dyscrasia. It is not uncommon for a well-characterized
gouty habit to exist, manifesting itself by many and varied gouty
phenomena, without the occurrence of any acute lesions, and repeated
attacks of acute articular gout may occur without the development of
the progressive impairment of health and the tissue-changes which
distinguish the chronic malady. The recognition of this fact is
important, inasmuch as the occurrence of acute gout is commonly
regarded as an essential element in the diagnosis of the gouty
dyscrasia. Acute articular attacks, as already noted, are very rare in
women, in whom the subacute and irregular forms of the disease are by
no means infrequent. So far as acute articular gout is of value in the
diagnosis of the constitutional vice, it is perhaps as significant if
established in the history of a near relative as in the individual in
whom the disease is suspected.

The general symptoms of atonic gout--or, as it may more properly be
called, the gouty dyscrasia--are similar to those which sometimes
precede the development of the acute form. The difference lies in their
persistence, in the subacute character of the local lesions, and in the
absence of the relief to the constitutional symptoms which follows
acute attacks.

The dyspeptic symptoms are perhaps the most pronounced and uniform in
the history of the evolution of chronic gout. These symptoms have been
already described, but the fact which seems especially to distinguish
them is that they are chiefly provoked by the acid fermentation of the
carbohydric elements of the food, the sugar and starches, and
especially by the fermented preparations of alcohol; the ability to
digest these articles of diet appears to be deficient in the gouty
dyspeptic.

The changes in the urine in the gouty dyscrasia are especially
important. In the formative stages of the gouty vice the amount of
urine may not vary much from the normal quantity, but the proportion of
solid constituents, especially of the urea, is increased, so that the
specific gravity may rise to 1.030 or 1.035. The acid reaction is
intensified by the excess of the acid urates and phosphates upon which
the normal acidity depends. Sometimes crystalline deposits of uric
acid, urates, and oxalates take place in the tubuli of the kidney and
in the bladder, and lead to the nephritic and vesical irritations which
are often the source of much inconvenience and pain. Where the urine is
free from these crystalline constituents as it comes from the bladder
it may deposit them within a few hours after its passage. At a later
stage in the development of the gouty dyscrasia the quantity and
quality of the urine undergo marked changes. The quantity is increased;
the color is pale, partly in consequence of dilution and partly through
a diminution in the amount of coloring-matter. The quantity may be so
considerable as to constitute a polyuria. The reaction is neutral or
only feebly acid; crystalline sediments of uric acid and calcium
oxalate may occasionally appear, and the specific gravity may be so low
as to indicate not only a relative but an absolute diminution in the
daily excretion of urinary solids. Traces of albumen and of sugar are
not infrequently observed.

{121} The articular symptoms of chronic gout are subacute. They affect
the joints, as a rule, which are most exposed to strain and injury, and
hence are most common in the hands and feet, but they may involve the
knee and the hips, the elbow and the shoulder, and even the
intervertebral joints. The pain is less severe, because the tension is
never so considerable; the tenderness is often a source of great
discomfort; the swelling varies with the acuteness of the inflammatory
process, the joints being more or less permanently enlarged by
hypertrophic changes affecting the articular structures and by tophous
deposits. The deformities are increased by ankylosis, by contractions,
by absorption of the cartilages, by partial luxations, and by the
atrophy of disused muscles. Crepitations are often observed in the
affected joints. Exacerbations of the local symptoms are often provoked
by movements, by imprudence in diet, by changes in temperature or in
barometric and hygrometric conditions, and not infrequently by
psychical disturbances.

The frequency with which tegumentary affections, mucous as well as
cutaneous, are observed as correlative phenomena of arthritic lesions
in gouty persons and in gouty families justifies the inference that the
same lithæmic vice which determines articular inflammations is often
responsible for derangements of nutrition in the skin and mucous
membranes. The French school of dermatology, which has always
maintained the humoral origin of many cutaneous diseases, has long
recognized the arthritic nature of a large class of affections of the
skin. Bazin[7] has given the most precise description of the
arthritides, as he terms them. He insists upon certain functional
derangements of the skin as characteristic of the gouty diathesis, such
as excessive perspiration, especially in certain regions, as the head,
the axillæ, the hands and feet, and the sexual organs, and also
affections of the sebaceous glands, causing the different forms of
seborrhoea and the premature falling of the hair. He notes the
liability in gouty persons to certain neurotic affections, such as
pruritus, general or localized, about the arms and genital organs.
Erythematous affections, especially urticaria, erythema nodosum, and
the fugitive erythema which occurs about the face, causing sudden and
evanescent swelling of the eyelids, cheeks, lips, and even the tongue
and soft palate, are recognized by him and other observers as arthritic
in their origin. Among the erythemata which are observed in gouty
persons the peliosis rheumatica should be mentioned.

[Footnote 7: _Affections génériques de la Peau_, Paris, 1862.]

The more persistent inflammatory lesions of the skin, such as eczema
and psoriasis, which are characterized by long-continued hyperæmia with
hyperplasia, are now recognized as among the possible transformations
of gout. They are certainly often observed alternating with arthritic
lesions, and associated with all the characteristic derangements of
nutrition which belong to the gouty habit. The frequency of the various
forms of acne, the inflammatory, as well as those which result from
excessive function of the glands, in persons having a strong gouty
inheritance, is recognized by many dermatologists. I have noticed these
lesions especially in young women belonging to gouty families. They are
generally accompanied by marked dyspeptic symptoms, and not
infrequently by neurotic derangements.

Garrod, in a paper read at the International Medical Congress in 1881
{122} on "Eczema and Albuminuria in Relation to Gout," affirms that
each year strengthens his conviction that gout and eczema are most
closely allied. Since his attention was first called to this relation
in 1860, he has found a gradually increasing percentage of eczema in
the cases of gout that have come under his observation. Dividing all
the cases from 1860 to 1881 into ten groups, he found the percentage
rose from 10 in the first group to 47 in the tenth. He accounts for
this rapid increase in the percentage in the fact that in the first few
years the eczema was only observed when it was very patent; during the
past two or three years he has had made more careful inquiries as to
the presence of eczema or other skin eruption in every case of gout,
and by these means has frequently discovered its presence when it might
otherwise have been overlooked. Garrod believes that eczema is the
special skin-lesion of gouty subjects, and does not regard psoriasis as
having anything more than an accidental connection with gout. He admits
that the latter is often associated with rheumatoid arthritis. It must
be remembered, however, that Garrod does not admit that gout ever
exists without lithatic deposits.

In regard to the location of gouty eczema, it appears to affect by
preference the more tender and vascular regions of the skin. The
eyelids, ears, the scalp, and back of the neck, the fingers and toes,
particularly the dorsal and lateral surfaces, and in old people the
legs, are especially liable to be attacked. The subjective symptoms of
gouty eczema are often the source of great suffering; the burning and
itching are sometimes intolerable. This is especially true of persons
of highly neurotic constitution.

It is not possible to affirm that there are lesions of the mucous
membranes which are strictly analogous in their transient character to
the erythematous affections of the skin, but it is not unreasonable to
suppose that many of the temporary disturbances of indigestion to which
gouty patients are subject are caused by an evanescent hyperæmia
corresponding to the vaso-motor derangements which are observed in the
external integument. In regard, however, to the more persistent
catarrhal lesions, there can be no question as to their analogy with
those which affect the skin. The continuity of these lesions at the
orifices of the mucous tracts, and the frequent association of external
eczemas with catarrhs of mucous membranes, are facts of common
experience. Greenhow[8] of London first called attention to the
frequency with which chronic bronchitis is associated with the gouty
dyscrasia. In an analysis of 96 cases of chronic bronchitis he elicited
the fact that in 34 out of the 96 a distinct gouty history attached
either to the patients themselves or to some of their immediate
relatives. In 14 of the cases the patients were subject to attacks of
acute regular gout as well as to bronchitis. He also noted the
association in a number of cases of bronchitis and psoriasis with
gravel and gout. My own experience confirms these observations, and
also the alternations of catarrhal and parenchymatous tonsillitis, of
pharyngeal and laryngeal catarrh, and of asthma and chronic bronchitis,
with the more common manifestations of regular and irregular gout.

[Footnote 8: _On Chronic Bronchitis_, E. Headlam Greenhow, M.D.,
London, 1869.]

The occurrence of subacute gastro-duodenal and intestinal catarrhs
{123} with hemorrhoidal complications is even more common that the
catarrhal affections of the respiratory tract. The lesion, in fact,
which gives rise to the manifold dyspeptic symptoms in gouty subjects
is doubtless a catarrhal one.

The genito-urinary tract exhibits also the tendency to catarrhal
affections in sufferers from the gouty dyscrasia. It is certain that
gouty persons are especially liable to vesical catarrh, and it is
generally admitted that rheumatic and gouty persons are particularly
susceptible to gonorrhoea. My own experience leads me to suspect that
chronic urethral discharge resulting from acute urethritis is more
common in rheumatic persons than in those not having this taint. The
etiological relations of gonorrhoeal rheumatism and kerato-iritis are
still involved in obscurity, though I am inclined to believe that a
careful examination of the personal and family history in cases of
these diseases would establish the opinion that has been maintained as
to their gouty origin.

The presence of albumen in the urine of persons suffering from acute
gout is occasionally observed. Under these circumstances it is
transient, and has probably no more significance than is usually
attached to this symptom in the course of any acute febrile disease. In
chronic gout it is by no means infrequently observed as a more or less
persistent symptom. It is associated under these circumstances with a
copious discharge of urine of pale color and low density, and with the
general signs of what Rayer first described as the néphrite goutteuse.

The importance of this symptom is very great when we consider the
insidious development of this form of disease and the difficulty of its
early diagnosis. Recent investigations point to the value of the
changes in the urine in the progress of the gouty dyscrasia as bearing
upon this question. It has already been noted that in the early history
of gouty persons the urine is often scanty, high-colored, excessively
acid, of high specific gravity, occasionally albuminous and saccharine,
and frequently depositing sediments of urates and calcium oxalate.
McBride of New York[9] has recently called attention to this condition
of the urine and its association with high arterial tension as the
functional stage of the granular kidney--as the stage, that is to say,
during which the necessity of eliminating large amounts of imperfectly
oxidized nitrogenous material maintains a constant state of renal
hyperæmia, which finally induces the changes in the tubular and
intertubular structures which constitute the anatomical features of
this form of disease.

[Footnote 9: _The Early Diagnosis of Chronic Bright's Disease_, T. A.
McBride, M.D., New York, 1882.]

The occasional presence of sugar in the urine of gouty persons has
already been noted. I have repeatedly observed this symptom in the
urine of gouty dyspepsia. It occurs more commonly in obese subjects,
and is usually intermittent and easily controlled by dietetic
restrictions. In these cases it is not necessarily associated with a
very large amount of urine. In chronic gout and in connection with the
granular kidney a more serious form of glycosuria is occasionally
observed. Under these circumstances it increases largely the polyuria
which is characteristic of gouty nephritis, and is sometimes overlooked
because it occurs in a urine of a low density, often not more than
1.010. It is not controlled by diet {124} to the same extent that it is
in the cases previously described, and is in my experience a prognostic
sign of bad import.

Some of the most distressing symptoms to which gouty persons are
especially liable are those connected with the passage of gravel from
the kidney to the bladder. Where gravel alone passes, it may cause
little uneasiness, and the fact is only recognized through the
discovery of blood in the urine in connection with uric acid or calcium
oxalate crystals. When, however, the sand forms concretions in the
pelvis of the kidney, their dislodgment and passage through the ureter
are accompanied by the well-known agonies of renal colic.

Dysuria is a symptom from which gouty persons often experience much
inconvenience and suffering. It is usually associated with extremely
acid urine of high density containing crystalline sediments. It may
manifest itself only in frequent and painful micturition, or it may be
associated with such a degree of vesical tenesmus as to cause retention
and necessitate the use of the catheter.

DIAGNOSIS.--If the term gout be restricted to that form of arthritis in
which an excess of urates is found in the blood with tophous deposits
in the affected joints, the cartilages of the ear and nose, and in the
subcutaneous connective tissue, then the diagnosis of this disease is a
simple one. It is a disease with a pathognomonic sign. But if the
pathology of gout consists rather in a more complex morbid condition of
the blood, of which an excess of urates in the serum is only one of a
number of phenomena, and not necessarily the sole and essential cause
of the local lesions, then the question of diagnosis involves a
consideration of all the correlated morbid conditions which are so
frequently associated in gouty persons and gouty families as to justify
the inference that they have a common origin in a perverted nutrition,
the essential nature of which is imperfectly understood.

The very existence of the terms gouty rheumatism and rheumatic gout
which are in common use shows that what is regarded by many excellent
authorities as the confounding of distinct entities must have some
foundation in clinical experience. If we consider gout, in its
strictest pathological sense, acute inflammatory rheumatism, rheumatoid
arthritis, or gouty rheumatism, and senile arthritis or the arthritis
deformans and gonorrhoeal rheumatism as separate and distinct diseases,
we shall find ourselves compelled to ignore certain common clinical
facts which indicate a bond of union between them. Heredity, for
example, is common to them all, and more than this, there appears to be
a tendency to a differentiation of the taint in families. It is well
known, for instance, that the children of gouty parents are especially
liable to acute rheumatism, and acute rheumatism in youth is often
followed by gout in later years. It is also a fact of common experience
that while the men in gouty families are the victims of true gout, the
women are apt to be the subjects of rheumatoid arthritis. The arthritis
deformans which develops with the degenerations of advancing years is
not infrequently associated with a family history of genuine gout.
Gonorrhoeal rheumatism also, according to the experience of many
trustworthy observers, often recognizes an inheritance to gouty
lesions. But it is not alone in heredity and the differentiation of the
type of the disease in families that the unity of these affections
displays itself. The same disturbances of digestion which {125}
characterize the history of true gout are observed in those who are
liable to acute rheumatism, to rheumatoid arthritis, and to arthritis
deformans. It is true that excesses in food and fermented liquors do
not determine, as in gout, attacks of acute rheumatism nor of the
chronic forms of arthritis, for these latter diseases are commonly due
to causes operating upon the nervous system, as exposure to cold and
dampness or to physical or emotional shock of some kind; still, there
is in the subjects of these diseases a more or less marked tendency to
the same dyspeptic disorders, and especially to the diminished capacity
in digesting the carbohydrates, which the subjects of true gout
exhibit. In the diagnosis of gout, therefore, it would seem that the
question of differentiating this disease from those which simulate it
is not one in which we are called upon to distinguish one morbid entity
from another, as typhus from typhoid fever or syphilis from cancer, but
rather to determine, first, the presence of a recognized constitutional
vice; and, secondly, to differentiate the variety of the lesions by
which this vice manifests itself.

In the diagnosis of the gouty dyscrasia the first point to determine is
that of heredity. This requires a careful inquiry into collateral as
well as direct descent, and does not necessarily involve the discovery
of arthritic diseases in the ancestors, though these are doubtless the
most striking and trustworthy proofs; but the tradition in the family
of persistent dyspepsia, or what is commonly called biliousness, of
chronic catarrhal affections of the skin and mucous membranes, and of
the chronic forms of renal disease, are significant indications of this
dyscrasia. In the personal history the evidences of the lithæmic
tendency, as indicated by the characteristic dyspeptic symptoms which
have been described, and especially by the feeble capacity for the
digestion of carbohydrates, are of great diagnostic value.

The diagnosis of gouty joint-lesions, whether acute or chronic, depends
partly upon the determination of the gouty dyscrasia, and partly upon
the differential distinctions which separate gouty inflammations from
acute rheumatism, rheumatoid arthritis, and from the arthropathies
which result from traumatism and from lesions of nerves and
nerve-centres.

Gouty arthritis may be distinguished from acute rheumatism by the fact
that it is more often hereditary--that it occurs in older subjects,
attacking generally the smaller joints, and, as a rule, in the acute
form, localizing itself in one or two joints. It is also noteworthy
that the constitutional symptoms are not as severe as in rheumatism.
Gout deforms the joints, while acute rheumatism leaves no traces of the
inflammatory process. In addition to these distinctions there is,
according to Garrod, the crucial test of an excess of urates in the
blood-serum.

From rheumatoid arthritis or rheumatic gout, gout in its acute and
regular form is distinguished by the more acute local and
constitutional symptoms. Gout is periodical in its attacks, while
rheumatoid arthritis is progressive. It attacks the smaller joints or
those most exposed to strain, while rheumatoid arthritis occurs in the
large as well as the small joints, and appears to be more independent
of traumatism as an exciting cause. Gout is more common in men,
rheumatoid arthritis in women. According to Garrod and other excellent
authorities, deposits of urates are never found in the joints in
rheumatoid arthritis, and there is no excess {126} of urates in the
blood. This statement is denied by Hutchinson. Ulcerations of
cartilages, contractions of tendons, atrophies of muscles with
subluxations of joints, are more common in rheumatoid arthritis than in
gout.

While these local distinctions are undeniable, it is proper to observe
that in rheumatoid arthritis the constitutional symptoms of the gouty
dyscrasia, especially the dyspeptic derangements and the nervous
disturbances, are often well marked; and it should also be noted that
the principal distinction, the absence of urates in the blood and in
the diseased joints, is one that is based on the exclusive theory that
uric acid is the materies morbi of true gout. If, as is still
maintained by some excellent authorities, uric acid is not essential to
gout, then it must be confessed that the other distinctions are purely
lesional, and that the common constitutional symptoms suggest that
these diseases are divergent branches of a single trunk.

Gouty arthritis is not always easily distinguishable from traumatic
inflammation of the joints, inasmuch as traumatism plays so important a
part as an exciting cause of gouty attacks. The history of previous
seizures and the presence of predisposing causes of gout are the points
upon which the determination of the gouty nature of the inflammation
would depend. A termination in suppuration would exclude the idea of
the gouty nature of an arthritis.

With the arthropathies of purely nervous origin, such as occur in
paralyzed limbs, in Pott's disease, and in tabes dorsalis, gout can
hardly be confounded, although the arthritic complications in these
diseases have been used to illustrate the neurotic theory of both gout
and rheumatism.

The diagnosis of irregular gout--_i.e._ of gouty affections of the skin
and mucous membranes, of the structures of the eye, and of the
parenchymatous organs--must be based more upon the hereditary history
and upon the correlated phenomena recognized in the personal history
than upon any specific character in the lesions themselves. In the
gouty form of nephritis there are, it is true, in the urinary symptoms,
in the anæmia, in the arterial fibrosis, and in the cardiac
hypertrophy, diagnostic signs of great value.

PROGNOSIS.--Acute, regular, articular gout is probably never a fatal
disease where it occurs in a robust person without visceral
complications. In rare instances the first attack may never be
repeated, or only two or three attacks may occur in the course of a
long life. In the majority of instances, however, frequent repetitions
are the rule, the intervals between the attacks growing progressively
shorter; occasionally repeated seizures go on through a long life, the
attacks becoming milder with advancing years, and, save the crippling
effects of the disease, the patient may enjoy in the intervals a fair
degree of health. This, however, is the exception. With the increased
frequency of the arthritic attacks the signs of the constitutional vice
become more marked. The dyspeptic disorders become more persistent and
rebellious to treatment, various transformations of the disease
manifest themselves, and tissue-changes make insidious and inevitable
progress. When this stage of the gouty disease is reached, the
prognosis becomes more grave because of the complications and accidents
to which the sufferer is liable. These complications and accidents are
the result of the nervous, vascular, and visceral lesions which have
been {127} described. Vaso-motor instability gives rise to a great
variety of painful functional derangements resulting from serious
cerebral, pulmonary, gastric, and renal congestions. Glycosuria is not
an uncommon complication in chronic gout, and seriously affects the
question of prognosis. Arterial degenerations may cause thrombotic
accidents, and the formation of miliary aneurisms in the brain may
determine a fatal issue by softening or hemorrhage. Anginal attacks due
to cardiac muscular degeneration may also imperil life.

The principal visceral lesion which leads directly or indirectly to a
fatal issue in gout is that of the kidney. This involves danger either
through the induction of a hopeless anæmia and its consequences in
dropsical effusions, or by determining inflammatory accidents of the
gravest nature.

That gout shortens life in the majority of cases is unquestionable--a
fact which is sufficiently attested by the care with which
life-insurance companies exclude risks in which a well-pronounced
inherited tendency or existing manifestation of the disease can be
substantiated.

The prognosis varies of course with the rapidity with which the
constitutional dyscrasia is developed, and this rapidity will depend on
the intensity of the inheritance and the mode of life. Some gouty
subjects escape the vascular and visceral complications of the disease
for a long period, although crippled and deformed by its articular
ravages, and attain advanced age; others may succumb in comparative
youth to its most profound lesions. It is a happy circumstance that
under wise hygienic management and judicious medication acquired gout
may be checked in its progress, and even a strong inherited tendency
may be largely controlled.

TREATMENT.--A logical consideration of the treatment of gout embraces,
first, the treatment of the constitutional vice, based, as far as
possible, on the nature and causes of the disease; and, secondly, the
treatment of the lesions which the disease determines. If we regard the
accumulation in the blood-serum of the salts of uric acid as the
essential cause of the gouty lesions, then the origin of the
constitutional vice is in the conditions which bring about this
accumulation. As we have urged, none of the theories of the production
of the lithæmic state harmonize all its phenomena. It is impossible to
represent the complex processes of nutrition by chemical formulæ, and
equally impossible to divorce chemical reactions from a share in their
production. We can trace the metabolism of the azotized and
carbonaceous foods through many changes to their ultimate
disintegration into urea, carbonic acid, and water, but we do not know
all the steps by which this conversion is effected, nor the organs or
tissues in which it is accomplished. We may reasonably assume that the
agent through which the potential energy of the food is evolved is
oxygen, and that the process of nutrition is hence partly, at least, a
process of oxidation. This chemical view of the digestion and
assimilation of food may be said to be the rational basis of the
treatment of the lithæmic state. To control the accumulation of
azotized matters in the blood, and to secure their thorough combustion
and conversion into urea, carbonic acid, and water are the recognized
aims of the treatment of the vice upon which gout depends.

DIET.--The prevention of the accumulation of azotized matters in the
{128} blood involves, first, a consideration of the question of the
diet appropriate to the gouty dyscrasia. The almost uniform counsel
upon this point of all the authorities from Sydenham to the present
time is, that albuminous foods should be sparingly allowed in the diet
of the gouty patient, and that vegetable foods, especially the
farinaceous, should constitute the principal aliment. This counsel is
based upon the theory that uric acid is the offending substance, and,
this being the outcome of a nitrogenous diet, the nitrogenous element
in diet must be reduced. My own observation has led me to believe that
while this may be a legitimate deduction from the uric-acid theory of
gout, it is not supported by the results of clinical experience. If
there is one signal peculiarity in the digestive derangements of gouty
persons, it is their limited power to digest the carbohydrates, the
sugars and starches. In whatever form these foods are used, they are
more commonly the source of the dyspeptic troubles of sufferers from
gout than the albuminous foods. They provoke the acid and flatulent
dyspepsia which so generally precedes the explosion of the gouty
paroxysm; and it must have attracted the attention of every observer
who has studied the dyspeptic disorders of sufferers from inherited
gout, who have sought to control their unhappy heritage by abstemious
habits, that these disorders are especially provoked by over-indulgence
in saccharine and amylaceous foods.

It is not possible to explain satisfactorily why the lithæmic condition
should be induced by the carbonaceous aliments, but we believe there
can be no question as to the fact. If, as modern physiological
investigations tend to show, the liver is the organ in which urea as
well as glycogen is formed, it may be that the overtaxing of its
functions manifests itself more readily in the conversion of the
albuminous than in that of the carbonaceous foods; or it is possible
that the carbonaceous foods are destined chiefly for the evolution of
mechanical energy, and that when this destiny is not fulfilled through
indolence and imperfect oxygen-supply, they escape complete combustion,
and so vitiate the blood. But whatever may be the cause of this
anomaly, the clinical fact remains that in gouty persons the conversion
of the azotized foods is more complete with a minimum of carbohydrates
than it is with an excess of them--in other words, that one of the best
means of avoiding an accumulation of lithates in the blood is to
diminish the carbohydrates rather than the azotized foods.

The diet which a considerable experience has led me to adopt in the
treatment of the gouty dyscrasia is very similar to that which
glycosuria requires. The exclusion of the carbohydrates is of course
not so strict. Abstinence from all the fermented preparations of
alcohol is perhaps the most important restriction, on account of the
unfermented dextrin and sugar which they contain. This restriction
accords with the common experience respecting the part which wine and
beer play as predisposing causes of the gouty disease and as occasional
exciting causes of gouty lesions.

Next to the fermented liquors, the use of saccharine food in the diet
of gouty persons needs to be restricted. This limitation also is one
which common experience confirms. Sweet foods cannot be said to be as
provocative of the dyspeptic derangements of the lithæmic subjects as
wine and beer, but they are certainly often responsible for the
formation of {129} the dyscrasia and for perpetuating many most
distressing ailments. Their more or less strict prohibition may
constitute the essential point of treatment not only in controlling the
progress of the constitutional vice, but in subduing some of the most
rebellious lesions. It is important to observe that this prohibition
sometimes involves abstinence from sweet and subacid fruits, in the raw
as well as in the preserved state. Paroxysms of articular gout have
been known to follow indulgence in strawberries, apples, watermelons,
and grapes, and the cutaneous and mucous irritations which follow even
the most moderate use of these fruits in some gouty persons are
certainly not uncommon.

Next in order to the saccharine foods as the source of indigestion in
gouty persons come the amylaceous aliments. These constitute,
necessarily, so large an element in ordinary diet that the limitation
of them in the dietary of gouty persons applies, in the majority of
cases, only to their excessive use. This excessive use, however, is
often observed. There is a popular prejudice in favor of this class of
foods, and a corresponding prejudice against the too free indulgence in
animal foods. The purely starchy aliments, such as potatoes and the
preparations of corn and rice, and even those which contain a
considerable portion of gluten, like wheat, oatmeal, and barley, often
provoke in gouty subjects a great deal of mischievous and painful
indigestion. This feeble capacity for the digestion of farinaceous
foods is most frequently observed in the children of gouty parents, and
especially in persons inclined to obesity, and in those whose
occupations are sedentary and whose lives are passed for the most part
in-doors, and they are least common in those whom necessity or pleasure
leads to much active muscular exercise in the open air.

The fats are as a rule easily digested by gouty dyspeptics. This is a
fortunate circumstance, for the reason that in the anæmia which is
frequently one of the consequences of chronic gout the fatty foods are
of inestimable value. In cases of persistent and rebellious lithæmia an
exclusively milk diet constitutes a precious resource.

The succulent vegetables, such as tomatoes, cucumbers, cauliflower,
cabbage, and the different varieties of salads, constitute for the
gouty as well as the diabetic subject agreeable and wholesome additions
to a diet from which the starchy and saccharine vegetables have to be
largely excluded.

The quantity of food proper for gouty persons to consume can only be
determined in individual cases by the age, the habits, and the
occupation. It is fair to assume that in adults, in whom there is no
longer any provision to be made for growth, the daily quantity of food
must be regulated according to the amount of energy which is expended.
In this energy must be reckoned the amount necessary for the
maintenance of animal heat and the other vital functions, and the
amount which is necessary for the operation of every variety of nervous
force. In other words, the potential energy latent in the food must
correspond to the active energy exhibited in the daily evolution of
vital, intellectual, and mechanical work. The more nearly this balance
is maintained the more closely the physiological standard of health is
preserved. That an excess of food is a most frequent cause of the gouty
dyscrasia among the well-to-do classes is undeniable, and it is
possible that regulation of the quantity according to the rule above
mentioned {130} may, after all, be the most important point in the
management of many gouty patients. It may be, also, that the reason why
the withdrawal of the carbohydrates produces its good effects upon
these patients is that we thereby exclude a large amount of
force-producing foods which do mischief because they are imperfectly
consumed.

EXERCISE.--Next in importance to diet as a hygienic regulation in the
management of gouty patients is enforced exercise. The axiom of
Abernethy, "to live on a shilling a day, and earn it," comprises the
philosophy of the true relations of food to work, and of both to the
highest development of physical health. Exercise is to be enforced not
simply as a means of securing an active respiration, and thereby an
abundant supply of oxygen, but also as a means of converting the
potential energy of the food consumed into vital energy. The essential
condition, moreover, of healthy nutrition in every organ and in every
tissue is the maintenance of a vigorous functional activity. Over-use
is not more productive of tissue-degeneration than disuse. Hence the
question of exercise in its largest sense involves not only muscular
work, but work of all kinds, which tends to promote a healthy activity
of the psychical as well as the physical functions. Muscular exercise
in the open air has a special value for the victims of this gouty
dyscrasia by equalizing the circulation, quickening the respiratory
movements, and stimulating the elimination of effete matters from the
skin and lungs, but mental work and wholesome diversions are not less
important as antagonizing the evil effects of indolence and
over-feeding, which are among the common predisposing causes of
acquired gout. In persons who are incapacitated by neuræsthenia or by
excessive corpulence, the result of long indulgence in indolent and
luxurious habits, it may be necessary to resort to passive exercise by
rubbing, massage, and electrical excitation in order to secure the good
effects of voluntary work.

BATHING.--Another hygienic regulation of great value in the treatment
of gouty dyscrasia is the promotion by bathing and friction of the
eliminative function of the skin. Daily sponging with cold water, where
it is not contraindicated by a feeble circulation and a slow reaction
from the shock, is a practice to be commended. Where, for the reasons
mentioned, it is not practicable, tepid baths and frictions may be
substituted. In cases where the arthritic lesions are progressive and
advanced much benefit may be derived from hot baths. It is doubtful
whether the thermal alkaline and sulphur spas owe their renown in the
treatment of chronic gout so much to the mineral ingredients of their
springs as to their high temperature. The Russian and Turkish baths
furnish most efficient means for increasing the functional activity of
the skin, but they often have a depressing effect on the action of the
heart, producing faintness and dyspnoea, and should always be advised
with caution.

CLIMATE.--In rebellious forms of the gouty dyscrasia a warm climate is
unquestionably a hygienic condition of great value. The geographical
distribution of gout, which shows that the disease is much less common
in warm than in temperate and cold climates, while it may not perhaps
be wholly explained by temperature alone, is very certainly largely due
to it. The possibility of out-door life and the increased functional
activity of the skin which warm climates favor are circumstances more
or less antagonistic to the development of the gouty diathesis.

{131} MEDICINAL TREATMENT.--The objects to be aimed at in medicinal
treatment of the gouty dyscrasia are--

1st, the improvement of the primary digestion.

2d, the relief of the gastro-intestinal catarrh, which is the cause of
the direct and reflex dyspeptic symptoms which belong to this
diathesis.

3d, the augmentation of food-oxidation, so as to secure its thorough
combustion.

4th, the promotion of the elimination of the waste products of
nutrition.

1. The improvement of primary digestion--or, as it has been aptly
called, exterior digestion--often requires very strict attention beyond
the proper selection of alimentary substances. The distressing symptoms
that indicate primary gastric and intestinal indigestion are certainly
often relieved by the rigid exclusion of certain articles of diet, but
in many cases it is necessary to assist the preparatory processes which
are essential to perfect food-absorption by artificial methods based
upon the knowledge derived from physiological experiment. To no one is
the knowledge of these methods more largely due than to Roberts of
Manchester. Preparations of pepsin and pancreatin, by which the
proteids and starches are peptonized and the fats emulsified, are often
of inestimable value in the treatment of gouty dyspepsia. Pancreatin,
especially, which by means of its trypsin, diastase, and emulsive
ferment possesses the threefold property of aiding the digestion of the
azotized, amylaceous, and fatty elements of food, is certainly the most
valuable of the artificial means for augmenting the efficiency of
primary digestion.

2. The relief of the gastro-intestinal catarrh in gouty dyspeptics may
often be accomplished solely by dietetic restrictions and by the aid
which may be given to primary digestion. It is often necessary,
however, to direct some special medication toward the relief of the
catarrhal lesion. The circumstances which demand this special
medication are the existence of portal congestion, the result of
functional derangement, or of chronic atrophy of the liver, or of
chronic diffuse or interstitial nephritis, or of cardiac disease. The
hydragogues, such as calomel, podophyllin, colocynth, and other
vegetable cathartics, with the salts of sodium and magnesium,
constitute the most common and efficient means of relieving portal
congestion, whether it arise from temporary functional derangement or
from organic disease. The renown of some of the more famous mineral
springs in relieving the miseries of gouty sufferers is due mainly to
the relief of portal congestion and the washing away of the catarrhal
mucus which obstructs the process of primary food transformation and
absorption. This is especially true of the sulphate of sodium waters,
like those of Carlsbad, Marienbad, Friedrichshall, Pullna, and Hunyadi
János. While the value of these waters in chronic gout is
unquestionable where their use is properly regulated, there is good
reason to believe that their long-continued employment is often harmful
by relaxing the mucous membrane, and thereby tending to aggravate the
condition they are given to relieve. This is markedly true of their use
in weak and anæmic persons. For these the milder magnesian waters, such
as those of Kissengen, Hombourg, Wiesbaden, and Saratoga, are to be
preferred.

3. The augmentation of food-oxidation may be accomplished in a large
degree by regulation of the diet and by out-door exercise. The {132}
regulation of the diet according to the occupation and habits of life
is a point of primary importance in securing proper blood-elaboration.
My experience leads me to believe that the evil consequences of in-door
occupations and sedentary habits are most common in those who live upon
a diet composed largely of starchy and saccharine foods, and that a
diet in which animal foods and fats predominate is best suited to
indoor workers, whether they be engaged in mechanical or intellectual
labor.

The medicines which help to promote the oxidation of the food-elements,
especially the carbohydrates, are alkalies and iron. Clinical
observation establishes this fact as strongly in the treatment of gout
as in that of glycosuria. The relative power of the salts of potassium
and sodium in augmenting oxidation is not clearly determined. The salts
of sodium appear to be most useful in aiding the process of primary
digestion, and the potassium salts in improving the process of
sanguification. It is well known that potash predominates in the
corpuscles and soda in the serum of the blood. The efficacy of the
combinations of iron with the salts of potassium, as in Blaud's pills
and in the citrate and tartrate of iron and potassium, in the treatment
of anæmia, is well known. In the most renowned ferruginous springs,
however, such as those of Schwalbach, Spa, Pyrmont, and St. Moritz, the
iron is combined with salts of sodium, calcium, and magnesium. It would
appear, therefore, that the increased energy of iron in augmenting
hæmatosis, when combined with alkalies, is not relatively greater with
potash than with either of the other alkaline bases.

4. The promotion of the elimination of the waste products of nutrition
is to be accomplished by remedies which act as solvents of uric acid
and as diuretics. As solvents of uric acid the salts of lithia and
potash have been shown to be superior to those of soda. The urate of
lithia is the most soluble of the uric-acid salts, and the low chemical
equivalent of the metal lithium makes the neutralizing power of the
oxide much greater than that of equal proportions of the other
alkalies. It is used in the forms of carbonate and citrate, and is
generally combined with potash and soda. It exists in some of the
mineral springs of Europe and of this country, but in such minute
proportion as probably to be of little value. In administering the
salts of potash and soda it is generally admitted that the carbonates
and the neutral salts of the organic acids are to be preferred to
solutions of the caustic alkalies. They have less power in neutralizing
the acid of the gastric juice, and enter the circulation as neutral
salts, where they are decomposed into alkaline carbonates by the
oxidation of the organic acids, increasing the alkalinity of the serum
and acting as diuretics. The combinations of the alkalies with sulphur,
with iodine, and with mineral acids, as in the alkaline springs, are
frequently used in the treatment of gouty lesions of the subacute
variety. The sulphur salts probably owe their chief value to their
alkaline bases when they are used internally; and in sulphuretted
baths, as before remarked, the good effects are probably due to the
high temperature at which the bath is usually administered.

The salts of iodine are generally supposed to have a special action in
removing the consequences of chronic fibrous inflammation in gout and
rheumatism. They often disturb the digestion and provoke troublesome
irritations of the skin and mucous membranes. In removing the sclerotic
{133} effects of gouty inflammation they do not exhibit the same
sorbefacient power which they show in their action upon the granulation
tissue of syphilitic origin. It must be admitted, however, that in
certain catarrhal affections of a gouty nature the iodides of potassium
and sodium are almost specific in their good effects. In the
pharyngeal, laryngeal, and bronchial catarrhs from which some gouty
persons suffer, where there is a dryness and irritability of the mucous
membrane, the administration of these salts produces the most prompt
and beneficial result. As solvents of uric acid they do not appear to
equal the salts of the organic acids.

As to the mode of administering salines in the treatment of the gouty
dyscrasia, it is hardly necessary to observe that it must vary with the
effect desired. As antacids in acid dyspepsia they should be given soon
after meals, and for this purpose the salts of soda are to be
preferred, for the reason that they not only neutralize excessive
acidity, but they increase the efficiency of the peptonizing process.
Where it is desired to introduce these salts into the circulation for
their solvent action, as diuretics or to assist the process of
sanguification, they should be given three or four hours after meals
and largely diluted with water.

Before concluding the consideration of the treatment of the gouty
dyscrasia it should be remarked that the ability of water as a solvent,
as a means of stimulating tissue-changes and eliminating waste, is not
generally estimated at its true value. The use of copious libations of
hot water in the treatment of gout, recommended by Cadet de Vaux in
1825, has been revived from time to time, and is at present attracting
considerable attention.

TREATMENT OF ACUTE ARTICULAR GOUT.--There are three distinct methods of
managing an attack of acute gout--the antiphlogistic, the expectant,
and the abortive.

The antiphlogistic method, in the strict application of the term, is
practically obsolete. Bloodletting, both general and local, brisk
catharsis and diaphoresis, with low diet, were formerly advocated as
the natural and imperative antagonists of gout as well as of all other
acute inflammatory affections. Carried to its extreme degree, this
method was deprecated by Sydenham and his disciples as tending often to
prolong the attack and precipitate the manifestations of atonic gout.
The natural reaction from the vigorous antiphlogistic practice was what
has been termed the expectant method.

The expectant method may be said to be founded upon the aphorism of
Mead that "gout is the cure of gout." The discovery of the salts of
uric acid in the blood-serum and in the affected tissues gave a
scientific basis to the humoral pathology of gout and led to the
formulation of definite principles in the application of the expectant
method of treatment. These principles are the prevention of the further
accumulation of the urates in the blood and the promotion of their
oxidation and elimination. The first principle involves restriction to
a rigid diet during the attack, excluding albuminous foods and the
fermented preparations of alcohol, and allowing only milk and
farinaceous gruels. The oxidation of the urates is encouraged by the
administration of alkalies and by an abundant supply of air, the
inhalation of oxygen even having been recommended. The elimination of
the urates is accomplished chiefly {134} by diuretics and moderate
catharsis. The local treatment commonly used with this medication
consists in the application of alkaline and anodyne fomentations or of
dry flannel or cotton. Local bloodletting and blistering are now rarely
commended. Under this treatment the intensity of the inflammatory
process is abated, the suffering is allayed, but the progress and
duration of the disease are not materially modified. The recovery,
however, is satisfactory, and it is claimed that the chances of early
recurrence of the attack are diminished. This method has many
advocates, though it cannot be said to represent the common practice of
the present day. It is becoming traditional, and may be said to be
gradually giving place to the specific or abortive method.

The abortive method consists in cutting short the attack by the
administration of colchicum, veratria, or the salicin compounds.

The value of colchicum in joint affections is a tradition of the
earliest records of medicine. It shares its curative effects in acute
gout with veratria, and, though the active principle of the meadow
saffron and the veratrum album are not isomeric, their effects are
similar. They constitute the basis of the famous nostrums so
extensively patronized by sufferers from gout. Colchicum is the active
agent in the eau médicinale de Husson, in Wilson's and Reynolds's
specifics, and in the pills of Lartigue and Blair, while veratria is
supposed to be that of Laville's remedy. The action of these substances
is not understood. The physiological action of colchicum is that of a
local irritant and a cardiac depressant of great energy. It purges
violently when given in large doses, causes nausea and vomiting, and
may produce collapse. In therapeutical doses in a gouty paroxysm it
acts as a diuretic and an antipyretic, and allays, sometimes in a most
magical manner, the objective and subjective symptoms of the disease.
As simple purging by other cathartics does not abort the gouty seizure,
the value of colchicum cannot be ascribed to its purging effect, and,
besides, purging is by no means necessary to its efficiency. Nor can
its utility be ascribed to its diuretic property. There is some
question in regard to its claims as a diuretic, and there seems to be
no doubt that it often does good where this effect is not observed. Its
influence upon the heart does not explain its marvellous action upon
the local process, for the same influence obtained by other drugs has
no such result. We are driven, therefore, to the conclusion that
colchicum has a specific action in gout as certain and as inexplicable
as that of quinia in malarial fever, or iodide of potassium in
constitutional syphilis. For those who accept the theory that gout is a
tropho-neurosis the therapeutical action of colchicum is a strong
confirmation of its neurotic origin, for the reasons that colchicum has
no influence upon arthritic lesions which are not gouty, and that its
physiological effects point to its action on the nervous system.

It is useless, however, to speculate on the way in which colchicum and
allied substances affect gouty inflammation; the practical question to
be determined is: Are they the best and safest remedies to control it?
Upon this point there is a wide diversity of opinion. The objections to
the colchicum treatment are based upon humoral pathology, and upon the
idea that the attack is an effort of nature to cast out the poison and
purify the blood. Colchicum, it is claimed, arrests this process; the
poison is retained, diffuses itself through the tissues, and lays the
{135} foundation of vascular and visceral lesions. It shortens the
intervals between the attacks, and tempts the patient to continued
indulgence in the habits which perpetuate and exaggerate the disease.
The advocates of the abortive treatment, on the other hand, claim that
these arguments have no real force as applied to its therapeutical
value. The cure accomplished is, to all appearances, complete, and the
patient is saved the suffering and exhaustion which result from the
expectant method. The fact that he is so easily and speedily cured, and
that he resumes his vicious habits and suffers recurring attacks in
consequence, proves only that the treatment lacks the quality of moral
discipline which belongs to prolonged suffering and the penance of
vigorous medication. It is an acknowledged fact that the great majority
of sufferers from acute gout decide sooner or later in favor of the
abortive treatment; and as professional opinion has heretofore
generally advocated the expectant or eliminative treatment, they
commonly resort to the use of some one of the quack remedies which
contain colchicum or veratria.

In view of the present uncertainty of our knowledge of the true
pathology of the acute gouty arthritis, as to whether it is a
tropho-neurosis or the result of the local irritation caused by the
salts of uric acid, the specific treatment seems to be justified by a
regard for the comfort of the patient and as a means of protecting him
against falling into the reckless use of quack remedies. A speedy
relief of the acute symptoms, followed by the treatment appropriate to
the gouty habit, would seem to be the most rational and safest mode of
managing the acute articular attacks of gout.

The selection of the preparation of colchicum in the treatment of an
acute paroxysm is a matter of individual experience and preference. The
acetous extract and the wine of the seeds are most commonly used, and
many practitioners are not scrupulous in prescribing the proprietary
preparations of Reynolds, Laville, and Blair. The wine of colchicum may
be given in doses varying from 20 to 40 minims, alone or combined with
Epsom salts in drachm doses, with small quantities of opium, every six
or eight hours. Under this medication the pain, tenderness, and
swelling rapidly abate, and sometimes with an abruptness that is
magical. As soon as the acute symptoms subside, the colchicum should be
continued in smaller and less frequent doses until the fever and local
tenderness subside. The use of quinia with small doses of colocynth
after the colchicum has been discontinued helps to re-establish the
strength and regulate the digestive functions. The patient should
always be warned against the possible demoralizing effects of a speedy
recovery from a serious disease. Recurrence after the colchicum
treatment is certainly more common than after the expectant method, but
this should not be ascribed so much to a defective cure as to the
temptation which the antidote offers to trifling with the poison. The
accidents which have been ascribed to colchicum through its causing
heart-failure are probably to be explained by its injudicious
administration in large doses where acute gout is complicated with
cardiac or renal degeneration.

Next in importance and value to colchicum in the abortive treatment of
gout are salicin, salicylic acid, the sodium salicylate, and the oil of
wintergreen. Unlike colchicum, which has no marked effect upon acute
rheumatism, these medicines appear to act with similar energy on {136}
gout and rheumatism. The rapidity and the almost uniform way with which
they allay the inflammatory symptoms in rheumatic fever are well known;
their value as specific remedies in both acute and subacute gout is not
so generally appreciated. Whether the specific action of colchicum in
gout differentiates this disease from rheumatism, or whether the
similar action of the salicin compounds indicates that these diseases
are allied in their etiology, are questions yet to be solved. The good
effects of salicin and the sodium salicylate in many of the forms of
irregular gout, and notably in the dyspeptic disorders and the
erythematous tegumentary lesions, are especially worthy of notice. In
acute attacks of articular gout the salicylic acid or the sodium
salicylate, in 15 or 20 grain doses repeated every three or four hours,
will often cut short the attack, and will very certainly allay within
twenty-four hours the acuteness of the symptoms. As in rheumatism, the
medicine should be continued in smaller doses after the acute symptoms
have subsided for several days, the tendency to relapse being marked if
the drug be discontinued too soon. In subacute articular gout and in
the irregular forms of the disease, where the medicine has to be
continued for some time, salicin and the oil of wintergreen are to be
preferred to salicylic acid and the sodium salicylate. They are less
liable to disturb the stomach and to produce toxic effects.

It is unnecessary to describe the treatment of the different forms of
irregular gout, inasmuch as the general principles described in the
treatment of the gouty dyscrasia involve the most important
considerations in the management of these affections.



{137}

RACHITIS.[1]

BY A. JACOBI, M.D.

[Footnote 1: There is a difference of opinion as to the correct
spelling of this word, and strong reasons exist to regard the form
_rhachitis_ as the proper one. It is true that this spelling of the
word has been remarked upon as unorthographical by many, mostly modern,
authors. Even Virchow writes "Rachitis," claiming that Glisson took the
term from "the then popular _rickets_." This is a mistake, as H.
Rohlffs points out (_Deutsches Arch. f. Gesch. d. Med._, 1883, p. 294).
Rachitis is a Greek word, and was used in the classical time of
Hellenism. It has, however, seemed best to preserve here the usual
spelling, rachitis, which has become sanctioned by general usage.]


DEFINITION.--Rachitis is a general nutritive disorder, almost always of
long duration, usually with an introductory stage of weeks or months
and a course mostly extending over months or years. Its beginning is
mostly gradual, its final recovery slow. It is complicated with or
dependent on disorders of the digestive or respiratory apparatuses,
which are preceded by a disposition probably created by an undue width
of the arteries. It exhibits amongst its prominent symptoms muscular
debility; perspiration; anomalies of the subcutaneous tissue, which is
either very much infiltrated with fat or deprived of it; disturbances
of the intellectual and moral functions, and of those of the large
thoracic and abdominal viscera and lymphatic glands; changes in the
latter may outlive all others. Its most perceptible symptom, however,
consists in an inflammatory disease of the primordial cartilage of the
epiphyses, a copious deposit in that region and also under the
periosteum of the bones; curvature of the diaphyses, and, while
absorption remains intact, softening and retarded ossification of the
bone. Without these affections of the osseous system the diagnosis of
rachitis is not complete.

ETIOLOGY AND PATHOLOGY.--The nature of rachitis has been considered to
be inflammatory by F. A. Walter.[2] Renard looked for that inflammation
in the periosteum. Guérin emphasizes the vascular increase in
periosteum, bone, and marrow; Trousseau and Lasègue the congestive
character of the local tumefaction, besides fever and pain. Virchow
also[3] inclines to the opinion that the rachitical process is of an
inflammatory nature, though it be impossible to state the exact cause
of the process. Still, he claims that we are no better off in regard to
other inflammations of unknown character--for instance, those of the
skin--and that we have to look for a future increase of our knowledge
of such constitutional predisposition of the organism and of such
specific qualities of the blood as will produce the local irritation of
the osseous tissue in rachitis. Last, and mainly, it is Kassowitz who
seeks the {138} essence of the rachitical process in a chronic
inflammation originating in the points of apposition of the growing
bones of the foetus or infant. During the chronic inflammation
blood-vessels are formed in large numbers, and a morbid congestion
takes place in all blood-vessels, but mainly in those of the localities
in which new bone is forming; thus in the chondro-epiphyses, in the
perichondrium and periosteum, and the sutural substances. Faulty
introduction or elimination of lime has nothing to do with this
process. It cannot be deposited in the current of a copious
circulation; in fact, it is not deposited in the immediate neighborhood
of blood-vessels to any extent. Even in otherwise normal bone hyperæmia
produced by the experimenter softens the bone, which was fully formed
before. If the relative percentage of lime were of any account in the
etiology of rachitis, the periosteal and cartilaginous proliferations
would find no explanation. But why is it that this peculiar process
takes place at an early age only? and in the bone only? Kassowitz urges
the fact that the growth of the bone differs in this from the
development of all other tissues: that the latter grow uniformly
through their whole mass; that the circulation in them is more uniform
and carries material through and into every particle simultaneously,
while in the bones the only places in which the whole circulation can
contribute to their growth--the few blood-vessels distributed in the
interior not adding to their growth at all--are the periosteum and the
places of apposition between epiphysis and diaphysis. Every morbid
irritation, whether resulting from bad air, habitation, and food, or
from either chronic or acute ailment, acts on the whole mass of other
tissues and organs, but in the bones only on the growing ends or
surface.

[Footnote 2: _Anatom. Museum_, Berlin, 1796, vol. ii.]

[Footnote 3: _Arch. f. Path. Anat._, vol. v.]

The results of the pathologists and experimenters are confirmed by
chemical analyses. Fat has been generally found somewhat increased in
the rachitical bones, and water largely so; chondrin is diminished
according to Marchand and Lehmann, but was found unaltered in the later
analyses of A. Baginsky. The latter found, after having deprived the
bone of fat, the organic and inorganic material to be in a proportion
of 100 to 563 in the normal, and of 100 to 160 in the rachitical
osseous tissue; and in 100 parts of dry bone, Gorup-Besanez found in
the

                       Ossein. Phosphoric Acid. Lime. Manganese Oxide.
  Healthy adult         34           26          34        0.3
  Infant of six months  34.9         27          35        0.5
  Rachitical femur      72            7           9        0.3
      "      tibia      60           12.9        17        0.3

Defective calcification of the forming bone is one of the principal
characteristics of rachitis. In it lime cannot either enter into the
composition of the osseous tissue or remain in it. Its elimination must
take place either through the kidneys or the intestinal tract. In the
feces Ad. Baginsky, and many before him, have found an abnormal
quantity. In regard to the urine, modern investigations do not agree
with former analyses. Thus, Baginsky concludes that there is no
increase of lime in the urine of rachitical as compared with that of
healthy children; Seemann found even a diminution of the percentage of
lime. Amongst modern writers only Rehn found an occasional increase of
lime in the urine of rachitis.

{139} In regard to the elimination of phosphoric acid, the analyses of
different periods do not agree any better. The conclusions of previous
researches, pointing to a quadruple elimination of phosphoric acid in
the urine of rachitis, are refuted by Seemann, who found no increase,
and by Baginsky, according to whose researches the phosphoric acid of
the healthy urine compares with that of rachitical urine as 40:12-37.

As far as the elimination of nitrogen is concerned, there appears to be
but little difference between normal and rachitical urine. Chlorine was
found to be diminished in rachitis by Baginsky. Lehmann and Von Gorup
found lactic acid several times. Several times albumen was met with; in
a case of Ritchie's, blood; in one of Von Gorup's, fat.[4]

[Footnote 4: E. Salkowski und W. Leube, _Die Lehre vom Harn_, 1882, p.
536.]

The etiology of rachitis must be studied from two points of view. It
has its predisposition and its direct and proximate causes. The former
has been studied by F. W. Beneke[5] upon an anatomical basis. He finds
that the arteries of rachitical patients are large all through the
body. This is so particularly in the carotids; it seems probable that
the changes taking place in the head are due to this anomaly in the
size of the arteries. Three cases in which the width of the arteries of
the neck was unusually large terminated fatally--one by hydrocephalus,
one with a very large skull, and one suddenly. This width of the
arteries is most marked, under ordinary circumstances, from the second
to the fourth year; that is, the exact time in which (except the cases
of early rachitis) the rachitical process is at its height. It is
considered by Beneke to be the cause of the local increase of vascular
irritation, particularly in the epiphyses with their retarded
circulation; and also of the increase of nutritive development which is
so often noticed during recovery from rachitis; and, finally, of the
many pulmonary complications of an inflammatory nature.

[Footnote 5: _Die Anatomischen Grundlagen der Constitutions Anomalien
des Menschen_, 1878, p. 75, etc.]

There is another interesting consideration in regard to the effect of
wide arteries on the relations between the blood and tissues. A great
many more blood-cells are required to fill the arteries when wide than
when narrow. Now, the formation of blood-cells is hindered by any
disease of the digestive and blood-preparing organs, so that the
tissues are liable to show the relative increase in the percentage of
water, which is uniformly confirmed for rachitis by the biochemists.

The pulmonary artery of the healthy infant is larger than the aorta by
not more than four millimeters. In the majority of cases of rachitis
examined by Beneke this difference in size was very much more favorable
to the pulmonary artery; it is abnormally large in rachitis. This
anatomical fact is suggestive of the pathological processes so
frequently found in the lungs and in the neighboring lymphatic and
large abdominal glands. For, while the amount of blood introduced into
the lungs through its wide artery is unusually large, particularly so
in a chest which is contracted in consequence of the rachitical process
in the bones, the exit from the lungs is relatively impeded. Not only,
however, the narrowness of the chest is a cause of this disproportion.
For even in rather normal chests the lungs of rachitical children are
relatively small.

The liver of almost all rachitical children is large. In but one-half
{140} of the cases this enlargement is accompanied with a large heart.
In pure cases of scrofula, on the contrary, Beneke found a small heart,
rather narrow arteries, and usually a small liver, the size of the
lungs offering but few anomalies.

The spleen also is large in the majority of cases. Its size is not
dependent on the large size of the liver or the small size of the
lungs. For these conditions are found in the majority of cases only,
not in all of them, and the large spleen is not always found with a
large liver and small lungs. The variability of the anatomical
conditions permits of various degrees of combination; so that varying
combinations of rachitis with other constitutional disorders may
correspond with the different sizes of the principal organs. After all,
as there is a great deal of independence of these organs, as to size,
of each other, the conclusion is justified that those differences are
not the result of the disease, but that they are congenital and stand
in some causal relation with it.

The kidneys are large in the majority of cases, like the spleen and
liver, while the lungs are small. This disproportion is apt to result
in a hyperæmic condition of all the organs of the abdominal cavity, and
especially of the kidneys. To what extent this undue amount of volume
interferes with, or increases, renal secretion, it is difficult to say.
The amount of urine secreted by rachitical children is about normal,
though, as already stated, the percentage of lime in it is rather
diminished, contrary to the opinions held formerly.

For the direct cause of rachitis Glisson looked to the inequality of
nutrition by the arterial blood, and for that of the curvature of the
long bones to their superabundant vascularization. He found the disease
mainly amongst the well-to-do classes, not unlike a modern American
writer, who declares infantile paralysis to be the result of the
nervousness of the better classes of the American people! John Mayow
(1761) held a disturbance of the innervation responsible; Zeviani (in
the same year), improper food in general, and particularly prolonged
lactation; and Selle (1791), a peculiar diathesis (acrimonia
rachitica). About that time a defective nutrition with abnormal
function of the lymph-ducts was looked upon as the cause of rachitis by
many--by others, an undue production of acid, and the softening of the
osseous tissue thereby. This result was attributed by some to the
influence of milk (Veirac, De Krzowitz). Attention was directed at an
early time to phosphoric acid and lime, with the view that variations
in the elimination of these substances might explain the occurrence of
rachitis. A large quantity of both was found in some urines (Malfatti);
a superabundance of phosphoric acid was presumed to prevail in the
whole system (Wendt, Fourcroy); while symptoms resembling rachitis were
found in animals fed upon small doses of phosphoric acid by Caspari
(1824). Chossat fed young animals on food deprived of lime, and claimed
to produce softening of the bones and death, a result which was denied
by Friedleben. Guérin claimed to produce rachitis by feeding young
animals on meat in place of their mother's milk, a result equally
denied by Tripier, who, like Friedleben, found the bones under such
circumstances more liable to fractures, but not rachitical. Wildt and
Weiske found the bones uninfluenced by withholding lime from food;
Forster, however, and Roloff claimed to notice a marked influence, and
the latter {141} stated that animals, after having been rendered sick
by depriving them of lime, recovered when they were again supplied with
it. Wegner, in his numerous experiments with phosphorus, found that in
growing animals it increases the growth and firmness of both long and
flat bones; after the growth of the animal has been completed it
renders epiphyses and vertebræ denser. There is no change, however, in
the relative chemical composition of those parts. He found at the same
time that results similar to those caused by the administration of
phosphorus were obtained when food deprived of its phosphate of lime
was given. But he met with no rachitical changes proper during these
several procedures. Teissier having found an increase in the urine of
rachitis after the administration of lactic acid, and lactic acid
having been frequently found in the urine of rachitical patients by
Ragsky, Morehead, Simon, and Lehmann, C. Heitzmann fed with lactic acid
both carnivorous and herbivorous animals, found the cortical layer of
the bones softened and the medullary substance hyperæmic, and claimed
to produce rachitis in the former and osteomalacia in the latter. Both
of these assertions were denied by Tripier and Toussaint, who insist
upon Heitzmann's having selected animals which have a peculiar
disposition to suffer from rachitis. Again, Milne Edwards and
Boussaingault found the bones softened when they withdrew both
phosphoric acid and lime from the food, without restoring the bone's
consistency by administering powdered bone. But, lately, Ad. Baginsky
states that he produced rachitis by withholding lime, and increased the
effect by introducing lactic acid. By so doing, however, he changed
only the relation of the mineral to the organic substances, without
interfering with the normal proportions to each other of the
constituents of the ashes. Beneke, finding oxalic acid in the urine in
many cases of rachitis, attributes to it the want of calcification in
rachitis, and Senator suggests that what impedes the deposition of bone
might be formic, acetic, and lactic acids, which are also found in the
young osseous tissue.[6]

[Footnote 6: L. Fürth, _Path u. Ther. d. Rachitis_, Wiener Klinik,
1882.]

Of these statements many are uniform, others contradictory. Thus far,
they are not convincing except in one way--viz. that both withholding
and introducing certain ingredients, mainly lime, influence the growth
of the bone considerably. This may prove nothing else but that lime is
of paramount importance in the building up of bone, and that bone in
the period of rapid development is amenable to a great many influences.

It is in the period of rapid development that rachitis is observed.
Thus it occurs in every stage of intra-uterine and infant life. It is
met with in the foetus in very early intra-uterine life; it is found as
a congenital affection, continuing to develop after birth when it has
originated in the latter half of foetal existence; there is, thirdly,
the rachitis of early infancy; and, lastly, that of advanced infancy
and childhood. Of 624 cases of rachitis enumerated by A. Baginsky,
there were 256 less than a year old, 313 in the second, and 63 in the
third year. After this time rachitis is rare, as far as the active
symptoms of the disease are concerned. But still, a retarded form of
rachitis (r. tardiva) has been described by some authors. It is said to
occur about puberty, and to exhibit local changes in the bones of
genuine rachitical character, but to be wanting in all the other
symptoms required for the diagnosis of general rachitis. Such cases
have been described {142} by C. Lucas.[7] He found it complicated, now
and then, with albuminuria. The occurrence of the latter at that time
of life had been referred to by Moxon.[8] The principal symptoms
described by Lucas are scoliosis, talipes valgus, and genu valgum. The
epiphyses were slightly thickened; there were pain in the limbs,
languor, and pallor. In some of the cases there were also rachitical
deformities dating from infancy. He believes rachitis of adolescence to
exhibit more symptoms belonging to relaxation of the ligaments than to
softening of the bones.

[Footnote 7: _Lancet_, June 9, 1883.]

[Footnote 8: _Guy's Hosp. Rep._, 1878.]

A case of rachitis of undoubtedly congenital nature has been reported
by Chiari. There were but twelve teeth. There were no other alveoli,
nor was there any intimation of the formation of alveoli in the shape
of the jaw, which resembled very much the usual senile form of
retrograde metamorphosis.

Twenty years ago I described the lesions in part of a rachitical
cranium removed from an infant who lived up to her eleventh day. She
was born at full term with hernia of the brain, about one-sixth of
which protruded through the small fontanel. Only the cranium could be
studied with regard to rachitis, and but small portions of the frontal
and the anterior half of the parietal bones surrounding the large
fontanel could be removed. In these few square inches of bone there
were between twenty-five and thirty openings of the usual craniotabic
nature, nothing but a transparent membrane being left. The bony edges
of these thin portions were partly sloping off gradually, partly very
steeply, and somewhat thickened. They were distributed over the whole
part of the skull removed; some were found in the immediate
neighborhood of the points of ossification. No recent deposits of soft
rachitical bone had taken place under the periosteum. Thus, evidently,
the process was of rather an early date of intra-uterine life, and had
at birth run the full course of its usual development without having
had an opportunity to terminate in the restitution of the normal
bone.[9]

[Footnote 9: _Amer. Jour. Obst._, Nov., 1870.]

In a case reported by Dr. F. A. Burrall[10] the infant (female) was
cyanosed at birth, and had a small head and feeble general development.
The respiration was shrill and piping from birth, as though from
congenital laryngismus; in a few days it became raucous. The
post-mortem examination proved the larynx normal, with no obstructive
growths. She was pigeon-breasted, and the last phalanx of her right
finger wanting.

[Footnote 10: _Trans. N.Y. Path. Soc._, vol. i. p. 81.]

In the meeting of June 27, 1883, of the Société de Chirurgie of Paris,
Guéniot presented a newly-born baby with well-pronounced rachitis of
the extremities which had healed at the time of birth. The bones had
recovered their firmness, and the characteristic deformities remained.
In the meeting of December 19th he could report that the child had
exhibited neither symptoms of rachitis nor of syphilis since. In regard
to the latter, a very rigorous examination of the baby's whole family,
made by Guéniot and Fournier, resulted in the existence of no trace of
syphilis.[11]

[Footnote 11: _Rev. Mens. des Mal. de l'Enfance_, Janv., 1884.]

Kassowitz has examined many still-born infants, and also children dying
at an early age, at the foundling hospital of Vienna. In a large
majority of the cases he found rachitical changes in the ends of the
bones. In {143} many of those who lived several weeks he found rachitis
developed to such an extent that the presumption of its intra-uterine
origin became conclusive. Here nothing is left but the conclusion that
the cause of congenital rachitis has to be looked for in the condition
of the maternal blood.

Thus, the foetal and congenital occurrence of rachitis cannot be
doubted. Both forms are represented in literature. Neither requires the
presence of rachitis in one or both of the parents. But the cause of
the intra-uterine disease has not been found. Perhaps a disease of the
mother with considerable nutritive disorders or a defective placentar
supply may be found responsible. The foetal form runs its course long
before the normal termination of pregnancy; the congenital may have run
its full course at birth or complete it afterward. The bones are found
of characteristic nature, the diaphyses suffering more than the
epiphyses; even a rachitical pelvis has been met with by Fischer. Early
foetal rachitis is probably dependent upon a defective development of
the very first cartilaginous deposits and the first osseous nuclei;
thus, many of the congenital synostoses find a ready explanation.
Besides these, abnormal circulation is accounted for. For periosteal
proliferation at that early period contracts the foramina carrying the
blood-vessels, and, while interfering with the size of the bones, the
foramen magnum also. Thus, a certain class of cretinism appears to be
due to foetal rachitis, mainly of the base of the cranium, which
results in early ossification of the synchondroses, particularly of the
sphenoid bone. But lately I have seen a case of this description,
which, however, had not terminated at the time of birth. For after
birth the rachitical process developed further, and in addition to the
rachitical deformity of the base of the cranium there were afterward
thickening of the epiphyses, pigeon breast, and thoracic grooving and
flattening.

Rachitis is found in city and country, less on mountains than in
valleys. Still, it is met with at elevations of two thousand feet. In
the tropical regions it is almost unknown. Why it should have been
considered quite a new disease in England but a few centuries ago, or
whether it did not exist before that time, it is difficult to say. It
is certain, however, that deformities have been described in antiquity
which we are accustomed to attribute to rachitis.

As the disease is one that occurs during the period of rapid growth,
and is a developmental disease, everything that interferes with normal
growth and development is apt to change physiological functions into
pathological conditions and to produce rachitis. In the pregnant mother
her ill-nutrition and the defective cell-material used in the building
up of the embryo and foetus, or a defective placenta, may come in for
the explanation of foetal and congenital rachitis, although the case of
Klein's, who reports twins, of which one was normal and one rachitic,
is rather difficult to explain on that basis only. Even rachitis of
early infancy is not easily accounted for otherwise, for its first
symptoms show themselves at a very early period; thus constipation,
adiposity, and afterward craniotabes and thoracic grooving.

The common form, and that which is the usual subject of the text-books
and monographs, has, however, in most cases a well-marked preparatory
stage in the shape of diseases or ailments reducing sanguification
{144} and nutrition. Some cases are ushered in by, or follow the course
of, acute exanthems or acute gastric disorders or the presence of
entozoa. A larger number appear to result from insufficient oxygenation
resulting from lung diseases, with a long chronic ailment following the
acute stage. Even acute pneumonia, with its direct influence on general
nutrition, stands often for the proximate cause of rachitis. Bad air
alone, even swamp air, does not appear to be a sufficient cause. When
it seems so, it is complicated with the main cause of rachitis; that
is, bad, insufficient, improper food, with its immediate result--viz.
intestinal catarrh. Cow's milk, particularly when acid, starchy food
administered too early or in too large quantity or too exclusively,
early weaning followed by improper artificial food, insufficient
mother's milk or such as is either too thin or too caseinous, lactation
protracted beyond the normal limit,--may all alike be causes of
intestinal disturbances and rachitis.

Is rachitis hereditary? A number of women who were rachitical
themselves have been known to have rachitical children. But it has been
said that the process runs its full course during infancy, and that
therefore a direct inheritance of rachitis from mother to child is an
impossibility. Still, we must not forget that the consecutive
conditions of the parents may, or will, influence the general condition
of the infant and result in similar disturbances. No rule, however,
exists. Dyscrasic parents may have healthy children, and healthy
parents sickly or dyscrasic ones. But the probability is greater that
diseased children should come from dyscrasic parents than from healthy
ones. Tuberculosis in the parents has frequently been accused of being
the cause of rachitis in the infant--not directly, but in consequence
of general impairment of the tissues. Gout has also been accused of
being the cause of rachitis, but it is a peculiar fact that the poor
have but little gout and a great deal of rachitis. In all of these
cases it is better to look upon rachitis as only one of the forms of
general mal-nutrition, and to speak of inheritance of the disposition
rather than of the disease. Thus it was that about the end of the
eighteenth century Portal spoke of scrofulous, syphilitic, scorbutic,
rheumatic, arthritic, and exanthematic rachitis. Particularly has
syphilis been accused of being the main cause of rachitis by some, and
even the only cause by others. Thus it was looked upon by Boerhaave. In
modern times Parrot maintained, from 1872 up to the time of his death,
which occurred recently, that every case of rachitis is of syphilitic
origin. As his proof he relied mainly on the condition of the teeth and
the bones. But those appearances in the teeth, the thin and ragged
edges, the friability and the grooving, either horizontal or vertical,
which have been considered characteristic of syphilis by Hutchinson and
others, have no such dignity, and moreover they are not observed in the
temporary teeth at all, but in the permanent only; the rachitical
softening of the bones also is not found in syphilis at all.
Particularly are there no curvatures in syphilis and no infractions. It
is true that marasmus is found in both rachitis and syphilis, but it is
met with in all sorts of diseases. The changes in the bones of syphilis
are found at birth; in rachitis they usually develop in later months.
When a baby is syphilitic and rachitic at the same time, the syphilis
may last very much longer than the rachitis, which meanwhile has
healed. The internal organs in rachitis do not exhibit any such {145}
changes as are known to occur in very many cases of syphilis. No
gummata are ever found in rachitis, and the interstitial inflammation
of the internal organs in syphilis is not met with to the same degree
in rachitis. What Parrot claimed as a desquamative syphilide of the
tongue--that is, red insulated spots, denuded of their epithelium,
small in the beginning, later extending backward and increasing in
size--is by no means always syphilitic, but is found in a great many
cases where there is no suspicion of syphilis. It is mainly Kassowitz
and Bouchut who have taken the stand against Parrot. The former, taking
rachitis for a peculiar inflammatory process, admits that syphilis can
be one of the causes. The latter directs attention mainly to the fact
that by changing food in certain ways rachitis may be produced in dogs,
but that they cannot be made syphilitic. There is no doubt, however,
that syphilis may give rise to rachitis by its general influence on
nutrition, and in this fact lies the key to the connection of great
nutritive disorders with each other. Syphilis will undoubtedly change
nutrition to such an extent as to result in rachitis. Rachitis will
affect the glands; the caseous and suppurative degeneration of the
glands will lead to metastatic processes, to acute tuberculosis, and so
on.

Malaria been claimed as the main cause of rachitis by Z.
Oppenheimer,[12] or, rather, rachitis is presumed by him to be the form
in which malaria makes its appearance in young infants. After disposing
of other alleged causes of rachitis, none of which is proved to give
rise to every case, and referring to the anatomical belief that the
peculiar hyperæmia and inflammation of rachitical bones is created by
the embryonic condition of the growing osseous tissue, he points to the
prodromi, amongst which he emphasizes chronic diarrhoea and the
nocturnal crying. The latter, with its perspiration and subsequent
sleep, he claims as evidence of malaria, and as a substitute for the
intermittent neuralgia of adults, the more so as he believes he finds
the spleen tumefied. The persistent diarrhoea of these infants is said
to be paroxysmal--to take place in the morning, contrary to what is
seen in the usual form of intestinal catarrh; the discharges are said
to be serous, not tinged with bile; the appetite to be good through the
rest of the day; the weight of the body not to be lessened, but anæmia
to develop gradually, and fever to occur occasionally. In other cases
infants have cold hands and feet and blue lips toward evening; the skin
is pale, the spleen enlarged; otherwise there are perhaps no symptoms,
but the infants try to get uncovered, and have an increase of
temperature of from 1° to nearly 3° F., and a perspiring head in the
morning. After a while the rachitical symptoms belonging to the bones
and the general system become apparent. After all of the author's
ingenious and emphatic assertions and deductions, it becomes evident
that malaria--in the severe forms in which it has been found by
Arnstein, Browicz, and Henck to cause bone diseases--may give rise to
rachitis, but it is also clear that he tries to prove too much. The
long series of attempts at proving that every form and case of rachitis
depends upon a single and uniform cause have proved futile. The
physiological hyperæmia of the bones and the rapid growth of all the
infant tissues are shaped into the complex ailment which we call
rachitis by more than a single disease or a single nutritive
disturbance.

[Footnote 12: _D. Arch. f. klin. Med._, xxx., 1881.]

{146} SYMPTOMS.--Before entering upon a more accurate and elaborate
enumeration of the symptoms of rachitis, I mean to dwell upon peculiar
differences which take place according to the age in which the disease
makes its appearance. Very young babies--that is, infants of a month or
two--develop rachitis in such a manner that many cases are overlooked
until it is too late to relieve them in time. This occurrence takes
place when there are no prominent causes, such as diarrhoea or other
nutritive disorders, nor any premonitory symptoms. Such infants appear
to be perfectly well; they have the average weight, and even more; they
have plenty of adipose tissue, and look well. The only anomaly appears
to be an undue degree of paleness. Without pain or flatulency they are
constipated. This constipation is not congenital, as it always is when
the colon is unusually long even for an infant, and when the sigmoid
flexure is of double or even treble length, but makes its first
appearance about the end of the first or the beginning of the second
month. It is relieved only when the increasing muscular power of the
intestine results in more effective peristalsis. The second symptom is
the thoracic groove, to which I shall allude later, and a gradual
thickening of the costo-cartilaginous junctures, with or without
periosteal pain on pressure. About the same time the cranial softening,
craniotabes, with its hyperæmia and perspiration of the entire scalp,
and baldness, and the first symptoms of maxillary rachitis, become
perceptible. During all this time the epiphysial swellings and the
diaphysial curvatures develop but very slowly; but at a very early time
chronic bronchial catarrh, with a loose cough, begins to be
troublesome. When rachitis begins at a late period--say, about the
sixth or eighth month--the aspect of the case is different. The infant
has suffered before either from bronchitis and broncho-pneumonia, or in
most cases from indigestion and intestinal catarrh. There is some
degree of emaciation; the skin does not fit the limbs, as it were--is
loose, thin, flabby, and rather dry. The tendency to diarrhoea
continues to prevail. The epiphyses, particularly of the lower
extremities, are thickened at an early time, curvatures of the tibiæ
become apparent, and all the rest of the bones participate in the
process, with the exception, sometimes, of those of the head.

The head, however, is liable to exhibit symptoms of rachitis at a very
early period of life. It is large, or appears to be so,[13] mostly for
the reason that the face is proportionately small. The forehead is
large, the frontal protuberances very prominent, as are also those of
the parietal bones. Thus, the head is more or less square. Dilated
veins are visible in and through the pale skin; there is but little
hair, on the occiput less than on the rest of the head. Sometimes the
occiput is quite bald, the hair having been rubbed off on the pillow.
The scalp feels warm, except during perspiration. The latter is very
copious, particularly on the occiput--to such an extent, indeed, that
the pillow is drenched--and will remain so for months. The sebaceous
follicles are often still larger and more numerous than they normally
are at that age, and seborrhoea is {147} often, though not always, met
with. The sutures are wide, sometimes one or two centimeters; the
posterior fontanel remains open; the large anterior fontanel is very
large, being sometimes several inches long and wide. The pulse is felt
very distinctly through it. The systolic cerebral murmur, which was
first found by Fisher of Boston in 1833, and considered to be a
positive symptom of rachitis (which certainly it is not, as it is found
in almost every healthy baby with a patent fontanel), is very audible.
The fontanel and sutures remain open for a long period. Instead of
closing, as they do normally at the fourteenth or fifteenth month, the
former ossifies about the end of the second or third year, or later.
Gerhardt reports a case in which it persisted to the ninth year. The
cranial bones appear to be thin, and give way under the pressure of the
finger. Ordinarily, it is true, the cranial bones of every baby, even
if perfectly healthy, are movable under pressure, but they are so only
along the sutures, where they may retain this mobility, in some
instances, a long time. Indeed, it appears that sometimes about the
middle of the first year the occipital bone becomes thinned out in
apparently quite healthy children. Moreover, even in the skulls of
infants who were taken to be in good health small defects in the bones
were found (Friedleben), with no uncomfortable symptoms at all.
Therefore it is rather difficult to draw the exact boundary-line
between the healthy and the morbid condition; thus it is possible that
some of those cases which exhibited apparently morbid local changes
without morbid symptoms may not have been diseased after all. In those,
however, in which rachitis is really developed in the cranium a
peculiar condition is found. In the posterior half or third of the
parietal bones, either the right or the left side being more marked,
there are in the tissue of the bone distinct spots in which the osseous
material is not only thinned out, but has entirely disappeared. In
fact, the bone is perforated, the edges of the holes being rather
steep, sometimes slightly thickened, and the scalp separated from the
brain only by a thin, transparent membrane, the remnant of the
periosteum. These holes can be easily found through the integument. The
finger, though ever so gently pressing down upon it, moves the cranium,
if any be left, before it; the bone feels like paper, and the sensation
as if it could be easily broken through is quite distinct and
embarrassing. Such perforations are usually quite numerous; from five
to twenty or more can often be counted. They are surrounded by normally
hard bone, and thereby can be recognized from the flexible part of the
cranium extending along the sagittal and lambdoid sutures. Where these
results of rachitical softening, craniotabes, are most prominent--that
is, on the part on which the infant is mostly reclining--the bone is
flattened, and may remain so for life, though in the majority of cases
the asymmetry will disappear. The flattening and perforations result
from the same causes--viz. softening of the bones and pressure upon the
bone between the pillow outside and the brain inside. With it go, hand
in hand, thick rachitical deposits under the hyperæmic periosteum of
other portions of the skull. Where craniotabes is largely developed on
the occipital portion, the frontal and the parietal bones (in their
anterior halves) are usually thus thickened. A cross-section with a
knife will reveal a diameter of the new osteoid material between the
periosteum and bone of one-half to one centimeter in thickness. It is
very hyperæmic--even more so than the bone itself, {148} which, when
cut into, exhibits an unusual amount of blood. Sometimes the deposits
are still larger, and are apt to change the appearance and weight of
the skull considerably after recovery has taken place and eburnation
and sclerosis have taken the place of the normal osseous tissue.

[Footnote 13: Boötius (1649), quoted by Haller (_Bibl. Med. pract._,
1779): "Infantibus caput grandescit, reliquum corpus contabescit, ossa
in articulis tument, dextrum hypochondrium tumore æquali prominet; hoc
malum multis millibus infantum molestum est" ("The infant head grows
large, the rest of the body emaciates, the articular bones swell, the
right hypochondrium is raised by a uniform tumor; this malady is a sore
affection in many thousands of infants").]

Such a case of rachitical cranial sclerosis I have described in the
_Amer. Med. Monthly_ of 1861. It was, however, by no means a mate of
the case related by E. Huschke. The latter skull was that of a girl of
seventeen years, and weighed 4117 grammes instead of the normal weight
of 600 grammes. The medullary (Havers') canaliculi were large and very
numerous on the surface, narrow and very few in the interior of the
sclerotic bones, and the osseous canaliculi were more spherical and
irregular in site and shape. The chemical composition was also
abnormal, phosphate of lime being 65.59, carbonate of lime 11.12,
sulphate of magnesia 1.14, cartilage and fat (very little), etc. 22.15
per cent. No fluorate of lime was found. Most of the bones were
exceedingly hard, but fragile when tried in small pieces; very white
inside, yellowish on the surface, the latter color being the remnant of
extravasated blood or other pigmentous matter. Another skull, in
Huschke's possession, and moderately sclerotic, weighed (lower jaw
excluded) 1075 grammes; a third, in the museum of the University of
Jena, is that of a young baboon,[14] in which all the bones covering
the hemispheres had become sclerotic.

[Footnote 14: Baboons suffer from rachitis very extensively. In the
_Transactions of the Pathological Society of London_ (xxxiv., 1883, pp.
310, 312) I. B. Sutton gives the description of two baboons, one of
which was six months, the other one year and six months old, when they
died. The careful description of the specimens exhibited leaves no
doubt as to the rachitical nature of the changes in both the periosteum
and the tissue of all the bones of the body.]

Of undoubted total cranio-sclerosis Huschke reports but ten
cases--those of Malpighi (1697), Cuvier (1822), Ribalt (1828), J.
Forster and Bojanus (1826), Ilg (1822), Kilian (1822), Otto (1822),
Vrolik (1848), Albers (1851), Huschke (1858). The disease does not
affect the auditory bones, the condyles of the maxillary and occipital
bones, nor the styloid process of the temporal bone. It is recognizable
in the posterior part of the cranium and basis cranii, but affects
mostly the bones of the face and the frontal, parietal, and cribroid
bones. Thus, the disease takes its origin in the anterior portion of
the skull, particularly in the superior maxilla, and proceeds upward
and backward, terminating in the basis cranii in the neighborhood of
the infundibulum and appendices. But two of all the cases were observed
during life. In all the disease was traced back to early life. The
chemical composition of the bones was greatly changed in all. Instead
of the normal proportion of earthy to organic material = 2.1 (or
1.5):1, it was from 3.5 to 4.4:1. Particularly the carbonate of lime
was greatly increased.

The brain and its meninges participate, in many respects, in the
changes worked by rachitis, and mainly in the abnormal vascularization
of the bones. They are very much congested, and succulent. A section
through the brain shows a great many large and small blood-points. This
hyperæmia may give rise to over-nutrition, which assumes the character
of real hypertrophy of the brain. When that hyperæmia, however, becomes
excessive, effusion will take place into the cavities, the tissue of
the arachnoid, and the substance of the cerebrum, which latter looks
{149} peculiarly brilliant, elastic, and sometimes white, in
consequence of the blood-vessels being emptied by the pressure on the
part of the enlarged mass of the cerebrum upon the blood-vessels. Thus,
instead of cerebral hyperæmia there may be anæmia. Every form of
hydrocephalus may follow the rachitical process. Afterward, when the
craniotabes has healed, the secondary effusions will generally also
disappear, but not a few cases of hydrocephalus may be traced to
rachitis occurring during the first half year of life. When that
occurs, the intellectual faculties may suffer, while, on the contrary,
complete recovery not infrequently exhibits an unusual degree of mental
development, for the same reason which improves the chances of the
development of the bone. The degrees of physiological and pathological
nutrition and over-nutrition are very variable in their nature and
results.

This condition of the cranial contents is not the only one brought
about by rachitis. The softness of the cranial bones permits a direct
pressure on the brain. The side on which the infant for the most part
reposes gets flattened, and the brain is also compressed. The skull
consequently bulges out in the opposite direction. This anomaly, as
stated above, is sometimes visible through life, though in the large
majority of cases after recovery from rachitis has taken place this
asymmetry will gradually disappear. Before that can occur, however, the
infant is liable to suffer from the rachitical changes. Convulsions are
by no means rare. Vogel has, however, been able to produce an attack of
convulsions by pressing upon the softened spots of the cranium.
Permanent or temporary contractures of the fingers and toes I have seen
in several instances. Gerhardt looks upon rachitis as one of the causes
of tetany.

A frequent symptom of the cerebral changes which take place during, and
in consequence of, craniotabes is the crowing inspiration, or
laryngismus stridulus, of infants. It may be mild or severe. The mild
form is very frequent, and consists in the occurrence of a shrill
inspiratory sound while the baby is either quite placid or excited or
crying. It is frequently overlooked entirely, is usually overcome after
a number of months, and gives rise to serious trouble in but very few
instances. The severe form is of a different nature. While the baby is
awake or asleep, without any premonitory symptoms, while playing or
crying, placid or excited, all at once respiration will cease. This
will take place, usually, after expiration. The limbs are hanging down,
as it were lifeless, the face turns pale, then purple, and slight
convulsive twitching may set in for ten or twenty seconds. There
appears to be a complete paralysis, and death from apnoea seems to be
imminent. All at once, a long, deep crowing inspiration will be heard,
respiration will commence again, and the whole terrible attack is
overcome. It may return a number of times every day, or sometimes not
for several days, during a period of many weeks or several months. The
attacks which set in after inspiration are apt to be more dangerous. In
such an one, but also in the other kind which sets in after the
expiratory movement, death may occur suddenly, or the attack may be
followed by a convulsion which may terminate fatally like any other
eclamptic seizure. In this manner it is that the majority of cases of
rachitis perish which terminate fatally during the active progress of
the morbid process. In this connection, however, it may be well to add
that craniotabes is not the only cause of laryngismus, particularly
when the {150} latter is found in the second year of life, or even
later. But almost every case, without any exception, which is observed
during the first eight or nine months is due to that very cause; and a
good many cases occurring later, when the craniotabic bones have become
normal, arise from the effects, either meningeal or encephalic, of the
rachitic process. Still, complications of craniotabes with a large size
of the thymus gland may occur, and enlargements of the tracheal and
bronchial lymphatic glands are quite frequent, as we shall see
below.[15]

[Footnote 15: Z. Oppenheimer prefers the name rachitic asthma in place
of laryngismus, and suggests an explanation of the symptoms from a
strictly anatomical point of view. If not correct, it is at all events
interesting, as everything this ingenious writer proposes. He points to
the ligament situated between the spinæ intrajugulares of the temporal
and occipital bones, which, as long as it is of normal consistency,
separates the jugular vein from the pneumogastric nerve. As it is
covered with periosteum and dura, it is apt to ossify, and forms an
osseous partition in the foramen jugulare, which participates in all
the changes taking place in the periosteum. As this becomes softened
and succulent, so will the ligament, either on both sides or on either.
Its influence on the neighborhood depends on its size or succulence (as
also on the difference in width of the foramen jugulare or lacerum,
which corresponds with the difference in size of the transverse
sinuses). The irritation of the pneumogastric is perhaps easily
explained thereby, but in very exceptional cases only the accessory
nerve would be affected. As, however, the latter controls the
sterno-cleido mastoid and trapezius, and also the laryngeal muscles,
and is apt to provoke cardiac paralysis during diastole, the occurrence
of sudden death would be best accounted for.]

While the size of the cranium is normal, or sometimes more than normal,
the face undergoes some changes which result in absolute or relative
diminution of size. These depend mostly on a reduction in the volume of
the jaws. Glisson knew of it, and therefore looked for the cause of
rachitis in the process of dentition. Now, both maxillæ are liable to
become rachitical at an early date, as early indeed as the bones of the
cranium. Rachitical deposits and softening take place in them very
generally. The lower maxilla is flat anteriorly, it loses its rounded
outline, is shorter in longitudinal direction, while the rami are thick
and clumsy; the whole bone is shorter than normal, and sometimes
asymmetric. Its changed appearance is greatly due to the effect the
muscles, with their powerful insertions, produce on the softened bone;
mainly the masseter, also the mylohyoid, which draws the lateral
portions inward, and the geniohyoid, which pulls at the central
portion. Of the latter, the lower portion is drawn out, the inner and
the alveolar part inward. Thus, the teeth, mainly the incisors, of the
lower jaw are turned inward to such an extent that, as those of the
upper look outward, the two rows of teeth do not touch but cover each
other. Besides, the periosteal proliferation around the alveoli is
excessive, sometimes so much so as not only to crowd the teeth into
irregular positions, but even to absorb and annihilate alveolar
processes in the course of the morbid changes. The cases in which the
number of teeth are actually diminished by rachitis are not at all
rare. In the superior maxilla the last-described anomaly is also
observed. Periosteal thickening is mainly noticed about the
intermaxillary bone--sometimes to such an extent that above and behind
it a considerable impression takes place. The shape of the upper jaw is
more spherical than normal, and the cheek-bones become very prominent.

The belief that maxillary rachitis is now and then met without any
other symptom of rachitis I do not share. What I said of craniotabes is
also valid in regard to this form.

{151} Irregular teething is a constant companion of maxillary rachitis,
but is also present where the latter is not well, or not at all,
marked. As a rule, the first teeth protrude late, about the ninth or
tenth or twelfth month. That the first year and more should elapse
without any tooth is of frequent occurrence in rachitis. Cases in which
the first teeth do not come before the second year is completed are not
very uncommon; in some there are none even when the child is much
older. In most cases the retardation of dentition goes hand in hand
with very marked retardation in the development of the rest of the
bones and in the closure of the cranial fontanel. But not in every case
of rachitis is there a retardation in the process of teething. In some
a few teeth appear at the regular period (at the completion of the
seventh or eighth month), or even at a very early age (in the fourth or
fifth month); after which there is an interruption in the protrusion of
teeth for an indefinite period. Evidently, the period in which rachitis
is developed exerts its influence on the teething process. When it
exists at a very early age, it will retard teething until recovery
takes place. Still, it is possible that a moderate amount of periosteal
and osteal hyperæmia and over-irritation matures the teeth abnormally.
In all those cases, however, in which rachitis does not occur before
the second half of the first year, the first teeth will appear at the
normal time, and a long period will follow in which no teeth at all
will make their appearance. Then, again, when the whole process comes
to a standstill, and recovery takes place with solidification of the
bones, and even eburnation, the teeth will come in rapid succession.
Whether they will, as is frequent, decay almost as soon as formed, or
whether they will be unusually hard, solid, and yellowish, depends on
the stage of the disease in which they made their appearance, and on
the complications aggravating the case. Of very grave import in this
respect are digestive disorders before and during the course of the
disease.

The vertebral column suffers also. In the normal infant it is straight,
but in the rachitic it exhibits a kyphotic deformity very soon. When
such a baby of three or six months is sitting up, the middle portion of
the back is protruding, as in Pott's disease. In almost every case,
however, this kyphosis is but apparent and the result of muscular
debility. In order to arrive at a diagnosis at once, it is sufficient
to place the patient on his face and support the head, and raise the
lower extremities and pelvis in the air. If the kyphosis is but
functional, the prominence disappears at once. By nothing can the
muscular insufficiency of early rachitis be better demonstrated than by
this little experiment. But actual deformity is also found in rachitis.
It softens both the vertebræ and intervertebral cartilages, and either
their anterior or posterior portion may be irregularly developed, and
be either too high or too low. Besides, the articulating surfaces are
sometimes too convex. Thus the causes of both kyphosis and scoliosis
are amply furnished, and complications of the two are quite frequent,
and the deformities resulting therefrom quite formidable. Scoliosis is
mostly to the left; kyphosis generally complicated with lordosis, and
sometimes the vertebral column exhibits a spiral shape.

The ribs of the convex half are prominent and divergent, those of the
concave side flattened and parallel. The two halves of the chest are
therefore very unequal indeed. Muscular traction, atmospheric pressure,
{152} the elastic traction of the lungs, the presence of pulmonary
complications, and the pressure from below on the part of the enlarged
viscera of the abdominal cavity, come also in for a considerable share
in the completion of the deformity.

The ribs and the sternum aggravate it considerably. Even without any
affection of the vertebral column they suffer seriously from the
general affection. The manubrium is thickened and drawn inward, the
ensiform process protuberant, the sternum often swelled and painful to
the touch. The ribs are sensitive to the touch on one or both sides.
The child cries when taken up or when fearing to be taken up. The
costo-cartilaginous junctures are thickened, mainly so from the fourth
to the eighth ribs. The insertion of the diaphragm becomes soon
perceptible by a deep groove around the chest. The anterior portion of
the ribs is flattened, posteriorly they are inserted at acute angles.
Thus the intrathoracic space becomes narrow, the sternum with the
costal cartilages is pressed forward (pigeon breast, pectus carinatum),
the thorax is deprived of its elliptical shape and becomes triangular,
the dorsal aspect being flattened, and the distance between the
vertebral column and the sternum increased. Below the diaphragmatic
groove the thorax expands, the liver and other abdominal organs
crowding the ribs outward. All sorts of changes are experienced by the
ribs in these conditions. Parts of them are flattened, parts undergo
infraction, parts are even concave; they are bent and twisted, now and
then to such an extent as to turn the concave side out, the convex
surface in. In addition to all this, the scapula is big and clumsy and
protuberant, the clavicle considerably bent and frequently infracted,
and not rarely covered with genuine callus.

That the respiratory and circulatory organs must suffer from such
anomalies, though they be not excessive, is certain. The heart is
crowded by the flattening of the ribs and the contraction of the
thoracic cavity. Its beat is visible over a large surface, and its
percussion dulness is extended over its normal space, though no
enlargement have taken place. This, however, is very apt to occur after
some time by overexertion. The latter is increased by the condition of
the respiratory organs. The ribs being flexible, the chest contracted
and compressed, the diaphragm raised, the respiratory muscles feeble,
respiration is insufficient, even without the presence of any further
complications; thus dyspnoea and a certain amount of cyanosis are
frequently met with in consequence of the anatomical changes only. In
addition to this, there is from the beginning a tendency to catarrhal
and inflammatory conditions. Even without any deformity the rachitical
process is accompanied from an early time with bronchial and tracheal
catarrh. A chronic cough in an infant, with very little or no fever,
disappearing and returning, mostly with copious secretion--which,
however, is swallowed as soon as it reaches the pharynx--rouses the
suspicion of general rachitis. It is often complicated with extensive
dulness over the manubrium sterni, due (to rachitical thickening of
this bone and) mostly to the persistence of a large size of the thymus
gland; and also with enlargement of the bronchial and tracheal glands,
the latter of which are often accessible to recognition by percussion.
They are to be looked upon as a frequent occurrence in rachitis, though
no associated diseases leading to their enlargement have been noticed.
They and the chronic tracheo-bronchial {153} catarrh are closely
dependent upon each other. They are each other's both cause and effect.
Neither of them, however, remain uncomplicated. Catarrh grows into
broncho-pneumonia, with frequent returns. Atelectasis, interstitial
pneumonia, dilatation of bronchi, and pulmonary consumption are often
traceable to such apparently slight catarrhs, which, when not
recognized as depending on their constitutional cause, cannot be
removed. Nor are the cases of miliary tuberculosis, resulting from
caseous degeneration of rachitical glands, very exceptional.

The anatomical changes in the abdominal viscera may be due to the
preparatory diseases or the complications of rachitis; but, at all
events, the abdomen yields a number of changes visible through the
whole duration of rachitis. It is very large; its size is due to the
contraction of the thoracic cavity and the downward pressure of the
chest-wall upon the contents of the abdominal cavity. It is also due to
the changes wrought by rachitis in the pelvis. Softening of bones and
synchondroses, torsion, the weight of the trunk, and the pressure of
the femora from below produce the change of the pelvis so well known
and much feared in the parturient female. The promontory and sacrum are
pushed in, the arcus pubis is large, the pelvis asymmetric; the small
pelvis is contracted, the large pelvis broader. Thus, the small pelvis
has no room for viscera, which, then, are crowded upward. The digestive
disorders which gave rise to, or formed the first stage of, rachitis
result in the accumulation of gas; the scrobiculus cordis is greatly
expanded. The liver[16] is large, congested, and in fatty degeneration.
The latter is the more frequent the more a certain degree of fatty
condition is a normal attribute of every infant liver. When the liver
is found but small in post-mortem examination, it is so because of the
general anæmia and emaciation. Sometimes it is amyloid, as are also the
spleen (mostly hyperplastic only), the kidneys, and the arteries of the
intestines in many instances.

[Footnote 16: Dr. Norman Moore presented a cast and drawing to the
Pathological Society of London (_Trans._, vol. xxxiv., 1883, p. 185)
showing how considerable may be the digressions of the diaphragm and
local pressure upon the liver in a case of rickets. Three large beads
caused as many projections from the under side of the diaphragm, and
corresponded with local thickenings of the capsule of the liver,
probably produced by the continued pressure through the diaphragm of
the beads, which were on the seventh, eighth, and ninth ribs, and the
largest of which was equal in size to a hazel-nut.]

The alimentary tract is the seat of many changes recognizable during
life. The tonsils are often large. The tongue is seldom coated to an
unusual degree. On it are found little islands, red, marginated,
deprived of epithelium. They will increase in size and number and
extend backward. They will heal and reappear. They are by no means
syphilitic, as Parrot would have it, and correspond exactly with the
erosions near the solitary glands and those of Lieberkühn in the
intestinal part, which mean nothing else but a nutritive disorder of
the epithelia, and give rise to nothing worse than incompetency of
absorption in that locality and abnormal secretion. The stomach is in a
condition of chronic catarrh, sometimes dilated. Acid dyspepsia is
frequent. Anorexia and bulimia will alternate. Feces contain an
abnormally large amount of lime. Diarrhoea and constipation will follow
each other in short intervals. The former owes its origin to faulty
ingesta or chronic catarrh; the latter, sometimes to improper food, but
more generally to muscular insufficiency. {154} This condition has not
been estimated at its proper value. Besides myself,[17] nobody but Bohn
has paid the attention to it which it deserves. Here, again, I have to
insist that rachitis is a disease of the whole system, and not
exclusively of the bones. Indeed, the muscular system is amongst the
first to suffer. In the same way in which the voluntary muscles are not
competent to raise and support the head or to allow a baby to sit up
without a functional kyphosis, the involuntary muscles of the intestine
are too feeble for normal peristalsis. The infant of a month or two
months of age may have had normal and sufficiently numerous
evacuations; gradually, however, constipation sets in; the feces become
dry, but are perhaps not much changed otherwise. If no other cause be
apparent, the suspicion of rachitical constipation is justified.
Seldom, however, after it has lasted some time--and only after some
time has elapsed relief will be sought--it will remain alone. Other
symptoms of rachitis will turn up and the case be easily recognized.
This constipation is an early symptom, as early as thoracic grooving or
craniotabes. Very often it precedes both--is, in fact, the very first
symptom--and ought therefore be known and recognized in time.

[Footnote 17: _Jour. Obst., etc._, Aug., 1869.]

The kidneys have been mentioned above. They are often found rather
large. Though the fact has been alluded to before, I will here again
state that it has always been the general impression that the amount of
lime eliminated in the urine of rachitic children is excessive. The
reverse of that is true. Seemann and Lander have proved beyond dispute
that in most stages of rachitis there is less than the normal amount of
lime in the urine. Thus, the theory that lime is eliminated by an
excess of acids in the blood is proven to be incorrect. But it is a
fact that the rachitical bone contains a proportionately small amount
of lime. The conclusion is, then, that its introduction must have been
diminished. On the other hand, every article of food contains a large
amount of lime, which might be introduced into the circulation if
digestion be not at fault. The fact is, that a large amount of lime
introduced is not utilized, and is eliminated with the feces.

In connection with these facts the following will be found very
interesting. It has been found by Bunge that when potassium, with the
exception of chloride of potassium, meets chloride of sodium, the two
will exchange their acids, so as to form chloride of potassium and
phosphate of sodium. They will be found in the blood also, will be
eliminated as such, and result in a comparative absence of chloride of
sodium from the serum of the blood. Now, comparative absence of
chloride of sodium diminishes the possibility of the development of
hydrochloric acid. Thus, it is not a surplus of acid, but a lack of
hydrochloric acid, which results from such chemical combinations. If
such be the case, calcium salts are not absorbed sufficiently. Thus,
they will appear in the feces, and not even be absorbed in the
intestines, because of the alkalinity of the intestinal secretion, by
which the lime cannot be dissolved. The more lime, then, is introduced
under these circumstances, the greater the incumbrance to digestion.

The correct proportion between chlorine, phosphorus, potassium, and
sodium is certainly exhibited in woman's milk. There is lime enough in
even the poorest article of that kind. But indigestion brought on by
{155} woman's milk in a disordered condition or by any other cause will
prevent the absorption of lime when a superabundance of phosphorus and
potassium disturbs the formation of hydrochloric acid. In these cases
not only the development of the bones, but also that of the muscles, is
disturbed. The latter is of great importance in regard to circulation,
because a large part of the circulation depends on the pressure on the
part of the muscular fibres exerted on the small blood-vessels. These
facts have been the reason why I insist upon the addition of chloride
of sodium to the food of infants and children, particularly those who
are fed on cow's milk; for cow's milk and vegetables contain a relative
superabundance of potassium compared with sodium. Even adults will find
cow's milk very much more digestible by adding table-salt to it.

The extremities begin to suffer at a later period than the ribs and
cranium. The opinion of Guérin, that the rachitical process begins in
the lower extremities and ascends gradually, is erroneous. It cannot
even be stated that the lower extremities are affected sooner than the
upper. There is no regularity at all; it is not even necessary that all
the osseous tissue should fall sick. But this can be taken as a fact,
that hands and feet, and particularly the phalanges, are the latest to
undergo the rachitical change. First in the line of morbid alteration
of the bones are the epiphyses, mainly of the tibia, fibula, radius,
and ulna. Their integument appears to be thin; now and then the
cutaneous veins are dilated. The periosteum of the diaphysis becomes
thick, softened, and painful to the touch and pressure, its compact
layer thin, the medullary space large, the whole bone flexible, at the
same time that the ligamentous apparatus of the joint becomes softened
and flabby. At this time babies are greatly admired and applauded for
the facility with which they introduce their feet into their mouths.
For at the same time the bones begin to curve under the influence of
the flexor muscles, which are always stronger, as they do in later
months under the weight of the body when the child begins to walk. The
curvature is not always a mere arching, but sometimes the result of
infraction (green-stick fracture), a complete fracture not being
accomplished because both of the softness of the osseous tissue and the
resistance on the part of the thickened and softened periosteum. Both
the legs and forearm bend on the external side, the resulting concavity
looking inward. The humerus bends in a direction opposite to that of
the forearm; the thigh, usually outward and forward.

The attempts at locomotion are often the causes of quite preposterous
anomalies; creeping, sliding, walking, turn the extremities in such
unexpected directions that talipes valgus, genu valgum, and now and
then double curvatures, are the results. These, however, may not always
be very marked, but there is one change in the rachitical bone which is
constant--viz. the impairment of longitudinal growth. In every case the
diaphyses remain abnormally short, and the proportion of the several
parts of the body are thereby disturbed. Chiari measured parts of the
skeleton of a rachitical woman of twenty-six years who was nine years
old before she could walk. Her height was 116 centimeters, the length
of the lower extremities 42, femur 23, tibia 15, fibula 20, humerus 16,
right radius 12.5, left radius 11, right ulna 15, left ulna 14
centimeters. In a second case the parts of the skeleton were measured
after they had {156} been extended with great care. The right arm from
the acromion to the middle finger (incl.) was 39 centimeters, the left
38; the right lower extremity from the trochanter to large toe (incl.)
39, the left 41.

The skin participates in the general nutritive disorder. It is soft and
flabby. In those infants who become rachitical gradually while proving
their malnutrition by the accumulation of large quantities of fat, it
exhibits a certain degree of consistency. When rachitis develops in the
second half of the first year or later, with the general emaciation the
skin appears very thin, flabby, unelastic. The veins are generally
large. Complications with eczema and impetigo are very frequent; where
they are found the glandular swellings of the neck and below are still
more marked than in uncomplicated cases. Circumscribed alopecia is
sometimes found (not to speak of the extensive baldness of the
occiput). It is not attended with or depending on the microsporon
Audouini, but the result of a tropho-neurosis. In the hair Rindfleisch
found fat-globules between its inferior and central third. Then it
would break, the axial evolution would cease, and the end become
bulbous by the new formation of cells.


Acute Rachitis.

There is a form of rachitis which may be, and has been, called multiple
epiphysitis or multiple periostitis of the articular ends of the long
bones. The changes which in the usual form of rachitis require months
to develop take place in a very short time. Not infrequently the
children were quite well before they were taken with this peculiar
affection. Cases have been known to occur between the fourth and
twenty-fourth months of life, and to last from two to six weeks, or
just as many months. They have been known to get well, or a few of them
terminate fatally. They are accompanied with fever and rapid pulse,
perspiration, now and then with diarrhoea, with eager or reduced
appetites. At the same time the epiphyses swell very rapidly, and are
painful. The same is true of the diaphyses and the flat bones of the
head. Many authors do not recognize this form as an independent
variety. Some call it an acute initial stage of certain cases of
rachitis, as they are not infrequently found in infants which exhibit a
very rapid growth. Some have taken it as an independent disease,
developed on the basis of a constitutional disposition; some look upon
it as a very intense acute form of rachitis; others, as an intense
growth of the osseous tissue only. Others call it an inflammation of
the bone. Some refer it to hereditary syphilis, and a few to the
influence of malaria. That the disease is epiphysitis and periostitis
there is no doubt. I do not hesitate to claim it as rachitis, for
epiphysitis and periostitis of early age not of rachitical basis are
not apt to run such a favorable course as this form frequently does.
The cases complicated with subperiosteal hemorrhages are claimed as
scurvy by Th. Barlow.

The differences of opinion would probably not have been so great if
every author had seen all the cases of the other observers. It will not
do to judge of unobserved cases by the light shed by a single case
under one's own observation. I have seen cases of acute rachitis which
were {157} the initial stages of general rachitis, and have observed
those of local or multiple epiphysitis, mainly after infectious fevers,
which were diagnosticated as such. They are, however, very uncommon.
But even without a preceding infectious fever, such as scarlatina or
more frequently typhoid fever, there are unexplained cases of rachitis
and deformity. Thus, R. Barwell had some before the Pathological
Society of London,[18] which are positive proofs that some forms of
ostitis may occur and result in the most formidable deformities without
being rachitical. A girl of seventeen years was perfectly well formed
up to the age of two and a half or three years. After that time the
deformities began to develop, and did not change after she was
thirteen, at which time the author saw her the first time.

  Her left humerus measured 7-3/4 inches from shoulder to elbow;
    distance 6-1/4
  Her right humerus measured 7-1/2 inches from shoulder to elbow;
    distance 4-1/4
  Her left tibia measured 10 inches from knee to ankle; distance 7-1/4
  Her right tibia measured 9-1/2 inches from knee to ankle;
    distance 4-1/2

Her bones were always very brittle. When she was between nine and
thirteen she broke her arms four times and her lower limbs on several
occasions. A male patient of twenty-two years, who was born healthy and
well formed, continued thus until five years of age, when he was
attacked with a fever, after which his bones became soft and bent.
Osteotomy was performed on him, and the femora were found to be mere
thin shells of bones surrounding cavities containing great quantities
of medulla, which flowed out of the wound as oil; five ounces were
discharged at once. In both cases there appeared to be a hypertrophy of
the medulla at the expense of the bone-substance--a condition which
Barwell proposes to call eccentric atrophy. "While these subjects are
still youthful very little bone-earth is deposited, or at least remains
in the very thin layer of osseous tissue that subsists. The
relationship between infantile ostitis and extreme development of the
intraosseous fat, though well known, is still occult; neither should we
lose sight of the possibility that the softening process of ostitis may
be due to a fatty acid. Now, fatty ostitis usually occurs in epiphyses.
In these cases the shafts were affected."

[Footnote 18: _Trans._, xxxiv., 1883, pp. 203-208.]

PROGNOSIS.--The course and the prognosis of rachitis are, as a rule,
favorable, but they change according to the degree and locality of the
affection and the age of the patient. Generally there is neither fever
nor rapid exhaustion. But the process lasts for months and even years.
In favorable cases, when recovery takes place the teeth will grow
faster, the bones become firmer, the epiphyses will diminish in
relative size, the bowels become regular. But the length of the bones
is, and remains, reduced, and the head remains large as compared with
the length of the body. Not only are the bones of normal firmness, but
the compact substance undergoes a process of hardening called
eburnation by Guérin. The internal organs also become very active,
perhaps because the total amount of blood has to supply only a body
less extended in length. Nor does the brain suffer after complete
recovery has taken place. On the contrary, it appears that the somewhat
more than normal vascular dilatation, which under unfavorable
circumstances leads to effusion, is {158} frequently apt to nourish the
organ of intellect up to a higher standard. In all cases of rachitis,
however, the curvatures of the extremities will not disappear
altogether, while mild ones, it is true, are hardly recognizable in
advanced age. Curvature of the ribs and of the vertebral column,
however, will remain, and interfere with the expansion and the normal
functions of the lungs and heart. In regard to the lungs, it appears
that in many cases they do not find sufficient space to expand. As far
as the heart is concerned, it touches the flattened, no longer
elliptic, chest-wall over a larger surface, and is very apt to give
rise to the suspicion of enlargement in consequence of extended dulness
on percussion. The rachitic pelvis is well known to the obstetrician
for the difficulties it gives rise to during parturition.

Thus, the prognosis would, as a general thing, be sufficiently
favorable if it were not for the number of complications or severe
symptoms. The chronic catarrh of the lungs accompanying rachitis, the
enlargement of the tracheal and bronchial glands and the lymphatic
glands in general, are apt to lead to inflammatory disease of the
lungs, which, after having returned several times, leads to
infiltration of the lungs with caseous deposits, and not infrequently
results in phthisis. The nervous symptoms accompanying craniotabes may
prove very dangerous. Spasm of the larynx and laryngismus stridulus may
prove fatal in a single attack by suffocation, or general convulsion
may set in during an attack of laryngismus or without it, in which the
child may perish. Therefore the prognosis in every case of laryngismus
and in every case of craniotabes has to be very guarded. It is my rule
to wait from six to eight weeks before giving expression to a decided
prognosis, because during that time medicinal and dietetic treatment
will probably have resulted in such an improvement of the symptoms and
condition as to render the prognosis more favorable. Under no
circumstances, however, ought we to lose sight of the fact that, though
rachitis may disappear, the causes leading to it may still linger on.
Defective nutrition, diseases of the lungs, and intestinal affections
which gave rise to or accompanied rachitis will complicate the
prognosis, though rachitis itself, as far as the bones were concerned,
be no longer in existence.

TREATMENT.--To meet the cause of a disease by preventive measures is
the main object and duty of the physician. He thus either obviates a
malady or relieves and shortens it. Now, if the original disposition to
rachitis, as has been suggested, is to be looked for in early
intra-uterine life, when the blood-vessels begin to form and to
develop, we know of no treatment directed to the pregnant woman or
uterus which promises any favorable result. But the more we recognize
an anatomical cause of the chronic disorder, the more we can appreciate
the influence upon the child of previous rachitis in the mother, and
are justified in emphasizing the necessity on the part of the woman to
be healthy when she gets married, and to remain so while she is
pregnant. After the child is born the most frequent cause of rachitis
is found within the diet or the digestion of the patient. To attend to
the former is in almost every instance equal to preventing disorders of
the latter; for most of the digestive disturbances during infancy and
childhood are the direct consequences of errors in diet. It is,
however, impossible to write an essay on infant diet in connection with
our subject. I have elaborated the subject in my {159} _Infant Diet_
(2d ed. 1876), in the first volume of Buck's _Hygiene_, and of C.
Gerhardt's _Handbuch d. Kinderk._ (2d ed. 1882). Still, the importance
of the subject requires that some points should be given, be they ever
so aphoristic.

The best food for an infant, under ordinary circumstances, is the milk
of its mother. The best substitute for the mother is a wet-nurse.
Woman's milk ought not to be dispensed with when there is the slightest
opportunity to obtain it, particularly when the family history is not
good and nutritive disorders are known to exist, or to have existed, in
any of its members. When it cannot be had, artificial food must take
its place, and it is in the selection of it where most mistakes are
constantly made. This much is certain, that without animal's milk no
infant can or ought to be brought up; as ass's milk can be had only
exceptionally, and dog's milk, which has been said to cure rachitis, is
still less available, the milk of either goat or cow must be utilized.
The former ought not to be selected if the latter is within reach,
mainly for the reason that it contains, besides other objectionable
features which it possesses in common with cow's milk, an enormous
percentage of fat. Cow's milk differs in this from woman's milk, that
it contains more fat, more casein, more potassium, and less sugar than
the latter, and that its very casein is not only different in quantity,
but also in chemical properties. Even the reaction of the two milks is
not the same, woman's milk being always alkaline, cow's milk often
either neutral or amphoteric, and liable to acidulate within a short
time. Thus, the dilution of cow's milk with water alone yields no
equivalent at all of woman's milk, though the dilution be large enough
to reduce the amount of casein in the mixture to the requisite
percentage of one, and one only, in a hundred. The addition of sugar
(loaf-sugar) and of table-salt, and sometimes alkali (bicarbonate of
sodium or lime-water, according to special circumstances), is the least
that can be insisted upon. Besides, the cow's milk must be boiled to
prevent its turning sour too rapidly, and this process may be repeated
to advantage several times in the course of the day. Instead of water,
some glutinous substance must be used for the purpose of diluting cow's
milk. As its casein coagulates in hard, bulky curds, while woman's milk
coagulates in small and soft flakes, some substance ought to be
selected which keeps its casein in suspension and prevents it from
curdling in firm and large masses. Such substances are gum-arabic,
gelatin, and the farinacea. Of the latter, all such must be avoided
which contain a large percentage of amylum. The younger the baby, the
less is it in a fit condition to digest starch; thus arrowroot, rice,
and potatoes ought to be shunned. The very best of all farinacea to be
used in diluting cow's milk are barley and oatmeal. A thin decoction of
either contains a great deal of both nutritious and glutinous elements,
the former to be employed under ordinary circumstances, the latter to
take its place where there is, on the part of the baby, an unusual
tendency to constipation. The decoction may be made of from one to
three teaspoonfuls of either in a pint of water; boil with a little
salt, and stir, from twelve to twenty minutes, and strain through a
coarse cloth. It ought to be thin and transparent. Then mix with cow's
milk in different proportions according to the age of the baby. Four
parts of the decoction, quite thin, and one of milk (always with
loaf-sugar), for a newly-born, equal parts for an infant of six months,
{160} and gradual changes between these two periods, will be found
satisfactory. Whenever there is a prevalence of curd in the passage the
percentage in the food of cow's milk must be reduced, and now and then
such medicinal correctives resorted to as will improve a disturbed
digestion. Care ought to be taken lest for the newly-born or quite
young the preparations of barley offered for sale contain too much
starch. The whiter they are, the more unfit for the use of the very
young, for the centre of the grain contains the white and soft amylum
in preference to the nitrogenous substances which are found near the
husk. Thus, it is safest to grind, on one's own coffee-grinder, the
whole barley, but little deprived of its husk, and thus secure the most
nutritious part of the grain, which is thrown out by the manufacturer
of the ornamental and tidy packages offered for sale. But very few
cases will ever occur in which the mixtures I recommend will not be
tolerated. In a few of them, in very young infants, the composition
recommended by Meigs[19] has proved successful. It consists of three
parts of a solution of milk-sugar (drachm xvij-3/4 in pint j of water),
two parts of cream, two of lime-water, and one part of milk. For each
feeding he recommends three tablespoonfuls of the sugar solution, two
of lime-water, two of cream, and one of milk: mix and warm. The baby
may take all of it, or one-half, or three-fourths.

[Footnote 19: In _Med. News_, Nov. 28, 1882.]

The recommendations given above are based on a long experience, and the
simplicity, cheapness, and facility of preparation of the articles. The
substitutes offered for sale under the title of infant foods are in
part worthless, all of them expensive when compared with the simple
articles recommended by me, and not recognizable as to their uniformity
and compounds. But no matter how appropriate my mixture may be, it is
always for the young infant to be considered as a makeshift. It is to
be used as a representative of mother's milk only when this cannot be
had. Therefore it is better to alternate with breast-milk when this is
secreted in but an insufficient quantity. Some good breast-milk is
better than none at all; but with this proviso, that it _is_ good.
There are some milks either too watery or too dense and white. The
former will produce diarrhoea, the latter hard and dense curd. The
former may be improved by feeding and strengthening an anæmic and
overworked mother; the latter, by giving the baby, before each nursing,
a tablespoonful of a mixture of barley-water and lime-water, or, when
it produces constipation, lime-water and thoroughly sweetened
oatmeal-water. The cases in which breast-milk, such as can be had, is
not digested by the infant are rare, but they will occur. In them the
proper substitute will yield a better result than mother's milk; for
mother's milk will not always be a boon, and must then be dispensed
with. Particularly is this so when it is too old. Weaning ought to take
place when the first group or the first two groups of teeth have made
their appearance. After that time mother's milk is no longer the proper
food, and instead of preventing indigestion and sickness it is a
frequent cause of them and of rachitis. Instead of muscle, it will then
give fat, and the large fontanels and big head, the paleness of the
rotund cheeks, the flabbiness of the soft abdomen and thighs, will tell
the story of rachitical disease slowly engendered by the persistent
employment of an improper article of food. I cannot insist too often on
this, that rachitis may develop with increasing weight, {161} and that
the use of the scales alone is no means of ascertaining the healthy
condition of a baby. As much harm, therefore, can be done by weaning
too late as by so doing too early or too abruptly.

At that early age we treat of here, digestive disorders are more
frequently the results of improper diet than of a primary gastric
disturbance. But when the latter is once established it furnishes its
own indications. A frequent occurrence, together with a general gastric
catarrh, is the presence of fat acids in the stomach, such as an
improper amount of lactic, acetic, butyric, etc. acids. Before
digestion can be anything like normal they must be neutralized. For
that purpose calcined magnesia, carbonate and bicarbonate of sodium,
prepared chalk, and lime-water have been found useful. The latter, as
it contains but a trifle of lime, in order to neutralize must be given
in larger doses than is usually done; a tablespoonful contains but a
quarter of a grain of lime. And all of the alkalies must not be given
in the food only, but also between meals. For when given in the former
way alone it neutralizes the abnormal and injurious acids, together
with the normal digestive secretion, the lactic and muriatic. Not
infrequently, when the infants have suffered for some time, general
anæmia will set in, and result in diminishing the normal secretions of
the mucous membranes (and glands). In those cases which do not produce
their own gastric juice in sufficient quantity or quality pepsin and
muriatic acid may be given to advantage. In these cases the plan
suggested by me is particularly favorable--viz. to add a fair amount of
chloride of sodium (one-half to one drachm daily) to the infant's food.
Also that of I. Rudisch referred to by me previously,[20] who mixes one
part of dilute muriatic acid with two hundred and fifty of water and
five hundred of milk, and then boils (one-half teaspoonful of dil. mur.
acid, one pint of water, one quart of milk). Again, there are the cases
in which wine and the bitter tinctures, which are known to increase the
secretion of gastric juice, render valuable service. The addition of
bismuth to any of the proposed plans is quite welcome. As a
disinfectant and a mild cover on sore and eroded mucous membranes it
has an equally good effect.

[Footnote 20: _Am. Jour. Obstet._, July, 1879.]

Under the head of roborants we subsume such substances, either dietetic
or remedial, which are known or believed to add to the ingredients of
the organism in a form not requiring a great deal of change. Rachitical
infants require them at an early period. Meat-soups, mainly of beef,
and of mutton in complications with diarrhoea, ought to be given at
once when the diagnosis of rachitis becomes clear or probable. Any mode
of preparation will prove beneficial; the best way, however, is to
utilize the method used by Liebig in making what he called beef-tea. A
quarter of a pound of beef or more, tender and lean, cut up finely, is
mixed with a cup or a tumbler of water and from five to seven drops of
dilute muriatic acid. Allow it to stand two hours and macerate, while
stirring up now and then. This beef-tea can be much improved upon by
boiling it a few minutes. It may be given by itself or mixed with
sweetened and salted barley-water or the usual mess of barley-water and
milk which the infant has been taking before. Older infants,
particularly those suffering from diarrhoea, take a teaspoonful of raw
beef, cut very fine, several times a day. It ought not to be forgotten,
however, {162} that the danger of developing tænia medio-canellata from
eating raw beef is rather great. Peptonized beef preparations are
valuable in urgent cases.

Iron must not be given during any attack of catarrhal or inflammatory
fever. The carbonate (cum saccharo) combines very well with bismuth; a
grain three times a day, or less, will answer well. The citrate of iron
and quinine (a few grains daily) can be given a long time in
succession. The syrup of the iodide of iron (three times a day as many
drops as the baby has months up to eight or ten), in sweetened water or
in sherry or malaga, or in cod-liver oil, acts very favorably when the
case is, as so frequently, complicated with glandular swelling.

Cod-liver oil, one-half to one teaspoonful or more, three times a day,
is a trusted roborant in rachitis, and will remain so. Animal oils are
so much more homogeneous to the animal mucous membrane than vegetable
oil that they have but little of the purgative effect observed when the
latter are given. The former are readily absorbed, and thus permit the
nitrogenous ingesta to remain in store for the formation of new tissue,
but still affect the intestinal canal sufficiently to counteract
constipation. As the latter is an early symptom in a peculiarly
dangerous form of rachitis, cod-liver oil ought to be given in time (in
craniotabes). Diarrhoea is but seldom produced by it; if so, the
addition of a grain or two of bismuth or a few doses of phosphate of
lime (one to four grains each) daily, may suffice to render the
movements more normal. There are but few cases which will not tolerate
cod-liver oil at all. The pure cod-liver oil--no mixtures, no
emulsions--ought to be given; the large quantities of lime added to it
in the nostrums of the wholesale apothecaries embarrass digestion and
bring on distressing cases of constipation. These mixtures have been
prepared and are eulogized on the plea of their furnishing to the bones
the wanting phosphate of lime. The bones, however, as we have seen
before, are not grateful enough to accept the service offered. But only
a certain amount of phosphate of lime is useful in rachitis and in
digestive disturbances. In small doses it neutralizes acids like other
alkalies; its phosphoric acid combines with sodium very easily, and
gives rise to the formation of glyco-phosphoric acid, which is of very
great importance in the digestive qualities of the upper portion of the
small intestines.

Plain malt extracts will be well tolerated by some older children. The
preparations which are mixed with a goodly part of the pharmacopoeia by
generous manufacturers are to be condemned.

Craniotabes requires some special care in regard to the head. The
pillow ought to be soft, but not hot; no feather pillow is permitted.
The copious perspiration of the scalp requires that it should be kept
cool, the perspiration wiped off frequently to avoid its condensing
into water, and the flattening side of the head may be imbedded in a
pillow with a corresponding depression. Copious perspiration indicates
the frequent washing with vinegar and water (1:5-6). The muscular
debility commands great caution. The baby must not be carried on the
arm, but on a pillow which supports both back and head, or in a little
carriage. No sitting must be allowed until the back will no longer bend
to an unusual degree. No walking must be encouraged at any time. The
patients will walk when their time has come. The bones are so fragile
that great care {163} is needed sometimes not to fracture or to infract
them and to avoid periosteal pain in lifting. The skin must undergo
some training by gradually accustoming the little patient to cool
water. It can be readily, but gradually, reduced to 70° for a bath at
any season. The addition of rock- or table-salt to the bath is a
welcome stimulant. And fresh air ought to be granted freely.

Laryngismus stridulus shares the indications for treatment furnished by
craniotabes. The general treatment remains the same. Prominent symptoms
and complications ought to be treated besides; constipation requires
the more attention the more convulsive attacks of any description may
arise from reflex action. The general nervous irritability may be
relieved by bromide of potassium, sodium, or ammonium. One gramme daily
(15 grains) of either, in three doses, is well tolerated for a long
period. When there are symptoms of an imminent convulsion, or to soothe
the convulsibility which may break out any moment, chloral hydrate,
eight or ten grains in from one hour to four hours, two grains in a
dose, will be convenient. If the stomach refuses or is to be spared,
from four to eight grains may be given in an enema of warm water. A
severe attack of convulsions ought to be checked with inhalations of
chloroform. When a warm bath is to be had, care should be taken that
the child be not tossed about. Hold the baby in a small sheet or a
large napkin, and immerge it thus into the water, raising the head and
cooling it with cold cloths or an ice-bag. Genuine attacks of
laryngismus with well-developed stages--the first paralytic, the second
spasmodic--give but little time for any treatment. The proposition to
apply the electrical current is well meant, but the attack has passed
by, or terminated fatally, or resulted in a general convulsion, before
the apparatus can possibly be in operation. I can imagine, however,
that a Leyden flask kept ready might be used to advantage during the
stage of apnoea for the purpose of bringing on inspiration. Sprinkling
with cold water, beating with a wet towel, shaking by the shoulders,
may certainly contribute to awake respiratory movements. The advice to
wait quietly until the attack has passed by is more easily given than
carried out. Marshall Hall's direction to perform tracheotomy will, I
hope, soon be forgotten.

Nothing is more gratefully appreciated by the little patients than air.
May it never be forgotten that night-air is better than foul air, and
that furnace-air means air greatly modified by injurious additions.
More than twenty years ago I was in occasional attendance upon a male
baby--now a medical man of some promise--with craniotabes and a number
of general convulsions. No treatment would remove, or even relieve, the
attacks, until, without the physician's advice, the father took the
baby into the street in the hardest winter weather. After the first
long absence from his furnace the baby was well of his convulsions, and
the physicians profited by their involuntary experience.

In the same way that salt-bathing is beneficial, so is sea-air. A
summer at the seaside is a great blessing to rachitical children.
Sea-baths have been arranged for them in France (Berx-sur-mer), in
Italy (San Ilario di Nervi, Viarreggio, Livorno, Volti, Fano), in
England (Margate), in Germany (German Sea, by Prof. Beneke), and for
some little time past in the neighborhood of our own large cities.

{164} Complications command great attention in rachitis, particularly
where there is danger from the affection of the nerve-centres, for the
slightest irritation in some distant part of the body may give rise to
an outbreak. Thus, in craniotabes it is desirable to watch even the
gums. Not sharing the etiological superstition which attributes so many
diseases of infancy to dentition, I still know that a slight irritation
of the gums may suffice to exhaust the slim resisting power of the
infant. If there be local swelling and congestion of the gums over a
growing tooth, it may become necessary, or at least advisable, to
lance. An otitis which under ordinary circumstances would give rise to
no symptoms at all besides some inconvenience or slight pain will prove
the source of great danger in a rachitical (craniotabic) infant. The
chronic bronchial catarrh and frequent broncho-pneumonia of such
patients require early attention, for they and the neighboring
lymphatic glands stand too much in the relation of a vicious circle of
cause and effect.

Rachitical constipation, depending on incompetency of the intestinal
muscle, must not be treated with purgative medicines. Now and then,
when a great deal of abnormal acid is formed in the stomach, calcined
magnesia, a grain or two given before each meal, will control that
disorder and at the same time keep the bowels open. But, as a rule,
every purgative after it has taken effect will leave the intestinal
muscular layer less fitted to perform its functions than before. Its
place may be taken by a daily enema of tepid water. Further indications
are--such a change in the food as will contribute to keep the bowels
moist and slippery, but principally such a modification of food and
such medical treatment as are known to prove beneficial when all the
symptoms of rachitis are fully developed. When the cause of the
infant's rachitis can be traced back to the mother or to an
insufficient quality of her milk, she must give way to a wet-nurse, or
the nurse must be changed for similar reasons. When neither mother nor
wet-nurse prove competent, or either be dangerous, artificial food will
take their place to advantage in the manner I have stated above.
Beef-soup or beef-peptone is to be added to the baby's food daily. Of
the two best farinacea, barley- and oat-meal, the latter is preferable
as an addition to cow's milk, because of its greatly laxative effect.
The percentage of cow's milk in the food ought to be more carefully
watched than in other conditions. Pure cow's milk or cow's milk mixed
with water only is borne worse in no other condition. Half a drachm or
more of table-salt and a few drachms of sugar ought to be added to the
daily mess. The general indications require the administration of iron,
which has no constipating effect in this ailment. Particularly is that
the case with the iodide of iron. Cod-liver oil, in three
half-teaspoonful or teaspoonful doses daily, acts very satisfactorily
both for its general rachitical and for its local effect on the mucous
membranes. Now and then massage, repeated many times a day a few
minutes each time, practised with the palm of the hand only, or gentle
friction, with the dry or oiled hand, of the abdominal surface, will
prove effective in bringing about peristalsis and strengthening the
intestinal muscle. An obstinate case may also require two daily doses
of one one-hundred-and-fiftieth or one one-hundred-and-twentieth of a
grain of strychnia for the same purpose, or such other improvements on
the above detailed plan as the judgment of the attending physician may
direct. At all events, the diagnosis of {165} any case, and the
appreciation of the cause of any ailment, are, to a well-balanced and
educated mind, of infinitely greater value than any number of specified
rules and prescriptions.[21]

[Footnote 21: _Jour. Obstetr._, Aug., 1869.]

It is not impossible that phosphorus, in substance, not in any of its
compounds, may prove of great utility in the treatment of rachitis.
Minimal doses of phosphorus render the newly-formed tissue at the
points of apposition of the bones more compact in a very brief time.
The new formation of blood-vessels in the osteogenous tissue gets
retarded by it. Larger doses of phosphorus, however, increase
vascularization, and osseous tissue is either less rapidly formed or
even softened. When the doses are still larger, vascularization and
softening may rise to such a point as to separate the epiphysis from
the diaphysis. Thus the administration of the drug results in an
irritation which, according to the doses employed, may give rise either
to normal condensation or to inflammatory disintegration. This
experience, arrived at by Wegner in a great many experiments made on
animals, Kassowitz has confirmed. For its therapeutic effect he tried
phosphorus in 560 cases of rachitis. Employing doses of one-half
milligramme (one one-hundred-and-twentieth of a grain) several times
daily (less will suffice), he soon found the skull to become harder,
the fontanel smaller, the softening of the bones of the thorax and
extremities to disappear, and all the other symptoms of rachitis to
improve. This result was obtained though no particular change in the
feeding of the patients was resorted to. To what extent this experience
will be verified by others we shall soon learn. My own is already
sufficiently extensive to base upon it a strong recommendation of the
plan of treatment I have detailed. My therapeutical results in other
diseases of the bones also encourage me to believe that phosphorus will
accomplish much in the treatment of rachitis. Ever since Wegner's
publications--viz. these thirteen or fourteen years--I have utilized
phosphorus in cases of chronic and subacute inflammations of the bones,
mainly of the vertebral column and the ankle-joint and tarsus. After
having taught the method for many years in my clinic and otherwise, I
made a brief communication on the subject to the Medical Society of the
State of New York.[22] Since that time, again, I have followed the same
plan in many cases of the same description, and feel sure that the
prognosis in this serious class of bone diseases has become more
favorable and recovery speedier. Infants of a year or more were given a
dose of one-eightieth or one one-hundredth of a grain of phosphorus
daily. One grain, dissolved in an ounce of oil or cod-liver oil, is a
convenient mixture, four or six drops of which may be administered
daily in two or three doses.

[Footnote 22: _Trans._, 1880.]

From what I have seen of phosphorus in bone disease, and what is thus
far known by experience in rachitis, it appears to me that it will be
of decided advantage in that form of acute rachitis which is apt to
destroy rapidly with the symptoms of acute epiphysitis, rapid pulse,
diarrhoea, rapid diminution of strength, and scorbutic gum. In the few
cases I have seen these last years it appeared to me to act
satisfactorily, together with immobilization of the whole body.

Rachitical curvatures are very apt to become less marked while growth
is increasing and the limbs extending. But many of them are so marked
{166} that they remain for life. Many of these might have been
benefited by timely orthopædic interference. That the application of
Sayre's jacket is indicated in every form and stage of spondylitis,
though it be not equally valuable in all forms, goes without saying;
and that infractions ought to be straightened and supported by splints
when observed and when practicable, is self-evident. But, as a rule,
while the chronic rachitical process is developing in the long bones
the use of mechanical apparatuses is of doubtful merit; they ought not
to be resorted to before the healing has at least commenced. Nor is it
advisable to postpone mechanical interference so long that eburnation
of the bones has time to take place. Surgical operations for the
purpose of removing the curvature are of different nature according to
the different types to be treated. Mere straightening of the curvatures
is indicated, and successful with children under two years.
Osteoklasy--that is, fracturing of the curvature while the periosteum
is left intact--is successful in children of three (or four, according
to Volkmann) years. The fracture does not injure the periosteum, and is
always transverse. In later years osteotomy has proved successful to an
almost unexpected degree, and is one of the happiest achievements of
modern surgery.

Partly as a preventive, partly as a curative measure, Gramba of Turin
and Pini of Milan point to well-directed gymnastics as a requisite in
the treatment of rachitis. For older children they have established
schools in which systematic exercises are brought to bear on chronic
deformities.



{167}

SCURVY.

BY PHILIP S. WALES, M.D.


SYNONYMS.--_French_, Scorbut; _Spanish_, Escorbuto; _Italian_,
Scorbuto, are the various terms in the Romance languages used to
designate this disease, derived from the Middle-Latin word scorbutus,
which is evidently an offspring of one of the early Gotho-Teutonic
dialects, perhaps of the Low German word Schärbunk, Danish Scorbuck, or
the Old Dutch Scheurbuyck, from _scheren_, to separate or tear, and
_bunk_, the belly. These terms originally denoted rupture of the belly,
and afterward scurvy, or scorvy, as it is found in the English dialect.
It has also been traced to the Sclavonic word scorb, disease. The first
is now believed to be the true etymology.

DEFINITION.--Scurvy is an acquired condition of the body whose
essential feature is a perversion of nutrition, which gradually arises
from prolonged employment of food deficient in succulent or fresh
vegetable matter, and progresses uniformly to a fatal issue, in a
longer or shorter time, if the dietetic errors remain uncorrected. This
condition becomes manifest by a change in the complexion to a dull
yellowish or earthy tint, lassitude, marked decrease in the muscular
power, depression of spirits and mental hebetude, breathlessness on the
slightest exertion, minute flecks at the roots of the hairs, especially
those of the legs; and, later, hemorrhagic effusion into the skin,
forming blotches and spots of varying sizes and aspects, which may
finally slough and lead to obstinate ulceration; sponginess of the
gums, which bleed easily and break down into a detritus that impresses
a malodorous taint upon the breath; ecchymotic staining of mucous and
serous surfaces, and, in advanced stages of the disease, effusions of
bloody serum or of blood into the cavities and tissues of the body.

HISTORY.--Obscure passages in certain of the ancient medical classics
(Hippocrates, Celsus) and historical works (Pliny, Strabo) have been
considered as descriptive of scurvy, but the earliest trustworthy
accounts are to be found in the writings of the thirteenth century.
Jacob de Vitry describes an epidemic which occurred among the troops of
Count Saarbrücken besieging Damietta in 1218, and Sire de Joinville
another epidemic among the troops of Louis IX. lying before the same
town in 1249. On both occasions the sufferings of the men were
inexpressible and the mortality fearful. The disease was directly
traceable to defective supplies of fresh vegetable food, aided by
exposure to wet and cold weather, fatigue, and mental depression.

{168} The almost total neglect of horticulture in Europe during the
Middle Ages, especially in its more sterile northern portions, the
habitual diet of salted, smoked, and dried flesh and fish, and the
prolonged spells of cold and damp weather of this region, were
conditions most favorable to the development of scurvy, and these
regions were the very first in which its devastating effects were early
observed and recorded. In the first half of the fifteenth century it
prevailed epidemically in the north of Europe and almost everywhere
endemically, more especially in the countries bordering on the Baltic
and North Seas, although the largest and richest cities were frequently
afflicted in the severest manner in consequence of imperfect
food-supplies and the wretched sanitary conditions under which the
inhabitants lived (Fabricius). The long voyages and imperfect diet of
crews of ships furnished a large quota of harrowing nautical
experiences with the scurvy, commencing with Vasco da Gama's voyage to
India in 1497, and running up to 1812. In this interval it was all but
universal on long voyages, both on single ships and in fleets, in the
mercantile marine and in the navy.

In 1798, through the better insight into the causes of the disease, and
especially through the exertions of Dr. James Lind in ameliorating the
dietary of British sailors, it was practically stamped out of the navy
or restricted to isolated occurrences. The influence of the success
thus achieved was not lost upon the navies of other nations nor upon
the growing fleets engaged in commerce, as the disease has become less
and less frequent, constituting at present but a very trifling
proportion of the diseases incident to seafaring people. This
remarkable result is in part attributable to the fact that the chief
maritime nations have enacted beneficent laws intended to compel the
owners and masters of merchant vessels to observe certain sanitary and
hygienic measures that protect the crews from scurvy. The number of
cases returned in the English navy for 1881, in an aggregate of 52,487
cases of all diseases, was 4; in the Prussian navy, 3 in 8659; in the
Austrian navy, 27 in 8096; in the U.S. navy, none in 13,387. Thus, in a
grand total of all diseases in the chief naval services of the world of
82,629 there were only 34 cases of scurvy--a ratio of .41 per 1000. In
the mercantile marine 62 cases occurred in 32,613 cases of all
diseases, of which 43 were on the Pacific coast: this gives a ratio of
1.9 per 1000. Altogether, the 115,242 cases produced only 96 of
scurvy--a ratio of .83 per 1000. The difference in favor of the naval
over the marine service is accounted for by the greater attention paid
to the health and comfort of the men in the former.

The U.S. steamer Jeannette spent two winters in the Arctic region, and
had a single case of scurvy. The U.S. steamer Rodgers was wrecked, and
the crew, during its sojourn of six months among the Siberian tribes,
suffered severely.

The operations of armies in recent times have not furnished the
frightful mortality which, from neglect of sanitary precautions,
formerly afflicted them. During the rebellion of 1861-64, out of
807,000 cases there were but 47,000 of scurvy, or 5.8 per cent., with a
death-rate of 16 per cent. The French army[1] of 103,770 men during the
Crimean struggle had 27,000 cases of scurvy, or 26.0 per cent., with a
death-rate of 1.5 per {169} cent. In the Bulgarian campaign of 1877-78,
in an army of 300,000 strong, there were, according to Pirigoff,[2]
87,989 cases of disease, of which 4234, or 4.8 per cent., were
frankly-expressed cases of scurvy. This gave a proportion of only 1.4
per cent. of the entire force--a result entirely due to the
maintenance, both before and during the war, of a high standard of
health.

[Footnote 1: Scrive, _Rélation Médico-Chirurgicale de la Campagne de
l'Orient_, Paris, 1857.]

[Footnote 2: _Krieg Sanitäts-Wesen_, Leipzig, 1882.]

ETIOLOGY.--Perhaps no disease has furnished a more fertile field for
etiological conjectures than scurvy. The father of medicine ranked the
disease in one place among those presenting enlarged spleens, and in
another with the twisted bowels. He recognized a putrescence of the
humors as the underlying factor--a theory that held sway until the
beginning of the nineteenth century. The disease attracted wide
attention in the seventeenth and eighteenth centuries from its frequent
epidemic and endemic occurrence in various parts of the north of
Europe, and was believed to be restricted to cold and particularly wet
districts--a view that has been long since abandoned with a better
knowledge of its habitats. It has been encountered alike in high
latitudes north and south, amidst sterile wastes covered with eternal
snows and ice, in the temperate zones and in the burning plains of the
equatorial regions of America and Africa.

Sex has no predisposing influence, and the fact that more males than
females are affected during an epidemic simply indicates that the
former are more exposed to the ordinary determining causes. During the
siege of Paris, according to the tables of Lasègue and Legroux, there
was a very large excess of male cases, and Hayem's figures show only 6
women in 26 cases.

Scurvy has been observed at all ages from infancy to advanced periods
of life; it is believed by certain writers that adolescence is less
predisposed than adult age.

The epidemic feature of the disease led many to the opinion that it was
contagious--a view that retained its hold for many years. It was also
considered to be of a miasmatic character, which, with the previous
feature, seemed to assimilate it in nature with typhus fever and other
diseases of the miasmatic contagious group. This view had a vigorous
advocate in Villemin, who in 1874 read a lengthy paper before the Royal
Academy of Medicine in its support. His arguments were specious,
inconclusive, and inaccurate, the weight both of facts and authority
being decisively against his view. Its occurrence among members of the
same family led a few to regard it as hereditary, and it was thought to
be transmissible from the mother to the recently-born as well as to
nursing infants. The depressing influence of certain emotions, fear,
anxiety, and nostalgia, upon the functions of nutrition has, as might
have been anticipated, been noted as contributing indirectly to the
manifestation of epidemics of scurvy in the presence of the essential
determining dietetic causes.

Scurvy cannot be regarded, as Lhéridon-Cremorne[3] has argued, as the
last term of nostalgia, the other alleged causes being secondary; nor
as the immediate result of mental depression, as Gueit[4] believed from
his experience in the ship Henry IV. during his service on the blockade
in the Black {170} Sea in 1858, because the disease first invaded those
laboring under nostalgia. The currency of such opinions may be readily
explained by the fact that ordinarily depressing mental influences
occur under the same conditions as those associated with scurvy--viz.
during sieges, after defeat, in prisons, and in workhouses; and,
further, the mental phenomena ordinarily occur as prodromes of the
disease long before the pathognomonic phenomena present themselves. Out
of these facts grew the mistake of regarding the mental change as
causative instead of consecutive. Murray went farther and regarded
mental despondency as at once cause and effect, and long ago scurvy was
compared to hypochondriacal diseases.[5] It may be concluded from the
recorded epidemics that no degree of mental exhilaration could ward off
the disease in presence of the determining causes, nor any degree of
mental despondency induce it with proper alimentation.

[Footnote 3: _Thèse de Paris_.]

[Footnote 4: _Thèse de Montpellier_, 1858.]

[Footnote 5: Dolée, 1684.]

The various qualities or changes in the atmosphere were regarded
individually or collectively at various times as the determining
causes. It was supposed that the air might become impregnated with
putrid exhalations from various sources, as the holds of ships, or
rendered impure by the vapors of the sea. The foul air of crowded
habitations, vessels, or cities was appealed to, or the common cause
was sought either in its temperature or humidity, or in both. The
earlier observers gave prominence to cold as a determining cause of
scurvy, and especially when combined with dampness, and hence its
frequency in the north of Holland, Brabant, Belgium, Russia, and
Germany. This was the current view in the seventeenth century. On the
other hand, with equal confidence the disease has been supposed to be
determined by excessively high temperatures, and its occurrence in
India, South Africa, and the equatorial regions has been alleged in
support.

Personal habits have been in the eyes of earlier observers an
all-sufficient cause, and thus excessive exertion attended with fatigue
and exhaustion has been considered the cause of several severe
outbreaks on shore and at sea. In contrast with this opinion we find
the English physicians placing great stress upon indolent habits and
lack of exercise as a predisposing if not a powerfully determining
cause.

The use of tobacco was inveighed against by Maynwaring and Harvey as a
powerfully morbific cause, while to the lack of the same narcotic its
occurrence was ascribed by Van der Mye. More recently it has been
referred by Fabre[6] to vaso-motor disturbance due to a miasm.

[Footnote 6: _Des Rélations Pathogéniques des Troubles Nerveux, etc._,
Paris, 1880.]

In the drink and food, however, most observers have sought the exciting
causes of scurvy. Instances have been reported where the disease seems
to have depended upon the use of impure water, etc. The imagination has
been tortured to seek in some quality or sort of food the specific
origin of scurvy. With regard to quantity, it may be stated that in
severe famines scurvy may or may not occur according as the food,
though scant, is in due proportions of animal and vegetable, though it
is true that the ordinary conditions of a famine preclude the
procurement of succulent vegetables. The quality of the food has
nothing further to do with the production of scurvy than by impairing
the general health, for it has often happened that putrid food has been
long used without scorbutic symptoms arising. The kind of food is
equally {171} innocent, although various special articles have been
charged with specific activity. The frequency of scurvy in Brabant was
attributed by Ronseus to the use of aquatic birds; Sherwin and Nitsch
assigned the same peculiarity to a free use of fish; and Henry Ellis to
the too free use of spirits. Even the generally widespread and
much-esteemed article of diet sugar was in disrepute with Willis. The
too free use of salted meats has been often accused of causing the
trouble. The fat rising on water in which salt provisions were boiled
was considered by Cook and Vancouver to be of particular pernicious
effect, and even the copper vessels in which they were cooked were
condemned by Travis as able to communicate the scorbutic poison to the
food. To the milk of animals browsing on verdure upon which pernicious
dew had fallen was referred an epidemic which occurred in Silesia in
1591. Diseased potatoes were considered sufficient to determine scurvy
in Ireland and Scotland by O'Brien.

The scurvy occurring on land was deemed to be different from that
occurring at sea, and its frequency afloat brought into unmerited
disrepute the sailor's salt diet, and its saline materials were even
considered the chief offending cause. This idea was rejected by
numerous observers, who assigned as the chief causative rôle in scurvy
deficiency in vegetable food, especially of the fresh, succulent
variety. The particular constituent of this sort of food, so powerful
in warding off scurvy and of curing it when prevention has failed, has
baffled discovery. Dr. Aldridge attributed it to mineral elements
generally. Dr. Garrod singled out the potassic salts as the particular
one to which the specific action must be attributed; but neither of
these views has gained in credit. From all the facts, both positive and
negative, we may reasonably assume that the essential dietetic error
leading to the development of scurvy, in the immense majority if not in
all cases, consists in a deficiency in the variety of food; that is to
say, there is not the requisite proportion of animal matter with a
diversity of vegetable substances. No single natural order contains
plants that supply all the elements essential to the nutrition of the
body and the right composition of the blood. The graminaceous and
leguminous articles of food, for instance, are numerous, but not
various; they all afford the same or analogous albuminous elements,
which have about the same nutrient value as the corresponding
substances in animal food, and hence health and vigor cannot be
sustained on a diet of flesh, combined with wheat, rice, and oatmeal or
with beans and peas, or with all of them together. Outbreaks of scurvy
have occurred on shipboard, where the ration is made up principally of
these articles; as in Anson's ship, when supplied with an abundance of
fresh animal, farinaceous, and leguminous foods. It is clear,
therefore, that in order to obtain a variety of materials required in
nutrition, we must resort to several of the natural groups, those
particularly which comprise the succulent vegetables and fruits.

MORBID ANATOMY.--The bodies of persons dead of scurvy are, in most
cases, much emaciated, because the quantity as well as the quality of
the food has usually been defective. When the food-supply is abundant
and only lacking in the elements indispensable in warding off scurvy,
the bodily weight is not noticeably decreased, although the
characteristic tissue-changes of scurvy are present. This was
noticeable in the cases recorded by Trotter of negro slaves dying of
scurvy while their bodies {172} presented a fat and sleek appearance.
Rigor mortis usually sets in early, and chemical decomposition invades
the tissues speedily. The skin presents the discolorations and
blotchings observed during life. The subcutaneous connective tissues
are soaked with serous exudations, especially in the lower extremities,
and in various localities are infiltrated with bloody or fibrinous
extravasations. The same changes occasionally affect the muscles, the
infiltration occurring beneath the fibrous sheaths and into the
intermuscular spaces, and the fibres are more or less torn. These
effusions occur most frequently about the knees, the elbows, and the
pterygoid muscles of the jaw.

The bones are sometimes necrosed by the mechanical influence of copious
effusion beneath the periosteum, forming nodes of varying sizes and
obstructing the supply of blood. The joints are occupied by serous or
bloody transudations; their synovial investment is destroyed in part,
so that the cartilage is exposed; and the latter not infrequently is
softened, and even separated from the subjacent osseous connections.
Sometimes the morbid changes occurring in the joints are the results of
disease in the subcutaneous connective tissues surrounding them.

The muscular system presents marked changes. The muscles undergo fatty
degeneration in a remarkable degree. The changes begin first in the
lumbar muscles, the fibres losing their striations and sarcolemma, and
finally being replaced by granular and fatty matter.

The brain has been found in rare instances the seat of softening and
infiltration, and the ventricles may contain serous or bloody fluid.
Similar effusions have also been noted in the arachnoid. Most
frequently, however, the brain and its membranes present an anæmic
appearance, there is less blood than natural in the vessels, and the
tissues are pale. Very often no changes whatever are observed.

The heart is smaller than normal, relaxed, and flabby, its fibres
easily broken, and a cut surface presents the yellowish aspect of fatty
degeneration in certain parts, with occasional extravasations located
in the cardiac walls. The valves of the heart are relaxed and illy
adapted to accurate closure. In certain recent cases soft coagula or
dark fluid blood, and in others firmly coagulated blood, are found in
the cavities; in those which have been prolonged the blood is more
likely to be found fluid and the coagula diffluent. The endocardium is
often blotched to a greater or less extent by sanguineous imbibition.
The pericardium often contains serum, and in the worst cases is
inflamed, lacerable, and contains bloody effusions. The inner surface
of the great vessels at the base of the heart is stained by imbibition.

The respiratory organs are variously affected. The mucous membrane
lining the nose, larynx, and trachea is generally pale and flecked with
extravasations of a dark-red color; more or less frothy fluid, tinged
with blood, is present in these passages, and occasionally oedema of
the glottis is encountered. The lungs are, as a rule, infiltrated with
a bloody serosity, particularly in those cases with renal complication,
or with a fibrinous or bloody exudation. The posterior portions of the
lobes often present evidences of hypostatic congestion, or even of
gangrene, and in the latter case the tissue is easily friable and emits
a disagreeable odor. Their surfaces are mottled with superficial
discolored patches of varying size and outline. The lungs may, on the
other hand, be found pale, {173} with empty collapsed vessels and with
little or no effusion. The pleural cavities commonly contain a serous
fluid, or, in rare cases, a copious effusion of blood. Traces of
inflammation and discoloration by sanguineous staining are traceable on
the pleural surfaces.

The digestive organs furnish strongly marked lesions. The mouth
presents the most constant scorbutic feature, a stomatitis in which the
gums are infiltrated, spongy, livid, and the seat of fatty
degeneration; the teeth are loosened or have already fallen out. The
stomach and small intestines are thin-walled, and the mucous membrane
is often softened, and in places ulcerated; similar changes have been
noted in the solitary glands. Follicular ulceration of the large
intestine occurs, with softening and infiltration of the mucous
membrane. Hemorrhagic effusions into the mucous membrane, forming
stippling, flecks, or patches, occur in various degrees along the whole
extent of the alimentary canal. The pancreas is occasionally found
softened and containing hemorrhagic effusions.

The kidneys are, as a rule, found in the normal condition in cases in
which albumen has been observed in the urine. Occasionally they are
engorged, with infarction of the cortical substance, and the mucous
lining softened and thickened and covered with blood-tinged mucus, or
they may present various degrees of parenchymatous degeneration. The
ureters and bladder sometimes present ecchymotic spots, and the
contained urine is mingled with blood.

The liver is always more or less altered by fatty degeneration, and at
times replete with blood and softened, and its surface ecchymotic. The
spleen is occasionally greatly enlarged, and its tissues very
lacerable, laden with blood, and infarcted.

PATHOLOGY.--The essential character of scurvy consists in perverted
nutrition, in which the blood undergoes such peculiar and profound
changes that its fitness for the maintenance and renewal of the various
tissues and organs is impaired; hence the nervous depression, loss of
muscular power and tonicity of tissues, and the transudation of the
blood or of its constituent parts.

The processes of secondary assimilation are chiefly at fault, leading
to the blood-changes, and through these to the textural lesions.
Primary assimilation remains intact, as the bodily weight is little
altered as long as the food is in sufficient quantity. This loss of
nutritive balance between the blood and tissue is due to the absence of
certain elements furnished by fresh vegetable matter. What these are,
and how their absence acts in inducing this disturbance, have not yet
been determined; we only know that the mysterious harmony of the vital,
chemical, and physical relations which exist between the blood and
tissues in health is deranged by their absence.

Endless explanatory surmises and assumptions have been proffered. The
earlier explanations involve either the Galenical theory of
putrefaction of the fluids and humors, a breaking down of the
blood-corpuscles, or the later chemical theories of superabundance or
absence of certain salts, sulphur, etc., and hence there were an acid
scurvy, an alkaline scurvy, a muriatic scurvy, etc.

The frequent effusions of blood in scurvy led Andral to suspect that
the chief peculiarity in scorbutic blood was the decrease of fibrin;
which was {174} in perfect accord with a theory that he had formed that
this change was the uniform cause of passive hemorrhage. Magendie had
already given experimental support to this conjecture by inducing in
animals phenomena analogous to those of scurvy by the injection into
the veins of defibrinated blood or of alkaline solutions. Andral[7]
believed his views confirmed when in 1841 he analyzed on two occasions
the blood of scorbutic patients and found the fibrin reduced to 1.6
parts per 1000. Similar results were obtained by Eckstein and Frémy. On
the other hand, the blood was analyzed by Busk, about the same time, in
three well-marked cases of scurvy that occurred on the Dreadnaught
hospital-ship, and in all of them the fibrin was in excess of the
normal amount, the least being 4.5 and the greatest 6.5 parts per 1000.
In perfect accord with Busk's results were the analyses of the blood of
five scorbutic females, communicated in a note to the Academy of
Sciences in 1847 by Becquerel and Rodier. In no case was the fibrin
diminished, but in some it was sensibly increased. In a subsequent case
Andral found that the fibrin, instead of being less, exceeded the
physiological mean, reaching 4.4 parts, and he concluded that a
diminution of this element was not a necessary and common occurrence,
but only an effect--a result of prior morbid modifications, and a
consequence which was produced more or less frequently according to the
severity and duration of the disease. Parmentier and Déyeux found the
blood of three scorbutics to resemble inflammatory blood in respect to
fibrin, while Frick obtained in one analysis 7.6 parts of fibrin and
Leven 4.3 parts.

[Footnote 7: _Essai d'hématologie pathologique_.]

In mild cases of scurvy neither the color, the alkalinity, nor the
coagulability of the blood differs from that of blood in health, though
Wood alleges that the clot is loose and cotton-like, and Canstatt that
its coagulability, in consequence of the large proportion of saline
matters, is diminished. In Busk's cases the separation of the clot and
serum was as perfect, and took place as rapidly, as in healthy blood,
and in two of them the blood was both buffed and cupped, as it was also
in Leven's cases. In two of the most severe of Becquerel's cases the
blood coagulated firmly, and in a slight case the clot was dark and
loose. The albumen of the blood shows no marked change as regards its
quantity. The five analyses of Becquerel and Rodier showed the average
amount of organic matters of the serum to be 64.3 parts in 1000, the
smallest being 56.2 and the largest 69.2 parts. 1000 parts of the serum
of the same cases gave an average of 72.1 parts of organic matter.
Frick's single case gave 87.045 parts per 1000, and the average of
Busk's was 78.2 parts, while Chotin and Bouvier obtained only 62.3
parts. The last-mentioned writers have recorded a fact in connection
with the physical characters of scorbutic blood that deserves notice:
the blood in one case did not coagulate at the usual temperature (about
158° F.), but required a temperature some degrees higher for that
purpose. The red corpuscles in all the foregoing cases were notably
diminished, the largest amount given being 117.078 parts per 1000,
while the lowest was 47.8 parts. In Andral's second case the globules
had decreased to 44.4 parts per 1000, the lowest amount yet recorded.

The alkalinity of the blood seems not to be changed, although Chotin
and Bouvier noticed a slight increase. The saline constituents do not
{175} vary greatly from the normal standard. The average amount in the
cases of Becquerel and Rodier and Busk was 8.1 per 1000, the smallest
being 5.5 parts and the largest 11.5. In Ritchie's two analyses the
proportion of saline matters is given as 6.44 and 6.82 parts per 1000.
Opitz and Schneider have found less than the physiological mean. In
Frick's case the amount was 8.8, the iron being 0.721 parts per 1000,
and 0.782 to 127 parts of globules; lime 0.110, chlorides 6.846, and
phosphates 1.116 parts per 1000. The iron was in excess of that in the
normal blood, but in Becquerel's cases the mean was 0.381--less than
the normal. The proportion of iron in Duchet's cases was respectively
0.393, 0.402, and 0.476 parts, giving a mean of 0.423 parts per 1000,
which nearly approximates the normal. Garrod in one analysis of the
blood found a deficiency of the potassium salts, upon which he erected
his well-known theory of the etiology of the disease. It is an
interesting fact that in the physiological state the quantity of sodium
chloride is not subject to variation, any excess introduced with the
food being thrown off by the kidneys. The quantity in the urine bears a
relation to the amount introduced as food, but the proportion in the
blood is constant.

The quantity of water in the blood has been found to be increased in
all the analyses which have been made. Chotin and Bouvier estimated
water and loss at 831.1; in Frick's case it was 791.69 parts per 1000;
and in Becquerel's five cases it was put at 807.7, 810.9, 811, 813.7,
and 854.0 parts per 1000, respectively. In Busk's three cases the
lowest amount was 835.9 and the highest 849.9 parts per 1000. The
specific gravity of the defibrinated blood was in all cases low in
comparison with the normal standard (1057), the average in Becquerel
and Rodier's cases being 1047.2, the lowest 1083.3, and the highest
1051.7. In the single observation of Chotin and Bouvier it was 1060.
The specific gravity of the serum was also less than normal (1027), the
average of four of Becquerel's analyses giving 1023.8, the lowest
1020.8, and the highest 1025.5. Busk gives 1025 in one case and 1028 in
another.

The results of the most recent analyses, those of Chalvet, are shown in
the following table, in which scorbutic blood is contrasted with that
of a healthy, robust female:

                                      Scorbutic blood.  Healthy blood.
  Water                                    848.492         772.225
  Solid matters                            151.508         220.775
  Dry clot                                 140.194         209.000
  Albumen                                   72.304          68.717
  Fibrin                                     4.342           2.162
  Globules                                  63.548         138.121
  Extractive matter--by absolute alcohol    10.312           8.013
                     by ether                1.002           1.300
  Ashes of clot                              3.000           5.691
  Peroxide of iron of globules               1.060           2.259
  Potassium of globules                      0.329           0.625

From the conflicting statements of various observers the following
conclusions may be drawn: that in scorbutic blood water is in excess;
that there is, on the one hand, a marked increase of the fibrin, and in
a less degree of the albumen and extractive matters, while on the other
hand there is a marked decrease of the globules and in a less degree of
the mineral matters. On the authority of Chalvet it may be also stated
{176} that demineralization of the muscular tissue is a notable
chemical feature in scurvy.

So far, microscopic examination has been entirely negative. Hayem[8]
found no appreciable alteration from healthy blood, and in this view
Leven[9] concurs; while Laboulbène[10] notes the occurrence of an
unusual number of white globules.

[Footnote 8: _Mém. de la Société de Biologie_.]

[Footnote 9: _Communication to the Académie des Sciences_, 1871.]

[Footnote 10: _Epidemie de Scorbut_.]

Petrone Luigi[11] injected scorbutic blood into the connective tissue
of rabbits. In three instances the animals died, presenting on the ears
distinct evidences of the formation of petechial extravasations. The
viscera revealed everywhere bloody effusions of larger or smaller size.
The spleen was enlarged and its parenchyma and capsule distended. In
the blood were found oval, shining, spontaneously-moving corpuscles,
which he regarded as the bearers of the specific poison of scurvy.

[Footnote 11: _Annali Univers. di Med. c. Chir._, 10, 1880.]

SYMPTOMS.--The symptoms of scurvy are insidiously and usually slowly
developed under the influence of the efficient causes, and the disease
runs a chronic course, often extending over five or six months,
especially in cases in which the hygienic surroundings of the patient
have been imperfectly or not at all rectified. In light cases the
course is much shorter. A gradual alteration of the nutritive processes
first occurs, until what might be called a scorbutic cachexia is
established in a period varying from a few weeks to several months. The
initial symptoms consist in the skin losing its color and tone and
assuming a yellowish or earthy hue: it is relaxed, dry, unperspiring,
and rough; in the legs particularly this roughness is very marked, and
the skin, when rubbed, sheds an abundance of furfuraceous scales. The
cutaneous follicles, markedly on the extensor aspect of the lower
extremities, are prominent, similar in appearance and feel to the
condition known as goose-flesh. Rouppe[12] calls this the signum primum
pathognomonicum. Dark-red or brownish flecks, of a circular outline and
of varying but small size, not unlike flea-bites, appear on the face
and limbs. The cutaneous circulation is feeble and the superficial
warmth less than natural; slight depression of the atmospheric
temperature produces a sensation of chilliness, and the feet and hands
are cold. On assuming the erect posture the patient complains of
headache and dizziness. The muscles are relaxed and soft to the feel,
and a corresponding loss of vigor and strength is experienced by the
patient, who is indisposed to exert himself in the performance of his
customary duties and seeks repose and freedom from feelings of fatigue
and languor in recumbency. This prostration is occasionally so extreme
that the slightest efforts in attempting to stand or walk are attended
with rapid action of the heart, accelerated respiratory movements, and
a sense of suffocation and breathlessness. The general circulation is
impaired; the heart acts feebly; the arteries are contracted; and the
pulse is slow, small, and compressible.

[Footnote 12: _De morbis navigantium_.]

The mental powers are equally impaired. The face wears a haggard
appearance and depressed expression; gloomy forebodings of evil and
disinclination to turn the attention to the usual mental pursuits are
markedly present--a disinclination that may subsequently merge into
complete apathy or indifference to passing events, or even into
somnolency.

{177} Pains in the legs, joints, and loins are early manifestations:
they closely resemble those of rheumatism, for which they are often
mistaken. The pains are not exacerbated at night, but, on the contrary,
are often more severe by day. Not unfrequently lancinating pains in the
muscles of the chest are complained of. The sleep is not disturbed
until the disease has made some advance, when it becomes broken and is
no longer refreshing.

The appetite is usually unimpaired in the early periods of the disease,
and even throughout its course the condition of the mouth alone
prevents the patient from indulging his desire for food, even, as is
occasionally noticed, to voracity. There may be a yearning for certain
articles of diet, principally those of an acid character; but, on the
other hand, some cases present exactly the reverse condition--a disgust
for food in general or for particular varieties; or the appetite may be
vacillating, at one time craving and at another repelling nourishment.
There is no noticeable change in the normal thirst, except on the
occurrence of febrile complications, when it is increased. The gums do
not, at this stage of the disease, present the livid, swollen
appearance of fully-developed scurvy, but, on the contrary, are
generally paler than usual, with a slight tumid or everted line on
their free margins, and are slightly tender on pressure. The breath is
commonly offensive, and the patient complains of a bad taste in the
mouth. The tongue is flabby and large, though clean and pale, and the
bowels are inclined to be sluggish.

This preliminary stage is followed, after varying intervals of time, by
certain local phenomena which are quite characteristic of the disease.
There is a marked tendency to extravasation of blood into the tissues,
either causelessly or upon the infliction of slight injuries or wounds.
Fibrinous exudations occur sooner or later into the gums, which become
darkened in color, inflamed, swollen, spongy, and bleed upon the
slightest touch or even spontaneously, and finally separate from the
teeth. These results are due, in part, to the considerable amount of
pressure to which these parts are subject in mastication, and it is a
conspicuous fact that the gums of edentulous jaws remain free from
these changes. In a few cases the gums are but slightly altered,
perhaps oedematous only or pitting upon pressure, or they become the
site of bloody extravasations. In severer examples, in later stages of
the disease, these various alterations progress to an extreme degree,
and the extravasation is so voluminous that the gums present great,
fungous, lacerable excrescences, which may finally break down into a
suppurating, brownish, and very fetid mass, communicating to the breath
an odor of a most offensive character. In certain epidemics of scurvy,
notably in that of Florence described by Cipriani, the lesions of the
gums were absent. The rest of the mucous membrane of the mouth remains
unaltered, or at most slightly ecchymotic. Samson and Charpentier[13]
in a large number of cases saw this but once, and in one of Leven's[14]
cases the fungous growth invaded the palatal mucous membrane, extending
to the anterior pillars of the fauces. The salivary glands are enlarged
and swollen; the tongue is imprinted with the form of the teeth, while
the latter become encrusted with tartar and more or less concealed by
the exuberant gums, or, becoming gradually loosened from the alveoli,
finally drop out. The morbid process may extend to the bone itself, and
necrosis and extensive {178} exfoliation follow. Mastication is more or
less painful, and often impossible, so that the patient is reduced to
the necessity of prolonging life by the use of fluid or semi-solid
food. Under the influence of appropriate treatment it is remarkable how
rapidly (in from two to four weeks) these marked changes recede and the
parts resume their normal condition, yet it occasionally occurs that
permanent, callous thickening of the gums results.

[Footnote 13: _Étude sur le Scorbut_, 1871.]

[Footnote 14: _Une épidémie de Scorbut_, p. 28, 1872.]

In the progress of the disease effusions of blood under the skin are of
early occurrence. They are at first located in the superficial stratum
of the cutis or just beneath the epidermis, especially around the roots
of the hair, and present themselves as roundish, bluish-red flecks,
varying in size from that of a pin's head to that of a split pea, not
effaceable by pressure with the tip of the finger, but slightly, if at
all, elevated above the surface, and enduring for weeks together. The
nutrition of the hair-follicles is impaired, so that the hairs are
often either lost, broken, or distorted. These petechiæ fade in color
with progressive improvement in the case, and finally disappear,
leaving brownish-yellow discolorations. They first appear on the
extremities, particularly the lower limbs, then on the face, and lastly
on the trunk. At a later period extravasations of a larger size and
more irregular form occur in the deeper layers of the derma. They vary
in size from that of a finger-nail to blotches two or three inches in
diameter; at first reddish in color and subsequently of a bluish red.
When recession occurs under appropriate treatment, the color passes
through various shades of violet, blue, green, and yellow, as in
ordinary traumatic ecchymosis. Outpourings of blood also occur into the
subcutaneous connective tissue, notably that of the legs, and in
localities where connective tissue is particularly abundant and loose,
as in the ham and axilla. The dispersion of blood in this tissue may be
so considerable as to cause the legs from the knees down to present a
uniform dark-blue coloration that in form may not inaptly be compared
to a stocking. The upper extremities also suffer, usually on their
inner side from the armpit down, the extravasation rarely reaching,
however, to the hand. These extravasations may take place after the
infliction of very slight injuries, as from blows or the pressure of
hard bodies, or even from the mechanical effects of prolonged
dependency of the limbs, as in riding on horseback. Extravasations of a
similar nature are occasionally present in the connective tissues of
the muscles themselves or between them, giving rise to swellings of
various forms and dimensions. Nearly always along with the sanguineous
effusions there is more or less oedema, usually beginning at the ankles
and gradually extending upward; in some cases there are puffiness of
the face and general anasarca, so that deep pits remain on pressure.

This profound impairment of nutrition of the skin continuing, in the
worst cases blood is effused beneath the cuticle, forming blebs of
varying size, which finally break and leave superficial ulcerated
surfaces, that ultimately become covered with flabby, exuberant
granulations, pouring out a purulent often offensive sanies and
bleeding upon the slightest touch.

In some cases the ulceration begins in the petechiæ at the hair-roots,
and a number of these, running together, form a large ulcer. The
destruction of tissue by ulceration is disposed to spread more widely
and deeply, and is often of a most intractable character. Old
cicatrices {179} are the first tissues in these cases to take on the
ulcerative action. Certain muscles, chiefly those of the legs, and
notably the gastrocnemii, the abdominal and pectoral muscles, the psoas
magnus, and pterygoids, may become the seat of fibrinous
extravasations, which finally change, by lapse of time, into hard, firm
tumors, impairing the functions of those parts and leading to
contractions of the limbs.

The symptoms in certain epidemics of extraordinary severity have
displayed alterations in still deeper structures. Effusions occur
between the periosteum and the bone, forming painful, hard, and
resisting nodes of varying dimensions, especially along the course of
the tibiæ, upon the scapulæ, and upon the maxillæ. In young persons the
epiphyses are separated from the shaft of the long bones, and in other
cases the ribs become necrosed and disarticulated from the sternum,
producing a creaking noise during respiratory movements, as related by
Poupart.[15] This occurs mostly on one side and about the middle of the
series, yet it has been noted to occur on both sides, so that the
sternum and attached cartilages, deprived of support, were perceptibly
sunken. Oserctzkowski[16] reports two fatal cases of scurvy attended
with spontaneous fracture of the ribs. There was extravasation into and
beneath the periosteum, and subsequent destruction of the continuity of
the bone. In one case the ribs on both sides were affected, so that the
anterior wall of the thorax sunk in and embarrassed the respiration,
which was chiefly maintained by the diaphragm. Inflammation of the
lungs succeeded, and the patient died in agony.

[Footnote 15: _Mémoires de l'Académie des Sciences_, p. 237, 1699, and
_Philosophical Transactions_, vol. xv.]

[Footnote 16: _Wratsel_, No. 51, 1881.]

Recently-repaired fractures have been known to recur under the
influence of scurvy from the destruction of the callus.[17]

[Footnote 17: Anson's _Voyage Around the World_, edited by Walter.]

The articulations as well as the bones in very severe cases of scurvy
present evidences of disease, consisting in periostitic effusions which
involve the surrounding soft parts, producing impairment of motion,
enlargement, and false ankylosis, and even destroying the normal
anatomical relation of the osseous surfaces, so as to determine
deformities. These changes are usually attended with severe pain, and
most commonly occur in the ankle-, knee-, shoulder-, and hip-joints,
and disappear tardily, requiring perhaps months for their recession, if
indeed this takes place at all.

The symptoms manifested by the circulatory organs are prominent from an
early period of the disease. The pulsations of the heart are slower,
feebler, irregular, and often intermittent; its impulse is decreased or
becomes quite imperceptible; and when the associated anæmia has
progressed to a certain extent a systolic murmur may be audible. The
arterial and venous channels are of diminished calibre; the pulse
becomes soft, of less volume, and tardier; and a venous murmur may
sometimes be heard in the cervical veins. The remarkable nutritive
changes in the capillary walls in part account for the numerous
hemorrhages which occur both by rhexis and diapedesis. The most
frequent is epistaxis; the slightest blows, sneezing, or blowing the
nose will often determine it, or it may occur spontaneously, and in
severer cases with such profuseness as to threaten impending
dissolution, requiring nothing less than timeous introduction of the
tampon to rescue the victim. Hemorrhage from the {180} lungs is of rare
occurrence, and when it does happen is rather indicative of
pre-existing pulmonary disease, such as phthisis, or of the approach of
a complication, such as infarction or gangrene, than a constituent
feature of scurvy. Hæmatemesis is less uncommon, but is by no means
frequent; the blood ejected from the stomach is usually small in
quantity, but in isolated examples the bleeding is profuse, producing
great exhaustion and a sense of cardiac depression which preludes
speedy death. Hemorrhage from the bowels is also an ill-omened feature,
completely blanching the patient and presaging early exhaustion and
death. Blood may also appear as a product of a complicating dysentery
which determines abundant, offensive discharges that may run on for
several weeks before the patient is finally exhausted. Hæmaturia
sometimes occurs, especially in broken-down and cachectic subjects and
in an advanced stage of scurvy. All of these forms of hemorrhagic
effusion, now mentioned as localized in the mucous membranes, are to be
deprecated as exercising a pernicious influence, seriously aggravating
ordinary cases and fatally jeopardizing the issue of severe ones.

Effusive and inflammatory complications are also encountered in the
serous structures, and usually in cases of great severity, though they
occasionally present themselves when the more common localized
phenomena of scurvy are not particularly prominent. These complications
may be marked by a gradual accession, or they may rapidly arise and
involve the patient, just before in apparent security, in the greatest
peril. These incursions are almost always attended by febrile
exacerbations and the usual grouping of clinical characters denotive of
the same pathological conditions arising under ordinary circumstances.
The local complications may either affect the pleura or pericardium, or
both. In Karairajew's[18] 60 autopsic examinations pericardial
effusions were noticed in 30, pleural in 30, pericardial and pleural in
6, peritoneal in 7, and arachnoidal in only 1. The exudations are
sero-sanguinolent or fibrinous in character, and sometimes reach the
inordinate quantity of four or five pounds, occasioning the patient the
utmost distress and embarrassing the respiratory and circulatory
functions. Although these augment in a high degree the risk to life,
yet under prompt and appropriate treatment recovery may take place and
the effusions vanish with surprising rapidity.

[Footnote 18: Himmelstiern, _Beobachtungen über den Scorbut_, S. 50,
Berlin, 1843.]

Hemorrhagic extravasation into the nervous centres is a very rare
occurrence. It has not been as yet recorded as having occurred in the
brain-substance itself, but has in several instances been noted between
the meninges, producing headache, dizziness, vertigo, and sometimes
somnolence, delirium, and coma. Opitz[19] relates an interesting case
in which convulsions suddenly occurred with unconsciousness, followed
by hemiplegia of the left side of the body and the corresponding side
of the face. After twenty-four hours consciousness returned and the
paralysis disappeared. There were, however, headache and hyperæsthesia
of the upper extremities present; twelve days later these also receded,
and the patient finally recovered. The same author records paralysis as
occurring in one case from extravasation into the spinal meninges.
Samson observed an instance in which a fibrinous effusion formed upon
the sciatic nerve, with consequent pain.

[Footnote 19: _Prag. Vierteljahrschrift_, S. 153, 1861.]

{181} In the circulatory system symptoms always of threatening and
often of fatal import may arise: embolism may occur at various points,
particularly in the lungs and spleen, occasioning hemorrhagic
infarctions, which have undoubtedly been the occasion of the sudden
deaths sometimes observed in scorbutic cases not apparently of a very
dangerous form nor attended with an excessive degree of exhaustion.

The urinary system supplies no prominent symptoms; the statements as to
the condition of the kidneys and the composition of the urine are
contradictory. The urine not infrequently contains albumen,
particularly in severe cases, but this is by no means indicative of
corresponding changes in the renal structure: on the contrary, this may
be found after death to be apparently free from disease.

The conclusions that would seem to be authorized by the statements of
various authorities are that the quantity of urine passed is decreased,
as well as that of the urea, while the amounts of the albuminoid and
mineral matters are increased.

Physical examination will reveal the frequent occurrence of enlargement
of the spleen, independent of malarial influences, and Krebel has
encountered one case in which the liver was involved in inflammation.

Some derangement of the visual organs is present in many cases. Foltz,
in the epidemic on the Raritan, reported four cases of nyctalopia and
two of hemeralopia, and other affections of the eye, such as
conjunctivitis, induration and irritation of the ciliary margins of the
lids, with a copious and acrimonious discharge, these conditions being
obviously due to the scorbutic diathesis. Medical Director J. Y.
Taylor, U.S. Navy, in a private communication to me states that
hemeralopia was a frequent premonitory symptom of scurvy that occurred
in the U.S. sloop-of-war Decatur in 1854 during a laborious and tedious
passage of three months through the Straits of Magellan. The men were
overworked and much exposed to cold and wet, and part of the time were
on diminished rations. The hemeralopia was at first erroneously
attributed to the reflection from the snow and glaciers--a species of
snow-blindness--but other phenomena speedily appeared in a majority of
the causes: a subacute inflammation, with considerable pain and
swelling of the small joints, especially those of the toes; sore and
tender gums, although only a few progressed so far as to exhibit
sponginess or bleeding; and debility, depression, anxiety, and
insomnia. In a few cases the blindness was so complete as to render
their subjects almost helpless after sunset. This was the most
pronounced and remarkable symptom and the one most complained of. These
incipient scorbutic symptoms were promptly arrested by the free use of
wild celery (Apium graveolens), which was found growing abundantly in
sheltered places. The short rations were also supplemented
advantageously by mussels (Mytilus edulis) whenever they could be
obtained. A few weeks later the crew appeared to be in ordinary health.

Hemorrhage may occur under the conjunctiva, raising it into small
pouches; into the anterior chamber, causing iritis and adhesions; and,
finally, into the choroid and vitreous humor, exciting a general
inflammation of the entire organ.

Dulness of hearing and buzzing in the ears have also been signalized as
occasional symptoms of scurvy.

{182} The phenomena of fever are always absent during the course of
uncomplicated scurvy, the temperature of the mouth sometimes falling as
low as 92° F., and being always one or two degrees lower than normal.
It is only in the later periods of the disease, when pathological
processes most often supervene in the internal organs, that an elevated
temperature and the other ordinary symptoms of fever are manifested.
The lowered vital resistance of scorbutic subjects particularly
disposes them to the incursions of fevers, especially those of malarial
and typhoid types: hence in the low, marshy districts of Northern
Europe and in sections of country afflicted by famine and overcrowded
dwellings these complications are very common.

DIAGNOSIS.--Little or no difficulty will be encountered in
discriminating scurvy from other diseases under the circumstances that
usually surround its development and prevalence. These circumstances
are altogether peculiar and characteristic, and involve the absence of
succulent vegetable food as the prime factor, and exposure to cold,
fatigue, mental despondency, or other depressing influences as
accessory in its production. This combination of causes has been
usually witnessed in all the outbreaks of scurvy in camps, besieged
towns, on shipboard, particularly on ships in Arctic service.

Sporadic cases may escape immediate identification in the absence of
some of these circumstances, but a close attention to the symptoms will
surely lead to a correct conclusion. The scorbutic cachexia denoted by
the sallow or earthy hue of the skin; the spongy gums; the
discoloration of the surface; pains in the limbs and joints; the sense
of weariness, and, later, the exhaustion, dyspnoea on the slightest
exertion; the bloody and fibrinous effusions into the connective
tissues and muscles about the joints, and into the pleuræ, pericardium,
and peritoneum; the stiffness and contraction of the legs,--furnish a
complexus of phenomena not met with in any other disease than scurvy.
The discoloration of the skin in purpura, leucocythæmia, anæmia,
chlorosis, and hæmatophilia, or other conditions involving hemorrhagic
extravasation, are easily discriminated from those of scurvy when taken
in connection with the other symptoms and the history of those
diseases. In the beginning of scurvy the pains in the back and limbs
might divert the attention to rheumatism, but an examination at this
early stage will, in all likelihood, disclose the peculiar gingival and
cutaneous lesions of scurvy.

The rapid improvement of scorbutic cases under a fruit and vegetable
diet is also a noticeable feature not witnessed in any of the foregoing
diseases.

PROGNOSIS.--The prognosis of scurvy is always favorable in the early
stages, and even in the very worst recovery occurs under improved
hygienic surroundings with remarkable promptness and certainty. It must
not be overlooked, however, that sudden death may occur in seemingly
light cases from failure of the heart's action or from embolism. There
is a ready disposition to the recurrence of the disease under slight
causes, and it may so impair the health as to lead to the development
of other fatal maladies. The gravity of the case is to be gauged not so
much by its seeming severity as by the accessibility of proper
food-supplies, for without these the worst results may be expected.
Where the case is embarrassed with complications of the respiratory and
circulatory {183} organs, involvement of the bones, and intercurrent
diseases, the outlook becomes correspondingly grave.

Throughout the world, in recent times, greater areas of territory are
devoted to agriculture and horticulture, and the products are
distributed over wide extents of country by the increased facilities of
communication by the highways and railroads, so that it would now be
impossible for an epidemic of scurvy to devastate a region of country
so provided as it did a century ago, or might do and has done in
regions of country where tillage is neglected and communications are
cut off by an absence of roads from more productive centres, as in
Southern and Eastern Russia.

Hygienic improvements that have almost stamped out scurvy on shore have
also done good service for mariners, and thousands of ships now cross
the ocean on long cruises with perfect security from the disease. In
the naval services of the world, as has been already shown, the disease
is rarely encountered, and it is greatly diminished in the merchant
marine, from which, it is hoped, in a few years, by a more rigid
enforcement of existing laws for the protection of sailors, it may also
entirely disappear. Even in exceptionally long and arduous cruises, as
in the Arctic regions, the disease may be arrested, as was the case
with the Jeannette, which was drifted about, locked up in ice, for
sixteen months, yet only a single case of scurvy appeared.

It is of the first importance to enlist a healthy crew for long
voyages, free from previous syphilitic, scorbutic, or other
constitutional taint; then, by observing proper hygienic precautions,
to maintain their health. One of the prime factors in securing this
result is a suitable dietary. The improved methods of preserving food
afford facilities for storing up adequate quantities of both kinds,
animal and vegetable, to last the cruise. To economize these stores it
will be well to start with a stock of live animals and recent
vegetables, such as can be now had in almost any quantity in any
considerable maritime city, and not until these are consumed are the
canned and preserved supplies to be opened. All the ordinary meats, as
beef, mutton, veal, and lamb; most vegetable products, as asparagus,
beans, peas, potatoes, and a great variety of fruits, as peaches,
plums, berries, etc., are obtainable at moderate expense, and should
form an integral portion of the ration. Eggs can be easily preserved so
as to keep for months by simply packing them in plaster or in salt, and
they furnish a valuable and acceptable article of diet. Among articles
of great nutritive value milk takes high rank, and it can be preserved
sweet and pure indefinitely. Sauer-kraut is an antiscorbutic of
considerable virtue, and should not be overlooked in laying in stores
for a distant cruise. Cheese and oatmeal will be found useful additions
to the ordinary ration.

It may be proper to state in the event of the occurrence of scurvy and
the exhaustion of the fresh vegetable stores that various
quickly-growing vegetables, such as mustard, radishes, turnips, and
cresses, could be cultivated on shipboard if seeds are provided.

With such a varied dietary, comprehended in the above enumeration, it
would be impossible for scurvy to invade the ship's company, especially
when aided by other wholesome agencies, as cleanliness, well-ventilated
and dry sleeping rooms, and clothing adapted to the weather. The
antiscorbutic virtues of lime-juice were known long ago, being
mentioned by {184} Albertus in 1593, but it was not until many years
later that it became an integral part of the English navy-ration. The
law requires it to be carried on board all merchant vessels, and to be
served out ten days after the crew has been living on salt rations. The
juice keeps well if properly prepared and preserved from contact with
air, especially when fortified with a small quantity of alcohol, the
usual strength being about 10 per cent. It should be carried in vessels
containing just enough to furnish a few days' rations to the whole
crew, by which plan only a small amount need be exposed to the
decomposing influence of the air. The juice can be reduced by
evaporation to a very small bulk. This method was adopted in supplying
the Arctic cruiser Rodgers.[20] The juice was reduced to a paste, each
pound of which represented one gallon of the solution of the ordinary
strength. It has also been used in the form of lozenges and biscuit. It
may be stated that great reliance has been placed upon malt, the acid
wines, and cider as good antiscorbutics.

[Footnote 20: _Report of the Surgeon-General of the Navy for 1880_.]

In connection with the food-supplies it is proper to mention those
influences of a depressing character which have a tendency to favor the
development of scurvy. The first is dampness in the sleeping apartments
of the men. This should be prevented by ventilation, drying stoves, and
taking care that no wet garments are permitted to remain in the
apartments. They should be taken off immediately and hung outside to
dry, and under no circumstances should the men be permitted to sleep in
them, as is sometimes done.

Exposure to cold is unavoidable under certain conditions, and the men
should then be protected by proper clothing adapted to the weather.
Protracted fatigue is a third favoring circumstance, and the crew
should be spared all the strain of hard work possible, especially in
high latitudes. The apartments should also be kept well ventilated and
scrupulously clean; and, lastly, depressing mental emotions, which are
so apt to arise from exposure to danger and want, should be dispelled
by cheering assurances, constant occupation, and whatever amusements
can be had. These are the chief influences which are to be considered
in adopting measures to prevent the occurrence of scurvy in
communities, armies, on shipboard, or in persons confined in houses of
detention.

The therapeusis of scurvy presents no intricate problems for solution.
Its origin in dietetic errors is admitted by almost common consent, and
it is surprising with what rapidity patients apparently beyond hope of
recovery gather health and strength with a change in the character of
the food. This is indispensable in the treatment, as drugs have little
or no curative influence without it; and, therefore, the first object
should be to supply the patient with lemon-juice or acescent fruits and
fresh vegetables, as garlic, mustard, cresses, sorrel, nasturtium,
taraxacum among the wild plants, and potatoes, onions, turnips, beets,
radishes, etc. among the domesticated plants. And in conjunction with
these fresh meats, in the form of soups if the solids cannot be
masticated, may be used with advantage. Ordinarily, the dietetic
treatment alone will suffice to re-establish the health. Should,
however, convalescence be delayed, the vegetable bitters with the
mineral acids and ferruginous tonics and quinia will furnish useful
adjuvants. These are the standard remedies; others have been
recommended at various times, as the juice {185} of the maguey, a
Mexican plant, potassium nitrate alone or combined with vinegar,
tincture of cantharides, etc.

Attention will often be required to the various scorbutic
complications, especially stomatitis, which is always a source of
discomfort and suffering. One of the best local applications for this
is pencilling the parts with a solution of nitrate of silver, which
often affords marked relief. Mouth-washes, composed of solutions of
chlorinated lime, potassium permanganate, carbolic acid, are beneficial
by suppressing foul odors, exercising local stimulative action upon the
gums, and promoting healing. Should ulceration attack the legs, as is
often the case, the application of mild astringents and stimulative
ointments will be all that is required. The parts should, of course, be
kept clean and protected from irritation by protective dressings.

Hemorrhages from the nose, gums, stomach, bowels, or into the serous
cavities should be treated upon the general principles applicable to
their character, as the local use of cold, astringents, and the
internal administration of hæmostatic agents--lead acetate, ergot,
tincture of iron, and other remedies, vegetable and mineral, of this
class. In desperate cases effusions into the chest, threatening death
by interfering with the respiratory and circulatory organs, may render
operation necessary as the last resort for their removal.

During the treatment it is important to obviate any sudden or severe
strain upon the heart by premature movements or exercises, as this is
fraught with danger.



{186}

PURPURA.

BY I. E. ATKINSON, M.D.


It has been customary with authors to describe under the general
heading Purpura a number of affections presenting as a common symptom
the extravasation of blood into the tissues, more especially of the
skin and mucous membranes, quite irrespective of etiological or
pathological considerations. Thus, the tiny ecchymoses caused by the
bites of fleas have been denominated purpura pulicosa; the larger
bruises resulting from external violence, purpura traumatica; the
extravasations occurring in the course of scurvy, purpura scorbutica;
those encountered in malignant small-pox, purpura variolosa; and so on.
These affections, differing widely in nature, possess as a common
symptom the escape of blood from the vessels into the tissues. It is
evident, therefore, that in the sense often employed the term purpura
is used to describe a symptom or symptoms common to a variety of
non-related maladies.

If there be a peculiar morbid process having for its constant and
characteristic symptom the spontaneous escape of the blood from the
blood-vessels, it is plain that interstitial hemorrhage from external
violence or from the action of a definite poison circulating in the
blood and disorganizing it and its containing vessels, as in
phosphorus-poisoning, or from the influence of certain zymotic
diseases, should not be designated by the title properly belonging to a
substantive malady. The question, therefore, is: Are there groups of
symptoms indicating morbid action of definite character, but of varying
intensity, to which the name purpura may with propriety be applied?

In the present light of pathological science it is impossible to answer
this question in the affirmative without considerable qualification. It
must be confessed that we do not possess a knowledge of any definite
chain of morbid processes constituting a distinct disease that may be
designated as purpura. And yet we are able to recognize a set of
symptoms varying greatly in intensity, from the most trivial petechial
eruption to profuse and fatal hemorrhages, accompanied by a train of
manifestations which we are unable to connect with any of the causes
already spoken of, and which, indeed, depend upon no fixed exciting
cause with which we are acquainted. It may be eventually proven that
purpura, even as we understand it, is merely a set of phenomena due to
widely-differing influences acting upon the blood and blood-vessels,
and that the term will disappear from our nomenclature as indicating a
disease, but will be preserved as denoting a symptom. For the present,
{187} purpura is understood to be a group of symptoms characterized by
the effusion of blood into the tissues of the body, or upon its free
surfaces, or into its serous cavities, which seem to arise
spontaneously, and for which we are unable to assign a definite cause.
With this view of the nature of purpura it becomes necessary to exclude
from present consideration blood-extravasations from internal or
external violence, the action of the specific principles of contagious
or infectious fevers, the dyscrasia of scurvy, the influence of
poisonous substances, and, in a word, any of those affections of which
the escape of blood from the vessels constitutes an epi-phenomenon.

Purpura may be conveniently considered as presenting three varieties:
1, purpura simplex; 2, purpura hæmorrhagica; 3, purpura rheumatica.

These three forms of the disease are not distinguished by
sharply-outlined differences, but merge the one into the other, now
one, now another set of symptoms predominating. To these may be added,
likewise for convenience, three sub-varieties--purpura urticans,
purpura papulosa, and purpura nervosa. The difference between these
forms of purpura should not be considered as of more than clinical
import. Whatever variations present themselves may with probable
propriety be ascribed to complicating influences.

PURPURA SIMPLEX.--This is the mildest form of purpura, and may in many
cases readily escape observation. It may begin abruptly, in the midst
of health, without the slightest subjective symptom, or the
extravasations may be preceded for several days by some discomfort,
aching of limbs, sluggishness, anorexia, even a small amount of fever.
The eruption usually appears first upon the lower extremities,
preferably the flexor surfaces of the thighs (Duhring), but frequently
upon the legs. It extends from these points to the upper extremities
and trunk, usually sparing the face. The lesions vary in size from that
of a pin-head to that of a fingernail (petechiæ), or they may be linear
(vibices). They remain discrete, and do not increase in size throughout
their course. Each spot of hemorrhage will endure for from one to two
weeks. At first the lesions are of a livid red color, and declare their
extra-vascular nature by remaining unaltered when subjected to
pressure. The color of these spots changes, as in ordinary ecchymosis,
in consequence of the metamorphoses of the hæmatin preparatory to its
final absorption, from crimson to purple, to blue, to green, to yellow,
and finally fades away. When recent, the spots appear sharply outlined,
with sometimes a faint encircling zone of hyperæmia, but as they become
older their margins grow indistinct. While the early lesions slowly
disappear, others continue to develop, and the affection may thus be
protracted for weeks. At times the petechiæ appear in crops, recurring
every few days, the patient at one time apparently nearly well, at
another time worse than ever. Finally, the symptoms definitely
disappear, to return no more, or they pass into those of other forms of
purpura. During the course of purpura simplex the blood-vessels of the
skin alone are affected, the deeper tissues and mucous membranes
probably remaining unchanged.

Throughout the attack the general health may--usually does--remain
good. As an occasional symptom there will be observed a few vesicles or
blebs, containing blood, upon the skin. The extent of the general
eruption may vary from a few scattered petechiæ to a copious and
startling {188} number of purpuric spots. The maintenance of the
upright position tends to perpetuate the evolution of the lesions.

In elderly persons purpura simplex is sometimes observed, and has been
described by many writers as purpura senilis. Hillier, following
Bateman, describes it as occurring in old women "upon the outside of
the forearms in successive dark, purple blotches of an irregular form
and various magnitude."[1] Aged men as well as women are liable to the
affection, which may quite as well appear upon the lower extremities of
either sex. It is altogether likely, however, that in such cases
degenerations of the vascular walls alone may cause the extravasations.

[Footnote 1: Reynolds's _System of Medicine_, vol. i. p. 792.]

PURPURA HÆMORRHAGICA (MORBUS MACULOSUS WERLHOFII).--In this form of
purpura there are added to the symptoms of purpura simplex hemorrhages
into and from the various mucous tracts, the nasal, faucial,
pharyngeal, gastric, intestinal, renal, uterine, rarely the pulmonary
mucous membranes, and exceptionally into the various serous membranes
and cavities. It may begin abruptly, in the midst of apparently
vigorous health, or after premonitory symptoms extending over several
days, vague sensations of discomfort--headache, pains, anorexia,
indisposition to exertion, and the like--or it may occur as a
transition from other forms of purpura. Usually there is no fever.

The hemorrhagic spots upon the skin appear much as in purpura simplex,
though the lesions are larger, acquiring the size of coins or even of
the palm of the hand. Spots soon appear upon the visible mucous
membranes, and free hemorrhages occur; indeed, the latter may be the
first symptom observed. Epistaxis is of most common occurrence, but
bleeding from the mouth, stomach, and intestines almost as frequently
results. The gums are almost constantly affected, and upon inspection
these may be found covered with blackish scabs, upon removal of which
the mucous membrane will be found pale and not swollen--an important
point in diagnosticating this affection from scurvy. Vesicles and blebs
filled with blood form both on the skin and mucous membranes. They
quickly rupture and discharge their contents. Bleeding from the stomach
and intestines is revealed--in the former case by the vomiting of a
brownish material resembling coffee-grounds; in the latter case by the
passage of black, tar-like evacuations. Pulmonary hemorrhage is to be
distinguished from hæmatemesis by the frothy and arterial character of
the blood. Hæmaturia may proceed from any part of the urinary tract.
Bleeding from several parts may occur at the same time, and may be very
copious.

In the mucous membranes extravasations of greater or less extent may
occur, as in the derma. Into the serous membranes they may take place
with or without effusion into serous cavities. It is only, however, in
cases that will almost certainly end fatally that the effusions into
these cavities are encountered. Hemorrhages into the substance of the
lungs, into the brain and other viscera, as well as into the tissues
generally, are occasionally observed.

At the outset of these bleedings the general health of the patient may
appear unimpaired, and if they be few in number and moderate in extent
but slight evidences of debility may be shown throughout the attack;
but it is often the case that the loss of blood is excessive and long
continued, and symptoms of profound anæmia supervene. The {189} patient
becomes greatly exhausted; intense pallor is developed, shortly
followed by general oedema. Attacks of syncope appear, and in fatal
cases--which are not common--death results from asthenia. This result
may occur after a few days from the profuseness of the hemorrhage;
usually, however, only after several weeks. Throughout the attack the
cutaneous lesions continue to develop, either irregularly or in
successive outbreaks, scattered over the general surface, involving the
face less frequently than other parts. These spots undergo the
color-changes peculiar to extravasated blood, and may be seen in all
the stages of involution in the same patient. Fever, usually absent
throughout the attack, may appear at the height of the affection, but
does not run high. Local inflammations are exceedingly rare. In
favorable cases recovery follows the gradual mitigation and
disappearance of the symptoms, but relapses frequently occur, and
convalescence may be retarded for months.

PURPURA RHEUMATICA (PELIOSIS RHEUMATICA).--Schoenlein in 1829 described
as peliosis rheumatica an affection in which the symptoms of purpura
simplex were associated with pain and often with effusion into the
joints, especially those of the knee and ankle. He considered it as an
independent malady. This opinion has been shared by Fuchs, Hebra,
Kaposi, Neumann, and many others. Kaposi[2] regards it as related to
erythema nodosum, with which affection, indeed, it possesses some
features in common. It probably, however, constitutes a complication of
ordinary purpura. That it is not primarily rheumatic is shown by the
almost invariable absence of many of the symptoms characteristic of
rheumatism; that it cannot be an independent affection appears from its
intimate relations with other forms of purpura.

[Footnote 2: _Hautkrankheiten_, 1880, p. 277.]

Purpura rheumatica commonly begins with malaise, anorexia, debility,
sometimes with mild fever. The patient is soon attacked with pains, of
a more or less acute character, in the joints, especially the knees and
ankles. There may be some effusion into the joint and cutaneous oedema.
After a few days the nature of the complaint will be revealed by an
eruption of petechiæ, first near the painful joints, but soon
extending, involving in many cases even the head and trunk. The
eruption may be at first slightly elevated and surrounded by a fine
halo of hyperæmic injection.

The pains usually subside upon the appearance of the eruption, and the
malady may be completed after a single outbreak. More commonly new
joint-pains are experienced, fresh crops of petechiæ appear, and the
trouble may be prolonged for weeks, even months, the patient meanwhile
suffering not very greatly in general health. The lesions may be
cutaneous only; rarely bleeding from mucous surfaces will occur
(Scheby-Buch). Albuminuria may be present (Kaposi). An annual type is
said by Kaposi, Neumann, and others to be sometimes observed, the
spring and autumn being the usual seasons for the outbreaks. This is
supposed to indicate a relationship with erythema nodosum and
multiforme. Cardiac murmurs have been detected in the course of purpura
rheumatica,[3] but these were probably anæmic or antedated the purpuric
symptoms. Purpura rheumatica never seems to result in endo- or
pericarditis.

[Footnote 3: Kinnicutt, _Archives of Dermatology_, i. p. 193; Mollière,
_Ann. de Dermatol._, v. p. 44.]

SUB-VARIETIES.--Henoch[4] and Couty[5] have described a form of {190}
purpura mostly observed in children, in whom rheumatoid pains occur
along with colic and vomiting of greenish or bilious matter, tenesmus,
and sometimes with loss of blood from the bowels. The disease may be
protracted throughout months by relapses. Cutaneous oedema frequently
occurs. Couty regards it as a form whose peculiarities justify its
assignment to a position of its own. The cause of the associated train
of symptoms is supposed (Couty) to reside in the sympathetic system,
and the name purpura nervosa is proposed for it. So many features of
ordinary purpura are manifested in these cases that it seems better to
consider them as examples of ordinary purpura complicated with
gastro-intestinal derangement. It has been suggested that the nausea,
vomiting, and abdominal pains may result from extravasation of blood
into the peritoneal tissue.[6]

[Footnote 4: _Berl. Klin. Wochenschr._, 51, 1874.]

[Footnote 5: _Gaz. Hebd._, 36 _et seq._, 1876.]

[Footnote 6: Immermann, _Ziemssen's Cyclopæd._, vol. xvii. p. 265.]

In the course of purpura there is frequently observed, more especially
in purpura simplex, a wheal-like arrangement of the eruption--such,
indeed, as occurs in urticaria. The term purpura urticans has been
given to this sub-variety, which may or may not be accompanied by
itching. Scheby-Buch has suggested that the urticaria may, with more
propriety, be attributed to the gastric disturbances that so often
accompany the forms of purpura presenting it.[7] The wheals are usually
seen upon the lower extremities, but may appear elsewhere. A
considerable degree of oedema may be present, particularly in lax
tissue, such as that of the scrotum, eyelids, etc.

[Footnote 7: _Deutsche Arch. f. Klin. Med._, B. xiv. p. 490.]

Purpura papulosa (lichen lividus, Willan) is a form of purpura where,
in the midst of ecchymoses, livid papules appear. These probably depend
upon a large amount of hemorrhage occurring within a limited space,
most often surrounding the orifices of hair-follicles, because these
are supplied with a capillary network that comes directly from the
deeper layer.[8] They are formed most abundantly on the legs of
scrofulous, cachectic persons who have purpura. Care must be taken to
distinguish this form of purpura from erythema multiforme and erythema
nodosum, where blood is usually extravasated secondarily into the
tissues. Those cases only where the purpura is primary should be
recognized as purpura papulosa.

[Footnote 8: Hebra, _Skin Diseases_, New Syd. Soc. Transact., ii. p.
425.]

The purpuric effusion appears to act as an irritant upon the tissues,
and to excite inflammation. Gangrene of the mucous coat of the
intestines has resulted from extensive hemorrhagic extravasations, and
from a similar cause cutaneous gangrene has been known. These
complications, however, are rare.

ETIOLOGY.--The immediate causes of purpura are quite unknown. Both
sexes and persons of every age are affected by it. While it is most
often seen in debilitated subjects, those in vigorous health possess no
immunity. It has often been observed during convalescence from other
maladies. It cannot be said that those who are miserably clothed, fed,
and lodged are especially predisposed to attacks of purpura. Between
purpura and hæmophilia, etiologically, there are many points of
difference. Purpura is not hereditary, nor is there a purpuric
diathesis in the strict sense of the term. Some persons, indeed, seem
to possess a {191} predisposition to the disease, and some authors
claim for purpura rheumatica a distinct annual type. This, however, is
not at all certain.

Recently it has been claimed that purpura hæmorrhagica depends upon the
presence of a minute organism in the blood. Petrone[9] injected blood
drawn from patients with this disease under the skin of rabbits,
producing widely-distributed hemorrhages. In the blood of these
individuals and of the injected rabbits micrococci and bacilli were
detected. Watson Cheyne[10] also describes a plugging of the
capillaries with bacilli. These were 1/7700 of an inch in length and
1/20000 of an inch in diameter, and were arranged in colonies. In
another case there were found micrococci arranged in chains. These
swarmed in the capillaries and some larger vessels, and sometimes
completely blocked them. Although an origin in infection has thus been
claimed for purpura hæmorrhagica, the fact that more than one variety
of micro-organism was observed cannot fail to excite suspicion of,
possibly, erroneous observation.

[Footnote 9: _Lo Sperimentale_, 51, 1883.]

[Footnote 10: _Lancet_, i., 1884, 344.]

PATHOLOGY.--In the foregoing description those extravasations of blood
due to simple mechanical violence, as from flea-bite, and sudden
increase of blood-pressure, as in the effort of coughing in whooping
cough, also from the deleterious influence exerted upon the
blood-vessels and blood by certain drugs, the specific fevers, Bright's
disease, and the like, have been excluded. Only those have been
considered where the effusion of blood seemed to occur spontaneously,
and the symptoms to result from some peculiar but not understood morbid
process. The hemorrhage is but a symptom; the process by which it is
brought about depends upon some change in the blood or blood-vessels.
We do not know what these subtle changes are. The blood of purpuric
patients has been carefully examined, but, with the exception above
mentioned, no definite changes have been discovered. Immermann[11]
found during the first stage of the disease the blood-corpuscles
perfectly normal in appearance, the white corpuscles subsequently
slightly exceeding the red in number--a simple result of copious
hemorrhage. No stated chemical changes in the blood are known in
purpura, nor is it known how the blood escapes from the vessels. It
undoubtedly escapes through alterations in the vascular wall, but it is
also true that red blood-corpuscles, as well as the pale ones, may find
their way in considerable numbers through the unruptured wall of the
vessels, per diapedesin, as was first suggested by Velpeau, but
definitely determined by Stricker. The causes of this migration are
obscure. Immermann[12] asserts that a fatty degeneration of the
vascular tissues and of the muscles takes place. This, however, is
manifestly a result of the loss of blood, and not its cause. Dr. Wilson
Fox[13] found extensive albuminoid disease of the muscles and
capillaries of the skin; but the albuminoid degeneration involved
several organs of a patient with syphilis, and the purpura was
certainly secondary to the morbid conditions. Rigal and Cornil[14]
think that the hemorrhages are a result either of sympathetic
irritation or of diminished action of the vaso-motor centre. It is
indeed altogether likely that the cause will ultimately be found to
reside in the vaso-motor system.

[Footnote 11: _Ziemssen's Cyclop._, xvii. p. 258.]

[Footnote 12: _Loc. cit._]

[Footnote 13: _Brit. and Foreign Med.-Chir. Review_, Oct., 1865.]

[Footnote 14: _L'Union Méd._, 5, 6, 7, 1880.]

{192} DIAGNOSIS.--The affection bearing the closest resemblance to
spontaneous purpura is scurvy; indeed, its supposed relationship to
this disease has given purpura one of its synonyms, land scurvy. The
two affections, however, are probably without the slightest
relationship. They possess in common the hemorrhagic symptoms, both in
the tissues and from free surfaces, but the resemblance does not extend
much beyond this. Scurvy depends upon deprivation of fresh vegetable
food and the use of unsuitable and insufficient food generally, and
upon bad hygienic surroundings. Purpura may--frequently does--appear in
broken-down constitutions, but it equally attacks the strong and
vigorous, while the character of food exerts no special influence on
its production. Scurvy only follows long-continued privations and as a
culmination of a train of distressing symptoms. Purpura appears in the
midst of health, or after brief premonition, or during convalescence
from totally unrelated diseases. In scurvy there is a decided tendency
toward ulceration, which is absent in purpura. In scurvy the mouth and
gums inflame and ulcerate, the latter becoming swollen, spongy, and of
a bluish-red color. In purpura, ulceration of the buccal mucous
membrane does not occur, and the gums are pale and intact. The curative
influence of fresh vegetables, lime-juice, etc. in the treatment of
scurvy is not observed in purpura. It has been claimed that purpura is
but a mild degree of scurvy: this cannot be so, for we may have a mild
scurvy or a severe, even fatal, purpura.

The hemorrhagic diathesis, or hæmophilia, presents points of analogy
with purpura. Here, however, is found the almost constant history of
heredity and the implication only of persons of the male sex. The
disposition to bleed at all times upon the receipt of the smallest
injury is quite unlike the suddenly-developed and transitory
hemorrhages of purpura, which are also more generally distributed.

With the secondary hemorrhagic effusions and ecchymoses that occur in
conditions of profound alterations of the blood and blood-vessels in
cases of malignant small-pox, scarlatina, typhus fever, etc., and in
some cases of poisoning, as from phosphorus, spontaneous purpura
presents identities, but the history of the complaint and the condition
of the patient will prevent error. A knowledge of the circumstances
will serve to distinguish purpura simplex from the petechiæ and small
ecchymoses produced by fleas, by diminished atmospheric pressure, by
coughing, in the course of Bright's disease, etc.

Purpura rheumatica presents, as has been shown, many points of
resemblance to erythema multiforme and erythema nodosum. The mild
fever, the joint-pains, the extravasations of the latter affections,
are much like the symptoms of this form of purpura. The nodular,
inflamed, tender condition of the lesions, their location--frequently
upon the extensor surfaces of the extremities--their course and
duration, usually serve to identify erythema nodosum, while with
erythema multiforme it is usually not difficult to observe its
essentially inflammatory character. Scheby-Buch has shown the
difficulties often opposed to the differentiation of purpuric lesions
and ecchymoses due to violence.[15] Where the petechial eruption of
purpura simplex is well marked, where the internal hemorrhages of
purpura hæmorrhagica are copious, the inquiries of the observer will
usually lead him to correct conclusions. Where the {193} ecchymoses are
larger and upon exposed parts of the body, the diagnosis from the
lesions alone becomes impossible, and due consideration of all
concomitant circumstances is essential. It should be remembered that in
purpura very slight violence may call forth extensive ecchymosis. This
circumstance has important medico-legal bearings.

[Footnote 15: _Viertelj. f. Dermatol. und Syph._, 1879, p. 99.]

PROGNOSIS.--Purpura usually terminates favorably. Its course runs from
two to six weeks, rarely longer. Relapses and remissions are frequent.
Purpura simplex is of very little gravity, and need excite little
apprehension. Purpura rheumatica almost always ends in recovery; fatal
terminations, however, have been known. Purpura hæmorrhagica is of much
more serious import. Even here, however, though the patient may fall
into profound debility from loss of blood, recovery is the rule, the
symptoms gradually diminishing in severity until health becomes
re-established. In fatal cases death ensues after prolonged and profuse
losses of blood. Purpura may subside after a single outbreak or many
relapses, and recrudescences may occur extending through months. Anæmia
may persist long after the disappearance of purpuric symptoms. A
tendency to purpura may be shown at irregular intervals for years, and
even throughout life.

TREATMENT.--Very mild cases of purpura simplex require no treatment,
not even confinement within doors. The patient is often first made
aware of his disease by accident; doubtless it frequently escapes
detection altogether. It has been observed that purpura often appears
upon the lower limbs of convalescents from other diseases when they
first essay the upright position. Relapses of purpura also frequently
appear as the patient leaves his bed. We have here an important
indication for treatment--viz. the maintenance of the recumbent posture
in cases of any degree of severity. Fresh vegetables and vegetable
acids do not have the same happy influence as in scurvy. It is
manifestly important that appropriate food should be administered in
sufficient quantity, both to improve the general health and to repair
the exhausting losses of blood. Milk is an exceedingly valuable article
of diet in these cases, being but little apt to irritate the mucous
membrane of the alimentary canal.

The patient should be guarded against violence. Injuries that may be of
no consequence to healthy persons may excite in the purpuric profuse
hemorrhage, free or interstitial. Violent emotions and physical efforts
should be avoided, as in stimulating the heart's action a condition of
increased blood-pressure ensues that may readily result in
extravasation.

There are no remedies that exert a specific influence over purpura, and
yet quite a number have enjoyed, and still enjoy, high reputation in
controlling the symptoms. Probably the most frequently employed remedy
against purpura is sulphuric acid, preferably the aromatic sulphuric
acid, in doses of from 15 to 20 drops, diluted well with water and
administered every third or fourth hour. It is certainly an agent of
value, though some authors maintain that it has no efficacy
(Immermann). Acetate of lead undoubtedly exercises an influence over
the course of the disease. More recently, ergot has been employed. Its
use has been highly extolled by Buckley and others. Very large doses
may be given. The hypodermic use of ergotin has been followed by
results most gratifying to those employing it. Oil of turpentine has
enjoyed considerable reputation. A remedy that undoubtedly has a good
effect is iron, both as {194} exercising a controlling action over the
bleeding and as assisting to repair the resulting anæmia. The tincture
of the chloride is the most suitable preparation, and may be given in
large doses (from minim xx to fluidrachm ss), well diluted, every
fourth hour. Care must be exercised to avoid irritating the digestive
organs with it. Formerly, venesection was employed to prevent the
occurrence of hemorrhage, but its efficacy in this direction is at
least doubtful, and cannot but help to intensify the disastrous
consequences of severe and protracted attacks.

The various complications that may arise, as well as the general
results of purpura, must be treated symptomatically. For the mucous
membranes astringent washes should be used, and in favorable situations
the tampon may sometimes be employed with profit. In purpura rheumatica
the arthritic pains will be alleviated by anodyne liniments and
plasters, and the often accompanying abdominal pains and colic by
anodynes internally administered. Hæmatemesis, hæmaturia, etc. must be
treated upon general principles. The results of profuse hemorrhage must
be combated with stimulants. Transfusion of blood has been proposed and
practised for the extreme anæmia that sometimes occurs, but without
encouraging results. If necessary, the bowels may be kept free by mild
aperients. In severe cases rest in bed should be rigidly enforced until
after the establishment of convalescence. Quinia, iron, and nux vomica
are indicated above all other remedies for the anæmia resulting from an
attack of purpura.



{195}

DIABETES MELLITUS.

BY JAMES TYSON, A.M., M.D.


Diabetes mellitus is a term applied to a group of symptoms more or less
complex, of which the most conspicuous is an increased flow of
saccharine urine--whence the symptomatic title. It is associated with a
derangement of the sugar-assimilating office of the liver, as the
result of which an abnormally large quantity of glucose is passed into
the hepatic vein and thence into the systemic blood, from which it is
secreted by the kidneys. The condition is sometimes associated with
alterations in the nervous system, at others with changes in the liver
or pancreas, while at others, still, it is impossible to discover any
structural alterations accompanying it.

[Illustration: FIG. 1. To show the position of the punctures required
to produce glycosuria, the lobes of the cerebellum are separated. Below
are seen the restiform bodies, the divergence of which circumscribes
the apex of the calamus scriptorius and the fourth ventricle. The
puncture _p'_ produces glycosuria; the puncture _p_, glycosuria with
polyuria; and a puncture a little higher up than _p_, albuminuria.]

PATHOLOGY AND PATHOGENESIS.--Notwithstanding that this disease has been
recognized for two centuries and a half, that abundant opportunity has
been furnished for its post-mortem investigation, and that experimental
physiology has contributed much information bearing upon the subject,
its pathology is still undetermined. Experiment has, however, rendered
it very likely that all cases of essential glycosuria--that is, all
cases in which saccharine urine is not the direct result of
over-ingestion of sugar or sugar-producing food--are accompanied by a
hyperæmia of the liver. This hyperæmia, with its consequent glycosuria,
can be induced by puncturing or irritating the so-called diabetic
area[1] in the medulla oblongata. This area corresponds with the
vaso-motor centre, and with the roots of the pneumogastric or vagus
nerve in the floor of the fourth ventricle; whence it was at first
inferred that this nerve is the excitor nerve of glycosuria. It was
soon ascertained, however, that when the pneumogastric was cut,
glycosuria ensued only when the central end was stimulated, while {196}
stimulation of the peripheral portion was without effect. Whence it
became evident that this nerve is not the excitor, but the sensory
nerve concerned in glycogenesis.

[Footnote 1: The diabetic area, as marked out by Eckhard, and which
corresponds with the vaso-motor area, as defined by Owsjannikow
(_Ludwig's Arbeiten_, 1871, p. 21), is bounded by a line drawn four or
five mm. above the nib of the calamus scriptorius, and another about
four mm. higher up.]

It was also learned in the course of continued experiment that
glycosuria resulted upon transverse section of the medulla oblongata,
of the spinal cord above the second dorsal vertebra, of the filaments
of the sympathetic accompanying the vertebral artery, upon destruction
or extirpation of the superior cervical ganglion, and sometimes, but
not always, after division of the sympathetic in the chest (Pavy); also
after section or careful extirpation of the last cervical ganglion,
section of the two nerve-filaments passing from the lower cervical to
the upper thoracic ganglion around the subclavian artery, forming thus
the annulus of Vieussens,[2] and after section or removal of the upper
thoracic ganglion.

[Footnote 2: Cyon and Aladoff, reprint from the _Mélanges biolgiques_
and _Bullétin de l'Académie Impériale de Petersbourg_, vol. xiii. p.
91; cited by Dr. Brunton in the Lectures named in note on p. 198; also
_British Medical Journal_, Dec. 23, 1871, p. 731.]

[Illustration: FIG. 2. The last cervical and first thoracic ganglia,
with circle of Vieussens, in the rabbit, left side. (Somewhat
diagrammatic, many of the various branches being omitted.)

_Trach._, trachea; _Ca._, carotid artery; _n. vag._, the vagus trunk;
_n. rec._, the recurrent laryngeal; _sym._, the cervical sympathetic
nerve ending in the inferior cervical ganglia, _gl. cerv. inf._ Two
roots of the ganglion are shown--_rad._, the lower of the two
accompanying the vertebral artery, _A. vert._, and being the one
generally possessing accelerator properties; _gl. thor. pr._, the first
thoracic ganglion. Its two branches, communicating with the cervical
ganglion, surround the subclavian artery, forming the annulus of
Vieussens. _sym. thor._, the thoracic sympathetic chain; _n. dep._,
depressor nerve. This is joined in its course by a branch from the
lower cervical ganglion, there being a small ganglion at their
junction, from which proceed nerves to form a plexus over the arch of
the aorta. It is this branch from the lower cervical ganglion which
possesses accelerator properties, hence the course of the accelerator
fibre is indicated in the figure by the arrows. (Modified from Foster's
_Physiology_.)]

All these operations paralyze the vaso-motor nerves by which, in
health, the blood-vessels of the liver are kept in a state of tonic
contraction; hence these vessels dilate when the nerves are cut. From
the facts named we also learn the path of the glycogenic influence,
which must be from the medulla oblongata into the spinal cord, thence
by the filaments of the {197} sympathetic which accompany the vertebral
artery into the lower cervical ganglion; thence through the annulus of
Vieussens into the first dorsal ganglion; and thence through the
prevertebral cord of the sympathetic, and branches not precisely
determined, to the hepatic blood-vessels as shown by the dotted line in
Fig. 3.

[Illustration: FIG. 3. Diagram showing the course of the vaso-motor
nerves of the liver, according to Cyon and Aladoff. These nerves are
indicated by the dotted line which accompanies them: _a_, vaso-motor
centre; _b_, trunk of the vagus; _c_, passage of the hepatic vaso-motor
nerves from the cord along the vertebral artery; _d_, fibres going on
each side of the subclavian artery and forming the annulus of
Vieussens; _e_, first dorsal ganglion; _f_, ganglionated cord of the
sympathetic; _g_, the spinal cord; _h_, the splanchnic nerves; _i_,
coeliac ganglion, from which vaso-motor nerves pass to the hepatic and
intestinal vessels; _k_, the lungs, to which fibres of the vagus are
seen distributed; _l_, the liver; _m_, the intestine; _n_, the arch of
the aorta.]

I say, by branches of the sympathetic not precisely determined, because
our power to produce artificial diabetes fails below the first thoracic
ganglion; for section of the sympathetic between the tenth and twelfth
ribs, and of the splanchnics, is not followed by glycosuria, although
the vaso-motor nerves to the liver are known to pass through them.

According to Eckhard,[3] the phenomena of artificial glycosuria are
irritative and not paralytic. This view he believes sustained by his
own experiments, according to which if the splanchnics, through which
{198} the vaso-motor nerves of the liver pass, are cut prior to the
diabetic puncture, not only does this operation fail to produce
glycosuria, but it even renders ineffectual the puncture itself as well
as the section higher up. But Cyon and Aladoff remind us that it is not
mere dilatation of the hepatic vessels, but increased velocity in the
movement of the blood, which deranges the sugar-assimilating function
and causes glucose to appear in the urine. The vaso-motor nerves of the
intestinal blood-vessels also pass through the lower part of the
sympathetic and the splanchnics, and section of the latter must cause
these blood-vessels to dilate. Now, in rabbits, in which this
experiment is usually performed, the digestive canal is very long, and
the blood-vessels so capacious that when dilated they hold as much
blood as all the rest of the vascular system together, so that when the
lower sympathetic and splanchnics are cut, so much blood goes into the
intestines that the increased velocity required in the blood-vessels of
the liver to produce glycosuria is impossible. But if the vessels of
the liver be first dilated by puncturing the floor of the fourth
ventricle, section of the sympathetic or of the splanchnics may then be
made without arresting the formation of sugar; whence it would appear
that the glycogenic influence may still pass through the lower
sympathetic and splanchnics.

[Footnote 3: _Beiträge zur Anat. und Physiologie_, iv., 1859, p. 1;
vii., 1873.]

In view of the fact that Eckhard[4] has failed to confirm the results
of Cyon and Aladoff, but has traced the glycogenic influence down the
spinal cord as far as the fourth dorsal vertebra in rabbits, and even a
little lower, and that Schiff[5] has shown that diabetes sometimes
results after section of the anterior columns of the cord between the
medulla and the fourth cervical vertebra, Dr. Brunton[6] suggests that
the vaso-motor nerves of the liver may not always leave the spinal cord
to join the sympathetic by the branches accompanying the vertebral
artery, but sometimes pass farther down the cord, leaving it by the
communicating branches to some of the dorsal ganglia, as indicated in
Fig. 4.

[Footnote 4: _Beiträge zur Anat. u. Physiologie_, viii., 1877, p. 79.]

[Footnote 5: _Untersuchungen über Zuckerbildung in der Leber_, 1859, S.
108.]

[Footnote 6: _Lectures on the Pathology and Treatment of Diabetes
Mellitus_; reprinted from the _British Medical Journal_, 1874, p. 12.]

[Illustration: FIG. 4. Diagram showing another course which the
vaso-motor nerves of the liver may take. The letters indicate the same
parts as in Fig. 3. The hepatic vaso-motor nerves are here represented
as passing lower down the cord than in Fig. 3, and leaving it by
communicating branches to the second dorsal ganglion. It is possible
that they may sometimes leave by the branches to the first, and
sometimes by those going to a lower, ganglion. In such cases any
irritation to the third or one of the other cervical ganglia may cause
diabetes by being conveyed along the vertebral artery and up the cord,
as indicated by the dark line, to the vaso-motor centre, where it may
cause reflex inhibition in the same way as any irritation to the
vagus.]

It is evident that an agency involving any part of this tract in such a
way as to paralyze the vaso-motor nerves of the liver is capable of
producing glycosuria. Such cause may operate upon the central ganglia
whence the nerves emanate, as the vicinity of the oblongata and upper
parts of the spinal cord or the coeliac ganglion and its branches,
including those to the pancreas. Or the irritation may be peripheral
and its effects reflex. We have seen that irritation of the central end
of the cut vagus will produce glycosuria. Any irritation, therefore,
involving the peripheral distribution of this nerve may produce it.
Hence embarrassed respiration, whether due to disease of the
respiratory passages, strangulation, or inhalation of irrespirable
gases and anæsthetics, produces glycosuria in dogs and rabbits; and
this symptom has been known to attend these conditions in the human
subject. So, too, glycosuria may be produced by such substances as
woorara, strychnia, morphia, and phosphoric acid, introduced into the
blood and irritating the terminal filaments of the pneumogastrics, or
it may be brought about secondarily through the embarrassed respiration
these drugs produce. Such peripheral {199} irritation may reside also
in the stomach, intestines, liver, or any organ to which the
pneumogastric is distributed.

It is not unlikely that irritation of the extremities of sensory nerves
other than the pneumogastric may become the cause of reflex glycosuria.
Even puncture of the floor of the fourth ventricle itself may be reflex
in its operation, the roots of the pneumogastric being thus irritated.
The effect of the irritation conveyed to the glycogenic centre is to
inhibit the usual tonic influence of the vaso-motor nerve upon the
vessel walls. Among the experimental irritations, in addition to
puncture of the floor of the fourth ventricle, which produce glycosuria
by reflex action, are injuries of the cerebral lobes and cerebellum,
optic thalami, cerebral peduncles, pons varolii, middle cerebellar
peduncles, and even of the sciatic nerve and brachial plexus; whence it
may be inferred that pathological irritation in the same situations may
result in a glycosuria, which is temporary or permanent according as
the irritation is temporary or permanent.

Finally, there is no reason why an inhibitory reflex action should not
originate in the sympathetic itself. When we remember that this nerve
is both sensory and motor in function, and that the inhibitory
influence to which the heart's action is subject is accomplished
through the sympathetic as a sensory nerve and the pneumogastric as a
motor, there is no reason why similar results may not be brought about
by the sympathetic alone. This being the case, we need not ascribe
glycogenic phenomena to irritation in Eckhard's sense--that is, to a
direct stimulant action of the irritant upon the vaso-motor nerves of
the liver--but may suppose a sensory influence to ascend one set of
sympathetic filaments and an inhibitory influence to descend through
another.

Dr. Pavy has recently put forward some chemical theories which explain
the action of the hyperæmia in producing glycosuria, but they do not
account for the hyperæmia itself. In healthy digestion the
carbohydrates (starch and sugar) are converted, not into glucose, but
into maltose, C_{12}H_{22}O_{11}, dextrin being intermediate in
composition. Maltose is absorbed and assimilated, converted into
glycogen. So, too, when glucose is ingested as such, it is converted by
the glucose ferment into maltose in the stomach and intestines. For the
proper production of maltose and its assimilation a good venous blood,
producing a maltose-forming ferment, is necessary. In diabetes, in
consequence of the dilatation of the arteries of the chylopoëtic
viscera, the blood enters the liver too little deoxygenated, and a
glucose-forming ferment is produced. The glucose thus formed is not
assimilable, but passes off into the circulation and the urine.

MORBID ANATOMY.--Such are some of the facts bearing upon the pathology
of diabetes mellitus. Throwing out the milder type of cases, in which
glycosuria is the result of an over-ingestion of saccharine and
sugar-producing food--and these can scarcely be called instances of
essential diabetes--it is evident that glycosuria may be produced in a
variety of ways operating through the nervous system; and accordingly
we may infer that there is scarcely an organ in close relation with the
sympathetic system derangement of which is not capable of producing it.
Among these we would naturally expect to find conspicuous alterations
in the nervous centres, and yet I have never found changes in these
centres after death. At the same time, others have noted meningitis,
tubercular {200} and traumatic, apoplectic effusions, and tumors of the
brain, especially in the neighborhood of the medulla oblongata. The
alterations in the nerve-centres described by Dickinson as the
essential morbid anatomy of diabetes I have looked for in vain. These
changes are described as a cribriform or porous condition of the white
nervous matter, said to be visible to the naked eye. The spaces thus
produced are partially occupied by dilated blood-vessels, which, in
turn, are surrounded by dilated perivascular sheaths and broken-down
nervous matter, into which extravasations of blood have taken place, as
evidenced by the presence of pigment-granules. The changes are found in
the white matter of the convolutions of the brain, but fewer and larger
in the central portions. The corpora striata, optic thalami, pons,
medulla, and cerebellum are favorite seats for the largest and most
striking holes. In rapidly-fatal cases the cavities are sometimes
filled with a translucent, gelatinous substance, containing, besides
vascular elements, the globular products of nervous disintegration. In
the more chronic forms of the disease, as it occurs in elderly persons,
the excavations are usually empty, although the elements of nervous
decay are still to be found fringing the margins or collected as an
irregular sheath upon the dilated or shrunken artery. There are changes
in the cord similar to those in the brain, but less decided. But the
most striking alteration in the cord, according to Dickinson, although
not always present, is dilatation of the central canal, which in the
dorsal and lumbar regions is sometimes expanded to many times its
normal diameter, and forms a conspicuous object immediately after the
cord is divided.

These alterations have eluded the vigilance of other pathologists who
have sought for them in well-determined cases of diabetes mellitus,
while they have been found, on the other hand, in the nervous centres
when no diabetes was present. In the recent discussion on diabetes at
the Pathological Society of London, Douglas Powell[7] seemed to be the
only one who was convinced that most of Dickinson's specimens were
examples of positive lesions.

[Footnote 7: _London Lancet_, May 5, 1883, p. 776.]

A hyaloid thickening of the blood-vessels of the brain has been noted
by Stephen Mackenzie[8] and Seymour Taylor[9] in some cases, and
miliary aneurisms of the retina in one.

[Footnote 8: Discussion on Diabetes, Path. Soc. of London, _London
Lancet_, April 7, 1883, p. 593.]

[Footnote 9: Ibid., _Lancet_, May 5, 1883, p. 774.]

Of other organs, one of the most frequently found diseased is the
pancreas, and, according to Senator, it is fair to assume that disease
of the pancreas is present in about one-half of all cases of diabetes.
As the result of increased experience, I am inclined to attach much
more importance to pancreatic disease as a cause of diabetes than I did
a few years ago. Among the changes found is a pseudo-hypertrophy, which
consists chiefly in a hyperplasia of the connective tissue, fatty
degeneration of the gland-cells, and atrophy of the glandular
structure; cancerous disease; calculous concretions in the ducts with
or without obstruction; and cystic dilatation.

Facts bearing upon the relation of pancreatic disease to diabetes have
been accumulating since Cowley first discovered calculi in the pancreas
of a diabetic, and Bright pancreatic cancer in a similar case. Since
then {201} instances have multiplied to such extent that it would be
unprofitable to enumerate them. But in 1877, Lancereaux[10]
communicated to the French Academy of Medicine specimens of profound
lesion of the pancreas from cases dying of diabetes mellitus. This, he
alleged, constitutes a special and distinctive variety of diabetes,
characterized by sudden onset, emaciation, polydipsia, polyphagia, and
peculiar alvine dejections. More recently, Depierre[11] has confirmed
these observations, apparently establishing this variety of diabetes
mellitus, of which a very rapid course--six months to three years--and
the habitual presence of diarrhoea are characteristic; while the
presence of greasy or creamy stools, and the appearance in them of
undigested nitrogenous substances, may aid in the diagnosis. Precisely
such a case, running the same rapid course--less than one year--with
emaciation, uncontrollable diarrhoea, creamy stools, jaundice, and
pancreatic disease, came under the writer's care in 1882. At the
autopsy the pancreas was found enlarged, and numerous gritty particles
were disseminated through it.

[Footnote 10: "Notes et réflexions à propos de deux cas de diabète
sucre avec altération du pancréas," _Bull. Acad. de Méd._, Paris, 1877,
2d Serie, vi. 1215-1240.]

[Footnote 11: _Med. News and Abstract_, vol. xxxix., June, 1881, p.
344, from _Jour. de Méd. et de Chir. pratiques_, Dec, 1880.]

Supposing such pancreatic disease to be primary, it is evident that it
must operate through the coeliac plexus, which, with its ganglion, is
gradually encroached upon. On the other hand, it is also possible that
the disease of the coeliac plexus may be primary, and the coexisting
pancreatic disease and diabetes mellitus both secondarily dependent
upon it. This can only be settled by more careful study of the coeliac
plexus after death from diabetes, but up to the present time facts
would seem to support the view of primary pancreatic disease.

The liver is frequently enlarged--sometimes but slightly, at others
decidedly. It has been known to reach three times the size of the
normal organ. Again, it may be darker and harder--hyperæmic. By minute
examination the acini are found enlarged, the capillaries dilated and
distended; the liver-cells are enlarged, distinctly nucleated, rounded,
and indistinct as to their outline, appearing to fuse into each other.
A weak solution of iodine strikes a wine-red color, which, according to
Rindfleisch, is confined to the nucleus, but, according to Senator, may
extend to the whole cell. This reaction Klebs ascribes to post-mortem
changes in the glycogenic substance. They are more striking in the
portal or peripheral zone of the lobule, while the intermediate or
hepatic artery zone is often fatty, and the central part, surrounded by
the rootlets of the hepatic vein, is nearly normal. Stockvis and
Frerichs ascribe the enlargement of the liver partially to a new
formation of liver-cells--in other words, to a true hypertrophy. At
other times the organ has been found reduced in size.

Dickinson, Trousseau, and Budd describe an overgrowth of connective
tissue, as well as of the cells of the liver, producing a hypertrophic
cirrhosis.

According to Beale, Frerichs, and Folwarczny, the fat which is found in
small proportion in the liver-cells in health is often diminished, and
even absent, and quantitative[12] analysis by the last-named observer
{202} confirms this view. Such diminution may be the forerunner of an
atrophy of liver-cells which has been noted, and which, as the disease
continues, leads to the atrophy referred to as occasionally present. On
the other hand, intense fatty degeneration of the entire organ, similar
to that found in phosphorus-poisoning, has been met by Gamgee,
associated with a lipæmic state of the blood and symptoms of acute
acetonæmia.

[Footnote 12: Folwarczny, "Leberanalysen bei Diabetes Mellitus,"
_Wiener Zeitschr._, N. F., 1859, ii. 6.]

The kidneys, in cases which have continued some time, are apt to be
hyperæmic and enlarged, although primarily they are uninvolved. It
would seem that the long-continued hyperæmia which is a necessary
condition of the copious secretion of urine, results, sooner or later,
in an over-nutrition of the renal epithelium, a widening of the
tubules, and consequent enlargement of the whole organ. The changes are
mainly of a parenchymatous or catarrhal rather than an interstitial
nature, the epithelium being disposed to shed. These changes may reach
a more advanced stage of cellular degeneration, and may be attended by
albuminuria. The cells may become very large, present a yellowish-brown
color, their nuclei indistinct and non-responsive to ordinary staining
solutions, but may take a red stain with a weak solution of iodine,
similar to that described in the case of the liver-cells. Mackenzie
describes a hyaline degeneration of the intima of the arterioles and a
skeleton condition of the epithelium of the collecting tubes.[13] There
may also be a catarrh of the pelves of the kidneys and ureters, due to
irritation of the saccharine urine.

[Footnote 13: _Loc. cit._]

Atrophy of the testes has been noted by Romberg and Seegen in young
men, and recently Hofmeier[14] has reported the case of a young
diabetic woman, aged twenty, who came under observation for pruritus
vulvæ, in whom the uterus was found small, scarcely 5 cm. (2 inches)
long, and the ovaries very much atrophied. As this young woman had no
other ailment, the atrophy was ascribed to the diabetes.

[Footnote 14: _Berliner klin. Wochenschr._, 1883, No. 42.]

Among the most constant secondary lesions is the aggregate of changes
known as those of pulmonary phthisis. But a few years ago, when our
ideas on this subject were more definite than they are to-day, and when
it was thought we had three distinct varieties of phthisis--the
tubercular, the catarrhal, and the fibroid--the phthisis of diabetes
was regarded as typically catarrhal.[15] At the present time, however,
when the tendency at least is to regard all phthisis as tubercular,
diabetic phthisis must be consigned to the same category. At the same
time, if the tubercle bacillus is to be regarded as the essential
criterion of tuberculosis, it must be stated that the diabetic patient
is subject to two different lung processes--at least if the
observations of Riegel of Giessen[16] are to be regarded as correct. In
two cases of diabetic phthisis studied at his clinic, the sputum of one
contained numerous bacilli, while the other, although the case
presented the most distinct signs of infiltration of the apex, and
although more than fifty preparations were investigated, revealed none.
The sputum was also said to present some unusual physical characters.
So far as I know, no autopsies of cases showing these clinical
differences have been reported, although there have been found in
diabetes, distinct from the usual cheesy foci, fibroid changes with
small smooth-walled cavities. In such cases {203} tubercle bacilli
would be absent, while the physical signs of consolidation would be
present.

[Footnote 15: See the writer's work on _Bright's Disease and Diabetes_,
Philada., 1881, p. 256.]

[Footnote 16: _Medical News_, Philada., May 19, 1883, from
_Centralblatt f. klin. Med._, Mar. 31, 1883.]

As a part of the phthisical process in diabetes, cavities of various
sizes are found and gangrene of the lungs has been observed.

ETIOLOGY.--The problem of the etiology of diabetes mellitus is as
unsatisfactorily solved as is that of its pathogenesis. Certainly, a
majority of cases of diabetes cannot be accounted for. A certain number
may be ascribed to nervous shock, emotion, or mental anxiety; a few to
overwork; some to injury and disease of the nervous system; others to
abuses in eating and drinking. Among the injuries said to have caused
diabetes are blows upon the skull and concussions communicated to the
brain, spinal cord, or vaso-motor centres through other parts of the
body. Hereditation is held responsible for a certain number of cases.
Malarial and continued fevers, gout, rheumatism, cold, and sexual
indulgence have all been charged with producing diabetes.

Diabetes mellitus is most common in adult life, although Dickinson
reports a case at six years which was fatal, Bence Jones a case aged
three and a half, and Roberts another three years old; and in the
reports of the Registrar-General of England for the years 1851-60 ten
deaths under the age of one and thirty-two under the age of three are
included. This statement, in view of the experience of the difficulties
of diagnosis in children so young, seems almost incredible. I have
never myself met a case in a child under twelve years. At this age I
have known two, of which one, a boy, passed from under my notice, while
the second, a girl, recovered completely. The disease is most common
between the ages of thirty and sixty. The oldest patient I have ever
had died of the disease at seventy-two years, having been under my
observation for three and a half years.

It is decidedly more frequent in men than in women, carefully prepared
statistics of deaths in Philadelphia during the eleven years from 1870
to 1880, inclusive, giving a total of 206 deaths, of which 124, or
three-fifths, were males, and 82, or two-fifths, females. This is the
experience of all.

My own experience has been singular and interesting. Up to April, 1881,
I had never met a case in a woman. Of 18 cases outside of hospital
practice which I have noted since that date, 9 were men and 9 women.
But I still do not recall an instance of a woman in hospital practice,
although I have constantly cases among men.

Not much that is accurate can be said of the geographical distribution
of the disease. It seems to be more common in England and Scotland than
in this country, at least if the statistics of New York and
Philadelphia are considered. In the former city, statistics extending
over three and a fourth years show that out of 1379 deaths, 1 was
caused by diabetes; in Philadelphia, in eleven years, 1 out of 875; in
England and Wales, according to Dickinson from observations extending
over ten years, 1 out of 632; and in Scotland, 1 out of 916. According
to the same authority, the disease is more prevalent in the
agricultural counties of England, and of these the cooler ones,
Norfolk, Suffolk, Berkshire, and Huntingdon. According to Senator, it
is more common in Normandy in France; rare, statistically, in Holland,
Russia, Brazil, and the West Indies, while it is common in India,
especially in Ceylon, and relatively very frequent in modern times in
Wurtemberg and Thuringia. Seegen says it is more {204} frequent among
Jews than among Christians, but I have never seen a case in a Hebrew.

SYMPTOMS, COURSE, AND DURATION.--The earliest symptom commonly noted by
the diabetic is a frequency of micturition and the passage of larger
amounts of urine than is natural. Coincident with or immediately
succeeding this is an undue thirst and dryness of the mouth, which soon
becomes the most annoying symptom the patient has, the freest draughts
of water giving but partial or temporary relief. To this succeeds
dryness, and sometimes itching, of the skin and absence of
perspiration. A good appetite with fair digestion accompanies this
stage of the disease, but notwithstanding this the patient loses in
weight. If a male, his attention is sometimes called to his urine by
the white spot left after the evaporation of a drop of urine on his
boot or clothing or by the stiffness of his linen due to the same
cause. To these symptoms are sometimes added an intolerable itching of
the end of the urethra in males and of the vulva in females, probably
due to the irritation caused by the saccharine urine in passing over
and drying upon these parts.

As the disease progresses muscular weakness supervenes. This, however,
comes on at varying periods after the incipient symptoms make their
appearance. Sexual inclination grows less. The muscular weakness
gradually increases, if the disease is not checked, until the patient
can barely walk: he totters in his gait, and reminds one of a case of
Duchenne's disease. Even before this he sometimes gives up and goes to
bed. Often harassing cough ensues, adding its exhausting effect to that
of the essential disease. Percussion and auscultation discover
consolidation at one apex or over larger areas of the lungs. Dyspepsia
and indigestion replace the good appetite which attended the onset of
the symptoms, and all efforts to increase the latter are unavailing.
The heart begins to flag, and its action is irregular. It finally
ceases to act, and the patient dies suddenly, sometimes unexpectedly.
Or coma may supervene before death. This coma, known as diabetic coma,
is generally ascribed to the accumulation of acetone or
acetone-producing substance in the blood. It is supposed to be a
product of the decomposition of the sugar in the blood, and the
phenomena resulting from its presence are known as those of acetonæmia.
Some further account of it will be given in the section on changes in
the urine. It is sometimes recognizable by a fruity odor of the breath,
which may even pervade the atmosphere of the room in which the patient
lies, and may be recognized on entering. It has been compared to the
odor of a room in which apples have been kept, again to sour beer, and
again to chloroform.

During all this time the thirst and discomfort arising therefrom,
continue, although it sometimes happens that toward the end the
quantity of urine and its contained sugar diminish and the urine
becomes darker in hue.

Such is the course of a typical case of diabetes mellitus. Other
symptoms, less conspicuous, are a lowered temperature of the body, from
1° to 2½° F. or even more; cataract, dilatation of the retinal vessels,
intraocular lipæmia, functional derangements of vision, including
amblyopia, presbyopia, and loss of accommodating power; and
occasionally total blindness from atrophy of the retina may be present.
I have known almost total blindness to appear very early in the
disease, and {205} subsequently to disappear. Derangements of the other
special senses, as impairment of hearing, roaring in the ears, and
disorders of smell and taste, also occur. Boils and carbuncles are
occasional symptoms; although usually late in occurrence, the former
are said to be sometimes the first symptoms recognized. Numerous skin
affections may occur. Ulcerated surfaces are slow to heal, and gangrene
supervenes sometimes spontaneously, but more often as the result of
some trifling injury. It may start from a blister produced by
cantharides, although such instances are scarcely frequent enough to
justify interference with treatment demanding blisters. More frequently
surgical operations do badly. Allied to this tendency is a spongy state
of the gums, with recession and excavation, resulting, in asthenic
cases, in absorption of the alveolar processes and falling out of the
teeth. Eczema of the labia and vicinity in females, and a similar
irritation about the meatus urinarius in males, are annoying symptoms.
A purulent-looking discharge has been seen issuing from the urethra, in
which the spores of penicilium glaucum have been recognized by the
microscope.

The term diabetic coma is applied to a form of coma which is apt to
occur late in the disease, indeed most frequently to terminate it;
while it is also used to indicate a train of nervous symptoms of which
coma is the terminal one. To this train of symptoms the word acetonæmia
is also applied, and should alone be used, while the term diabetic coma
should be restricted to the terminal symptom. The coma, as well as the
previous nervous symptoms, is considered due to the accumulation in the
blood of a product of the decomposition of sugar, formerly believed to
be acetone, but now thought to be an acetone-producing substance,
probably aceto-acetic acid. It is likely that in all cases of diabetes
a small quantity of this substance exists in the blood, from which it
is separated by the kidneys and lungs, while it is only when these
channels are insufficient for its removal that it accumulates and
produces the symptoms described.

Usually, the coma comes on gradually, deepening until it terminates in
death. In other instances it is preceded by various symptoms, including
dizziness, drowsiness, cephalalgia, delirium, mania, muscular pains,
gastric and intestinal symptoms, including epigastric pain,
vomiting--sometimes of blood--and even purging; also dyspnoea, with
short, panting respiration like that of an animal with both vagi cut,
and a fluctuating pulse-rate which continues until coma is established,
after which it remains rapid and small. Both the breath and urine may
exhale the peculiar odor of acetone, or it may be absent, and the urine
strikes the peculiar burgundy-red reaction with perchloride of iron to
be again referred to.

These symptoms may be sudden in their occurrence, whence acute
acetonæmia, or they may ensue slowly. Ralfe,[17] who has studied the
subject of acetonæmia very thoroughly, has called attention to the
parallelism between the phenomena of acute acetonæmia and those of
acute yellow atrophy of the liver and of phosphorus-poisoning. The
sudden, sharp epigastric pain, with gastric disturbance and vomiting,
often of blood; the peculiar panting dyspnoea referred to; the short,
{206} noisy delirium, followed almost suddenly by deep coma; the fall
in temperature as the nervous symptoms develop; the irregular, and
finally rapid, pulse,--are all symptoms common to the two conditions.

[Footnote 17: _Clinical Chemistry_, 1883, p. 98; also Discussion on
Diabetes before Pathological Society of London, _Lancet_, April 7,
1883, p. 592.]

Although acknowledged to be a grave complication, and the most frequent
cause of death in diabetes,[18] yet it does not follow that a fatal
termination is inevitable when diabetic coma sets in. I have now a
patient, a woman, who considers herself in perfect health, but in whom
there remains a trifling glycosuria, who at one time was supposed to be
dying of diabetic coma.

[Footnote 18: Of 400 cases of diabetes which passed under the
observation of Frerichs, the majority died of acetonæmia (Frerich's
"Ueber den plötzlichen Tod und über das Coma bei Diabetes," _Zeitschr.
für klin. Med._, 1883, vi. 3-53). Of 53 persons dying of diabetes at
Guy's Hospital, London, during the last ten years, 33 died comatose
(Dr. Fred. Taylor, Discussion on Diabetes, Pathological Society of
London, _Lancet_, May 5, 1883). In my own experience acetonæmia has not
been so frequent a cause of death as phthisis, acute pneumonia, and
heart-failure.]

Crampy pains in the legs and facial paralysis are among the nervous
symptoms sometimes present, and the term diabetic neuralgia has been
applied to a special form of neuralgia peculiar to this disease. It is
characterized by its acuteness, stubbornness, and symmetry. Its
favorite seats are the inferior dental nerves and the sciatics.
Greisinger referred to the frequency of sciatica in 1859, Braun again
in 1868, and others still later; but Worms in 1881 established the
close relation between the two conditions and the features described.
Most recently (1884), Cornillon[19] collected 22 cases of diabetic
neuralgia, and has further elaborated the study. Believing that
diabetes affects particularly those persons who have had serious
attacks of rheumatism and gout, he is inclined to think the neuralgia
as much due to uricæmia as to hyperglycosuria, and that these
conditions cause, not neuritis, but transitory lesions in the
nerve-centres, but whether in the membranes or gray or white matter is
undetermined.

[Footnote 19: "Des nevralgies diabétiques," _Revue de Médecine_, 1884,
iv. 213-230.]

That the phenomena of acetonæmia are those of a toxic agent or agents
in the blood derived from the sugar there present is generally
conceded, although Sanders and Hamilton,[20] after a study of the
clinical histories and the result of autopsies in several cases, are
disposed to ascribe diabetic coma to slow carbonic-acid poisoning due
to fat embolism of the pulmonary vessels. So far as I know, these
conclusions have not been reached by any other observers. R. H.
Fitz[21] and Louis Starr[22] have each reported cases of diabetic coma
with lipæmia, carefully studied with this point in view, without
finding any facts to sustain the carbonic-acid theory.

[Footnote 20: _Edinburgh Med. Journal_, July, 1872.]

[Footnote 21: "Diabetic Coma; its relations to Acetonæmia and Fat
Embolism," _Boston Medical and Surgical Journal_, vol. cvi. p. 24, Feb.
10, 1881.]

[Footnote 22: "Lipæmia and Fat Embolism in Diabetes Mellitus," _New
York Medical Record_, vol. xvii., 1880, p. 477.]

Alterations in the Blood.--The blood of diabetics is variously charged
with sugar, which may be in such quantity as to impart a viscidity and
higher specific gravity to the plasma, which has reached 1033, the
normal being 1028. On the other hand, analyses have sometimes failed to
discover sugar in the blood after death, the result, probably, of the
tendency of the sugar to rapid disintegration. Alcohol and acetone, or
{207} acetone-producing substance (aceto-acetic acid), are occasionally
present as the products of such decomposition, to which are ascribed
the symptoms of acetonæmia already discussed.

The presence of fat in the blood of diabetics was noted by the earliest
students of the disease. It is sometimes sufficient in amount to
produce a milky appearance of the serum, while the analyses of Simon
revealed a quantity of 2 to 2.4 per cent., the normal being 1.6 to 1.9
per cent. The fat thus present is said to be sometimes sufficient to
cause fat embolism in the capillaries of the lungs, and cases of this
condition have been reported by Sanders and Hamilton,[23] Louis
Starr,[24] and Rickards.[25] Ralfe ascribes the lactescent appearance
of the blood to the action of the aceto-acetic acid, since acetic will
give a milky appearance when agitated with a dilute and slightly
alkaline mixture of fatty matter at 100°, and the injection of acids
into the blood of animals leads to the increase of fatty matter in the
blood and fatty infiltration of tissues.

[Footnote 23: _Loc. cit._]

[Footnote 24: _Loc. cit._]

[Footnote 25: _Birmingham Med. Review_, Jan., 1882.]

It must be admitted that the mode in which this lipæmic state of the
blood is brought about is imperfectly understood, and whether it be by
some chemical agency of the kind described by Ralfe, or by rapid
absorption of the subcutaneous fat, or from an imperfect oxidation of
absorbed fat, is undetermined. Possibly all may contribute.

Albert G. Heyl[26] has described an altered appearance of the retinal
vessels recognizable by the ophthalmoscope, which he ascribes to the
fatty blood-plasma at the periphery of the blood-current, the normal
plasma being invisible on account of its transparency.

[Footnote 26: For a detailed description of this appearance, with a
colored lithograph depicting it, see the author's work on _Bright's
Disease and Diabetes_, p. 262.]

The red blood-discs are diminished and their ratio to the white
corpuscles altered. In a count by F. P. Henry, in Louis Starr's case,
the number of red discs was 4,205,000 to a cubic millimeter, the normal
being at least 5,000,000; the white were 50,000 to a cubic millimeter,
or 1 white to 84 red, instead of 1 to 350 or 500.

Changes in the Urine.--The most important changes in the urine are its
increase in quantity and the presence of sugar. The variations in the
former are extreme, being from an amount which but slightly exceeds the
normal to as much as 50 pints (23.65 liters) in twenty-four hours, and
even more. The quantity is of course limited by the fluid ingested, and
although it may exceed this amount for a day or more, it cannot do so
for any length of time. It is generally a little less. The more usual
quantity in the twenty-four hours is from 70 to 100 ounces (210 to 300
cc.).

The quantity of sugar varies greatly in different cases and at
different times in the same case. The maximum quantity reported by
Dickinson was 50 ounces, or 1500 grammes, in twenty-four hours. The
proportion may reach as much as 15 per cent., but the more usual
amounts are from 1 to 8 per cent., or from 5 to 50 grains (.324 to 3.24
grams) to the fluidounce, or from 300 to 4000 grains (19.44 to 260
grams) in the twenty-four hours.

It is important to know that intercurrent febrile disease may produce a
decided diminution in the daily quantity of urine, and of the sugar
contained in it. A similar decrease, and even disappearance, is said to
take place sometimes toward the fatal termination of a case.

{208} The effect of exercise upon the sugar secretion is not uniform.
Bouchardat and Kuelz have noted a diminution, and even disappearance,
of sugar from urine as its result, and it is reasonable to suppose that
judicious exercise is at least without harmful effect, while it is
certain too that muscular exercise, if excessive, will increase
glycosuria.

Changes in diet of course modify the secretion of sugar, starches and
saccharine foods increasing it, while nitrogenous and oily foods
diminish it. So, too, the urine secreted on rising in the morning has
almost always less sugar in it than that passed on retiring; and it is
not rare to find no sugar in urine passed on rising, when that passed
on retiring at night may contain a small amount of sugar--from ¼ to 1
per cent. On the other hand, I have found a small amount of sugar in
the morning urine when the evening urine contained none. Anxiety and
excitement both increase the proportion of sugar.

Inosite, or muscle-sugar, is sometimes associated in urine with
diabetic sugar, and occasionally replaces it. So, too, in experiments
upon animals puncture of the fourth ventricle is sometimes followed by
inosuria instead of glycosuria, and in corresponding organic disease of
the brain the same thing is observed. The substitution of grape-sugar
by inosite in the course of diabetes is considered by Laboulbène[27] a
favorable change.

[Footnote 27: "Note sur l'Inosurie, succédant au diabète glycosurique,
et paraissant avoir une action favorable," _L'Union Médicale_, Oct. 14,
1883.]

As would be expected, the specific gravity of saccharine urine is
usually high--most frequently from 1025 to 1040--and Bouchardat noted a
specific gravity of 1074 in one instance. On the other hand, I have
found sugar easily detectable in urine with a specific gravity as low
as 1010. Pavy records an instance of the same specific gravity, and
Dickinson one in which the specific gravity was as low as 1008. It is
to be remembered that the sugar is rapidly destroyed when fermentation
sets in. A coincident diminution in the urea and other solids of the
urine will reduce the specific gravity of a saccharine urine otherwise
heavier.

The depth of color of diabetic urine is inversely as the quantity
passed. Hence, when this is very large the urine is pale, and even
almost colorless, but it may still contain considerable amounts of
sugar and possess a decided color, quite as deep as that of urine
passed in smaller quantity. When exposed to the air, diabetic urine
becomes rapidly turbid from the growth of fungi, including the yeast
fungus and penicilium glaucum.

The odor of diabetic urine just passed is usually in no way peculiar,
but as fermentation progresses an acetous odor is developed, which is
ascribed to acetic acid. At other times the odor is quite peculiar,
being spoken of as vinous or compared to that of sour beer, stale
fruit, alcohol, chloroform, or, as by one of my patients, to
sweetbrier.

Diabetic urine has almost invariably an acid reaction, which becomes
more decided as fermentation progresses. As a consequence of this
increased acidity, and sometimes independent of fermentation-changes,
the urine deposits a sediment of uric acid, but with this exception
diabetic urine is generally free from sediment. Diabetic patients on a
meat diet sometimes have a good deal of uric acid from this source.

Albuminuria may coexist with glycosuria, but is not generally found
until late in the disease, after changes in the kidney begin to make
their {209} appearance, unless, as may happen, glycosuria supervenes
upon primary renal disease.

Alcohol and acetone, or an acetone-yielding substance--aceto-acetic
acid--are sometimes found in diabetic urine. They are products of the
breaking up of sugar, but chemists do not explicitly agree as to the
exact method in which acetone originates in the organism. First
recognized in the distillate of urine and blood of a diabetic patient
by Petters[28] through its physical properties, odor, combustibility,
etc., rather than by actual isolation, it was further investigated by
Kaulich,[29] Gerhardt,[30] Rupstein,[31] and Markownikoff,[32] who
obtained it in an impure state from urine; by Deichmüller and
Tollens,[33] whose isolated substance was pure, and finally most
recently by Jaksch[34] and Penzoldt.[35] The former found it not only
in diabetic urine, but also in that of fever, and even of carcinoma.
The latter found it by the indigo test in but 18 out of 22 diabetics,
and by the iodoform test, either decidedly or feebly, in 20 out of 20;
in 3 out of 11 cases of typhoid fever, in 6 out of 7 cases of
pneumonia, in none of 6 cases of phthisis, in 1 out of 3 cases of
measles, and in 1 case of cerebro-spinal meningitis. Finally, v. Jaksch
has been led to believe, from his extensive investigations, that
acetone is a constant and normal product of tissue-change, although
Penzoldt considers such conclusion scarcely justified.

[Footnote 28: _Prager Vierteljahrschrift_, xiv. 3, 1857, S. 88.]

[Footnote 29: _Ibid._, xvii. 3, 1860, S. 59.]

[Footnote 30: _Wiener Med. Presse_, No. 28, 1865.]

[Footnote 31: _Centralbl. für d. med. Wiss._, No. 55, 1874.]

[Footnote 32: _Liebig's Annalen_, Bd. 182, S. 362.]

[Footnote 33: _Ibid._, Bd. 209, S. 25.]

[Footnote 34: _Zeitschrift für physiol. Chemie_, vi. 6.]

[Footnote 35: "Beiträge zur Lehre von der Acetonurie und von verwandten
Erscheinungen," _Deutsch. Archiv für klin. Med._, xxxiv., 2 Oct., 1883,
S. 127.]

Gerhardt early discovered a substance in the urine of diabetics and
habitual drinkers which struck a deep-red reaction with chloride of
iron. This he considered was the source of acetone, and was probably
ethyl diacetate or diacetic ether, which by decomposition yields equal
molecules of acetone and alcohol; thus:

  C_{4}H_{5}O_{3}C_{2}H_{5} + H_{2}O = C_{3}H_{6}O + CO_{2} +
                                                          C_{2}H_{6}O.

  Ethyl diacetate.            Water.     Acetone.            Alcohol.

This view is still held by some, but others, in view of the recent
discovery of Deichmüller and Tollens,[36] that diabetic urine when
distilled yields decidedly more acetone than alcohol, have suggested
that the substance is derived from aceto-acetic acid.

[Footnote 36: _Loc. cit._]

The first test suggested for acetone was Gerhardt's chloride-of-iron
test. A solution of chloride of iron added to urine containing acetone
strikes a burgundy-red color. But this reaction occurs with so many
substances that it cannot be considered entirely reliable. Ralfe's
modification of Lieben's iodoform test[37] is made as follows: About a
fluidrachm (3.7 c.c.) of liquor potassæ, containing 20 grains (1.2
grams) of iodide of potassium, is placed in a test-tube and boiled; a
drachm (3.7 c.c.) of the suspected urine is then carefully floated upon
the surface. When the urine comes in contact with the hot alkaline
solution a ring of phosphates is formed, and after a few minutes, if
acetone or its allies are present, the ring will become yellow and
studded with yellow dots of iodoform, which, in turn, will sink through
the ring of phosphates and deposit itself at the bottom of the
test-tube. A number of other substances {210} produce the iodoform
reaction, but only one of these, lactic acid, is likely to be met in
urine.

[Footnote 37: _Clinical Chemistry_, Philadelphia, 1884, p. 100.]

The perspiration, saliva, exudations, and effusions in diabetic cases
have all been found, at times, to contain sugar.

DURATION.--Diabetes is a disease of which the duration is measured by
months and years, and although cases are reported in which death
supervened in from six days to six weeks after the recognition of the
disease, it is evident that such periods do not necessarily measure its
actual duration. The disease may have existed some time before coming
under observation. On the other hand, a case is reported by Lebert
which lasted eighteen years; another, under the successive observation
of Prout and Bence Jones, sixteen years; and a third, under Bence Jones
and Dickinson, fifteen years. The younger the patient the shorter
usually is the course run and the earlier the fatal termination. Yet I
have known a girl of twelve recover completely. After middle age the
disease is usually so easily controlled by suitable dietetic measures,
if the patient is willing to submit to them, that its duration is only
limited by that of an ordinary life, while carelessness in this respect
is apt to be followed by early grave consequences.

COMPLICATIONS.--The almost sole complication of diabetes mellitus is
the tubercular phthisis which so often terminates it. Indeed, it is
doubtful whether this complication should not be regarded as a
consequence, as should also the boils, gangrenous processes, and
ophthalmic conditions which have been mentioned under Symptomatology.
Jaundice has occurred three times in my experience up to the present
time. Senator says that when not an accidental complication due to a
catarrh of the duodenum it may result from compression of the biliary
capillaries by the overloaded blood-vessels and enlarged gland-cells of
the liver. In one of my cases, in which jaundice appeared to be the
initial symptom, but which disappeared some months before death, the
autopsy revealed atrophy of the liver. It is well known that pancreatic
disease, especially cancer, is apt to be accompanied by jaundice, and
as pancreatic disease is often at the bottom of diabetes, it will
similarly account for the jaundice, while the presence of jaundice may
also suggest a pancreatic diabetes.

DIAGNOSIS, INCLUDING THE TESTS FOR SUGAR IN THE URINE.--The diagnosis
of diabetes mellitus, the disease being once suspected, is easy. The
passage of large amounts of pale urine of high specific gravity, the
presence of thirst, dryness of the mouth, fauces, and skin, and
progressive emaciation even while the appetite is good, can scarcely be
misinterpreted. In the urine from such a case the application of any of
the tests for sugar will produce prompt response. The urine is not
always so much increased as to attract attention, while its color is
also sometimes but slightly changed; but the symptoms of thirst and
dryness or clamminess of the mouth are seldom wanting. On the other
hand, the discovery of a glycosuria without these symptoms is, as a
rule, accidental. It is a question how far such degrees of glycosuria
as do not produce the usual symptoms of diabetes in an appreciable
degree are signs of positive disease. At the same time, its detection
is important, in that there is always danger of the simple glycosuria
becoming a diabetes--a danger which its recognition and suitable
treatment may avert. Accordingly, the urine of all persons having
unusual appetites without evident cause, {211} and of those who are
fond of eating and drinking, should be tested for sugar. This should
also be done for those who have passed through severe mental or
physical strain, have suffered shock or concussion of the nervous
system, blows upon the abdomen, etc.

Testing for Sugar.--Under the head of Diagnosis I prefer to include the
testing for sugar, which requires some detailed consideration. Unless
it be that the indigo test recently revived by George Oliver of London
prove more delicate, that form of cupric test known as Fehling's
solution is, with suitable precautions, all things considered, the most
satisfactory for general use.

Fehling's volumetric solution, suitable for both qualitative and
quantitative purposes, is made as follows: Dissolve 34.639 grams of
pure crystallized cupric sulphate in about 200 cubic centimeters of
distilled water; 173 grams of chemically pure crystallized neutral
sodio-potassic tartrate and 80 grams of potassium hydrate in 500 or 600
c.c. of distilled water. To the latter add the copper solution slowly,
and dilute the clear mixed fluid to 1 liter. One cubic centimeter of
this solution will be decolorized by 0.005 grm. of sugar, or 200 grains
will be decolorized by 1 grain of sugar. Or the copper may be dissolved
in 1 liter of water, and the tartrate and potassium hydrate in another,
and a cubic centimeter of each mixed at the moment they are to be used.

For qualitative testing, put a cubic centimeter of Fehling's solution
into a test-tube (or if the copper and the alkaline sodio-potassium
tartrate solutions are kept separate, a cubic centimeter of each), and
dilute with distilled water to 5 c.c. Boil, and if, after the lapse of
a couple of minutes, the solution remain unchanged, it is fit for
testing. If it becomes turbid or a red sediment falls, it is spoiled,
and a new solution should be obtained.[38] A cubic centimeter of the
suspected urine is then measured out and added drop by drop to the
solution kept hot. If there is much sugar, the first drop will throw
down a yellow precipitate of suboxide of copper, which becomes rapidly
red. If no reaction takes place after adding the entire cubic
centimeter of urine, the addition should be continued until 4 c.c. are
added, when, if, after the mixture has cooled, there be no response, it
may be concluded that the urine is free from sugar. By operating with a
cubic centimeter of the test-fluid and the same quantity of urine or
multiples thereof, we may roughly estimate the proportion of sugar.
Thus, if the cubic centimeter of undiluted urine just decolorizes the
cubic centimeter of Fehling's solution, sugar is present in the
proportion of one-half of 1 per cent.; or if a half cubic centimeter of
the urine removes all the color, the quantity is 1 per cent. If the
urine is highly charged with sugar, it may be diluted, and the degree
of dilution being remembered, a rough quantitative estimation may be
similarly made.

[Footnote 38: Should this not be possible, a little more soda may be
added and the fluid filtered, when it is again ready for use.]

If the urine contains very minute quantities of sugar, the reaction is
less satisfactory. The copper is reduced, but the suboxide is so small
in quantity that it is obscured by the excess of copper solution, and a
mixture results which is greenish or greenish-yellow or yellow or
milky, and on standing a small yellow sediment falls to the bottom.
Now, it dare not be said that it is sugar which produces such reaction.
It may be {212} sugar, but it may also be uric acid. Uric acid is
really more frequently a source of error than is commonly supposed. I
have myself seen the reaction due to it so vivid that I did not suspect
it could be due to any reducing agent excepting sugar; but, noting the
next day a copious sediment of uric acid which had fallen during the
night, a testing of the supernatant fluid then revealed no reaction
whatever. Such a urine, after being treated by the lead process to get
rid of the uric acid, fails also to respond. But this process is very
tedious,[39] and cannot be conveniently carried out by the busy
practitioner. The same thing is, however, accomplished by treating the
urine with hydrochloric acid, which in twenty-four hours precipitates
all of the uric acid. Simple precipitation by lead acetate solution and
filtration does not answer, because all of the uric acid is not thus
removed. Other substances, as hippuric acid, urates, hypoxanthin, etc.,
are said to act similarly, but they produce no practical interference
with the test. On the other hand, a small amount of sugar may be
present and yet fail to show the reaction, because the cuprous oxide is
held in solution by certain substances. Such are ammonia and
nitrogenous matters, including albumen, creatinin, pepsin, peptones,
urinary coloring matters, etc. The latter probably produce their effect
through the ammonia which is given off while heating them in the
presence of an alkali. Hence all albumen should be precipitated and
filtered out of urines suspected to contain sugar, and the heat applied
should not be too great. Finally, excess of glucose will also hold in
solution cuprous oxide, so that the suspected urine should not be added
in too large a quantity at a time, but rather drop by drop.

[Footnote 39: The details of this process will be found in the writer's
work on the _Practical Examination of Urine_, 5th ed., 1883, p. 63.]

But qualitative testing is not sufficient during the treatment of a
case of diabetes. The percentage of sugar and the quantity discharged
in twenty-four hours should be determined occasionally. The process is
done as follows: Place 10 cubic centimeters of Fehling's solution in a
porcelain capsule, and dilute it with 40 c.c. of distilled water. Fill
a Mohr's burette with the urine, which, if it contain more than 1 per
cent. of sugar, should be diluted with nine times its bulk of distilled
water. Slowly heat the contents of the capsule to boiling, and then
allow a little of the diluted urine to run in from the burette;
continue the cautious addition of urine and the gentle heating until
the blue color is completely removed from the Fehling's solution. To
determine the exact moment at which this takes place requires a little
experience, but its recognition is facilitated by carefully tilting the
capsule after each addition and stirring, so that its clear white
surface may be seen through the edge of the fluid and contrasted with
the latter. The number of cubic centimeters of urine used should now be
read off from the burette, the number of c.c. of undiluted urine
calculated therefrom, and each c.c. multiplied by .005 grm. The result
indicates the quantity of sugar in grams in the urine employed, whence
the percentage of sugar is determined, and also the twenty-four hours'
quantity, the amount of urine passed in that period being known.

The Fermentation Test.--A very simple and easy method of determining
the proportion of sugar is by Roberts's fermentation method, which,
although not so precise as the volumetric process, is still {213}
sufficiently so for clinical purposes. A small piece of German yeast or
a teaspoonful of liquid yeast is added to about four ounces (120 c.c.)
of the urine, which is kept lightly stopped, at a temperature of 20° to
30° C. (68° to 80° F.), for about twelve hours; at the end of this time
the sugar will have been converted into alcohol and carbonic acid. The
latter will have passed off, and the urine lost in weight because of
the destruction of sugar; while the difference between the specific
gravity before and after the fermentation indicates the number of
grains of sugar per fluidounce. Thus, suppose the specific gravity
before fermentation to have been 1040, and afterward 1025; there will
have been 15 grains of sugar to the fluidounce, whence, again, the
twenty-four hours' quantity can be calculated. If the metric system is
used, each degree of specific gravity lost will correspond to .2196
grams of sugar in every 100 c.c. of urine.

The specific gravity of the fermented urine should be compared with
that of the urine soon after it is passed, because saccharine urine
under suitable circumstances undergoes fermentation without the
addition of yeast; and, the specific gravity being thus lowered
spontaneously, the reduction in the urine fermented by yeast would
appear less than it actually is. At the same time, care should be taken
that the urine is of the same temperature when the specific gravity is
taken before and after fermentation.

The Picric Acid and Potash Test.--Although attention was called in 1865
by C. D. Braun,[40] a German chemist, to a reaction between grape-sugar
and picric acid, as the result of which the latter is converted into
picramic acid, very little attention seems to have been paid to this
announcement. Quite ignorant of it, George Johnson rediscovered this
reaction in 1882, and published it in 1883.[41] It is applicable to
both qualitative and quantitative purposes. In order to make use of it,
a standard comparison-solution is made as follows: Take 1 fluidrachm of
a solution of grape-sugar, 1 grain to the fluidounce; mix it in a long
test-tube with half a drachm of liquor potassæ (U. S. P. or B. P.) and
ten minims of a saturated solution of picric acid; dilute the mixture
to 4 fluidrachms with distilled water, to facilitate which a tube used
for the purpose may be marked at 4 fluidrachms. Raise the mixture to
the boiling-point, and continue the boiling for sixty seconds, to
ensure complete reaction between the sugar and picric acid. During the
boiling the pale-yellow color of the liquid is changed to a vivid
claret-red. Cool the liquid by cautiously immersing the tube in cold
water, and if it is not then at the level of the 4-drachm mark, raise
it to this by adding distilled water. The standard color thus obtained
is that which results from the decomposition of picric acid by a grain
of sugar to the ounce, four times diluted, or by a solution of sugar
containing one-quarter of a grain per ounce. But the picramic solution
rapidly becomes pale on exposure, so it becomes necessary to make a
more permanent solution to use as a standard. This may be accomplished
by combining liquor ferri perchloridi drachm j, liquor ammonii acetatis
drachms iv, acidum aceticum (glacial) drachms iv, and water enough to
make ounces iiss. The color of this is identical with that of the
picric acid reduced by a one-grain solution diluted four times, and,
{214} according to Johnson, it will retain its color unchanged for at
least six months. At the same time, whenever a new solution is made it
should be compared with that of the one-quarter grain per ounce
solution of sugar, boiled with picric acid and potash.

[Footnote 40: "Ueber die Umwandlung der Pikrinsaüre in Pikramminsaüre,
und Ueber die Nachweisung der Traubenzucker," _Zeitschrift für Chemie_,
1865.]

[Footnote 41: _British Medical Journal_, March, 1883.]

For qualitative testing Johnson directs: To a drachm of urine in a
test-tube add a few drops, enough to give a distinct yellow color, of a
saturated solution of picric acid. Add about 10 drops of liquor potassæ
and boil. If sugar is present, the mixture becomes promptly red in hue.

[Illustration: FIG. 5. _Johnson's Picro-Saccharimeter_.

The shading of the side tube indicates the ferric-acetate standard. The
darker shading at the bottom of the graduated tube shows the saccharine
fluid, darkened by boiling with picric acid and potash, and occupying
ten divisions between dilution.]

The quantitative estimation is based upon an accurate approximation, by
dilution, of the color of the tested fluid with that of the standard
solution. Johnson recommends the picro-saccharimeter figured in the
text. This is a stoppered tube twelve inches long and three-quarters of
an inch in diameter, graduated into ten, and each of these again into
ten other equal divisions. By the side of this tube, and held in
position by an S-shaped band of metal, is a stoppered tube of equal
diameter and about six inches long, containing the standard solution
corresponding to the reaction of the one grain of grape-sugar with
picric acid and potash diluted four times.

It has been found that ten minims of a cold saturated solution of
picric acid are rather more than sufficient for decomposition by one
drachm of a solution of grape-sugar in the proportion of one grain to
the ounce. A drachm of the solution will therefore contain one-eighth
of a grain of sugar, which is the strength of the solution used in
making the standard-color liquid. In making the analysis, while the
quantity of liquor potassæ used is always the same and the dilution is
always to four drachms, the picric acid must be added in proportion to
the amount of sugar present, so that if the urine contains as much as
six grains to the fluidounce, sixty drops or a fluidrachm of the
picric-acid solution would have to be used; and when the proportion of
sugar is higher than this, the urine should be diluted with distilled
water five or ten times before commencing the analysis, and the degree
of dilution remembered in the computation.

If, now, a drachm of a solution of grape-sugar, containing two grains
to the ounce, be mixed with the same quantity of liquor potassæ and
picric acid and increased by the addition of distilled water to four
drachms in the boiling tube, and boiled as before for sixty seconds,
the result will be a mixture of much darker color than will be produced
by the one-grain solution; but if the dark liquid be diluted with its
own volume of water, the color will be the same as that of the
one-grain solution or the standard.

It is plain, then, that if a given quantity of the dark saccharine
fluid produced by boiling--say, enough to cover ten divisions of the
graduated tube, as shown in the figure--has to have added to it an
equal bulk of distilled water in order to produce {215} the color of
the standard solution, the tested fluid will be of the strength of two
grains to the ounce; if three times, three grains; and so on; while
fractional additions, as indicated by the graduated markings, would
show fractional additions to the proportion of sugar.[42]

[Footnote 42: A more exact comparison of the saccharine liquid with the
standard is made by pouring into a flat-bottomed colorless tube six
inches long and an inch in diameter as much of the standard solution as
will form a column about an inch in height, and an exactly equal column
of the saccharine fluid in a precisely similar tube. The operator then
looks down through the two tubes at once, one being held in each hand,
upon the surface of a white porcelain slab or piece of white paper. In
this way slight differences of tint are easily recognized; and if the
liquid to be analyzed is found darker than the standard, it is returned
to the graduated tube and diluted until the two liquids are found to be
identical in color, when the final reading is made.]

The presence of albumen, even in considerable amount, has but little
effect upon the test, nor does the coloring matter of normal urine,
according to Johnson; but he says there is a coloring matter associated
with ser-albumen in albuminous urine, and with egg-albumen as well,
which has a reducing action on picric acid. This is partly separated by
filtering off the precipitated albumen, and entirely removed by
repeated filtration through animal charcoal. So, too, the albumen
removed by coagulation and filtration, if thoroughly washed, does not
give any red reaction if boiled with picric acid and potash diluted in
the same proportion as when testing for sugar. Neither do any other
unoxidized sulphur compounds found in urine decompose the picric acid
and render the test fallacious.

Johnson and his son, G. Stillingfleet Johnson, claim that the
picric-acid test is as accurate as any other, and that it is even more
accurate than either Fehling's or Pavy's process, because the picric
acid is not acted upon by uric acid or urates, which do reduce the
oxide of copper. The method of analysis by the picro-saccharimeter,
they claim, is at least as speedy and as easy as any other. The
materials and apparatus required are easily prepared, inexpensive, and
not, like Fehling's copper solution, liable to undergo rapid changes.

But while Johnson claims that neither coloring matters of normal urine
nor uric acid reduce the picric acid, he admits that he has tested with
picric acid and potash a large number of specimens of normal urine with
the almost uniform result of a depth of color indicating the proportion
of .6 of a grain of sugar to the fluidounce, the indication varying
between the limits of .5 to .7 grain. The ammonio-cupric method used at
the same time gave results of from .7 to .9 grain to the fluidounce, or
an excess of .1 to .3 grain. Now, if my own views, the grounds for
which are announced elsewhere,[43] are correct, strictly normal urine
contains no sugar, and any reducing action upon oxide of copper is due
to uric acid, either picric acid is reduced to a degree by uric acid or
by some other constituent of normal urine. This, in the light of
Oliver's[44] recent investigations, may be kreatinin. For he has shown
that kreatinin strikes in a few seconds a red color with the cold
alkaline picric solution, which is quickened by heat. From this it
would seem that the exact value of the picric-acid test has as yet to
be determined.

[Footnote 43: Tyson, _Practical Examination of Urine_, 4th ed.,
Philadelphia, 1884.]

[Footnote 44: _On Bedside Urine-Testing, including Qualitative Albumen
and Sugar_, by Geo. Oliver, M.D., London, Member of the Royal College
of Physicians of Lond., etc., 2d ed., London, 1884.]

{216} The Indigo-Carmine Test.--The fact that indigotine, the coloring
matter of commercial indigo, is converted into indigo when heated with
an alkali in the presence of glucose and certain carbohydrates, has
recently been applied by George Oliver of London in the construction of
a test-paper. Carmine of indigo is the sulph-indigotate of sodium, an
intensely blue salt, soluble in 120 parts of water. Sulph-indigotic
acid is made by heating indigo with sulphuric acid, and when combined
with a base, sodium, produces indigo-carmine. When sodium carbonate is
mixed with a solution of indigo-carmine, the latter is precipitated in
a minute state of division, but is redissolved on heating, when there
results a greenish-blue solution. A freshly-made mixture of the indigo
solution and sodium carbonate furnishes a fluid not unlike Fehling's
solution, which gives the reaction to be described with glucose.
Unfortunately, such a mixture will not keep, and the reagent would be
useless but for the happy idea of Oliver of making the test-paper. In
doing this bibulous paper is immersed in a solution of indigo-carmine
with carbonate of sodium.[45] The paper is then cut into strips an inch
long and one-quarter of an inch wide.

[Footnote 45: No more precise directions than this are given by Oliver,
either in his papers in the _Lancet_ for 1883 or in his little book
just published, _On Bedside Urine-Testing_. The sugar test-papers, as
well as the entire series of albumen test-papers, suggested by Oliver,
are now made by Parke, Davis & Co. of New York, and by Wilson & Son,
Harrogate, London.]

Mode of Testing.--One of the test-papers and a sodium carbonate
paper[46] are dropped into a half-inch test-tube, and water added until
the upper end is just covered; a column of fluid one inch in height and
half an inch in diameter will thus be produced, so that the solution of
carmine obtained on boiling will always acquire the same concentration.
Heat is now applied, the tube being gently shaken, and boiling kept up
for a second or two. A beautiful blue solution will result. The
test-paper may now be removed or allowed to remain.

[Footnote 46: Test-papers of the same size, charged with a saturated
solution of sodium carbonate.]

Not more than one drop of the suspected urine is let fall into the tube
from a pipette held in an upright position. Drops of equal size are
thus secured. The contents of the tube are again freely boiled for a
few seconds, after which the tube should be raised an inch or more from
the flame and held without shaking, while the solution is kept quite
hot, but not boiling, for exactly one minute. If glucose be present in
abnormal amount, the soft rich blue will be seen first of all to darken
into violet; then, according to the quantity of sugar, there will
appear in succession, purple, red, reddish-yellow, and finally
straw-yellow. When the last-named color has been developed the
slightest shaking of the tube will cause red streaks to fall from the
surface and mingle with the pale yellowness of the solution, while
further agitation will cause the return of purple and violet and the
restoration of the original blue.

The time required for the commencement of the reaction after the
boiling of the test liquid is in inverse proportion to the amount of
glucose present. When the latter is large, over 20 grains to the ounce,
it will be but a few seconds; but when small, 2 or 3 grains, from
thirty to sixty seconds may elapse. If the urine do not contain more
than the normal amount of sugar[47]--_i.e._ under half a grain to the
ounce--the color of the solution {217} at the end of the heating for
one minute will be unchanged. The test is available by artificial light
as well as by daylight.

[Footnote 47: It will be noted from this that Oliver accepts the view
that there is a small amount of sugar in normal urine.]

Precautions.--1. Care should be taken during the testing not to shake
the tube or to permit free ebullition. 2. While keeping the contents of
the tube hot, the latter should not be held up between the eye and the
sky, for then the early color-changes will probably escape observation.
The tube should be kept below the eye-level and its contents viewed by
the reflected light of some bright object, such as a sheet of white
paper propped up an inch or two beyond the tube as a background. 3.
Oliver is not aware that the presence of earthy carbonates will prevent
the carmine reaction, but as a precautionary measure he suggests the
use of a soda-paper whenever the water is exceptionally hard. 4. The
acids of the urine rob the carmine-paper of much alkali, so that the
addition of more than a certain number of drops of urine--varying of
course with the degree of acidity--will at first retard and then
prevent the reaction. The addition of the soda-paper will prevent any
such interference, although Oliver says that by invariably submitting
only one drop of saccharine urine to the test-paper, and keeping up the
heating for not less than two minutes, he has never failed to obtain
the characteristic reaction without using a soda-paper. It is well to
remember, however, that an excessively acid urine may thus interfere,
and that the soda-paper will prevent it. 5. The blue color of the
carmine is discharged by caustic alkali--liquor potassæ or sodæ. The
only chance of being misled by this reaction lies in using an
imperfectly cleansed test-tube which may have contained Fehling's
solution or the alkaline picric solution. The caustic alkali converts
the blue carmine into a green solution, which, on heating, disappears;
nor does it return by again shaking the contents of the tube.

Critical comparison of this test with Fehling's solution and picric
acid by Oliver has shown that of sixty-four substances experimented
upon, normal and abnormal constituents of urine or medicines which
after ingestion are eliminated in the urine, Fehling's was reduced by
fifteen, picric acid by eleven, and indigo-carmine by eight. The only
substances producing the characteristic play of colors with
indigo-carmine test-papers reacted with both picric acid and Fehling's
solution. They were unoxidized phosphorus, ammonium sulphide,
milk-sugar, dextrin, inosit, gallic acid, tannic acid, and iron
sulphate. Both the carmine and picric acid were reduced by inosit,
which merely turned Fehling's solution green. On the other hand, uric
acid and urates, which reduce Fehling's solution, do not react with the
carmine test, while kreatinin, which reacts with picric acid also, does
not respond to the carmine. Albumen, if abundant, interferes with
Fehling, but not with the indigo-carmine.

Detection of Inosit.--It has been said that inosit sometimes
accompanies, and even substitutes, grape-sugar in the course of
diabetes. It has been mentioned that it does not reduce Fehling's
solution, but turns it olive-green. It reduces the carmine and alkaline
picric acid solution, and is therefore not recognizable by these. The
methods recommended for its recognition in the books are troublesome,
and as its presence in the absence of sugar indicates a favorable
change, it is not likely that a more precise recognition than is
furnished by the olive-green reaction will be needed for clinical
purposes.

PROGNOSIS.--The prognosis in diabetes depends upon the organ whose
{218} involvement is responsible for the symptoms, upon the stage at
which the condition comes under observation, and upon the age of the
patient. It has appeared to me that the cases of diabetes depending
upon pancreatic disease are the most intractable, that their progress
is scarcely checked by treatment, and that they are comparatively
rapidly fatal in their termination. In the others, where the symptom is
one of a central nervous lesion, it has always seemed to me to be of
secondary importance that the glycosuria is itself less marked, that it
is unattended by the other distinctive symptoms of diabetes, and that
its issue is that of the nervous malady.

Again, it is well known that the later in life diabetes occurs the more
amenable it is to treatment, and that if a proper diabetic diet be
adhered to by the patient his life need scarcely be shortened. On the
other hand, diabetes mellitus is a disease in which the expectant plan
is dangerous. If it does not improve it usually gets worse; and many a
patient has fallen a victim to his own indifference and indisposition
to adhere to a regimen under which he could have lived his natural term
of life. This is especially the case when the disease appears after
middle life.

If, on the other hand, the condition becomes thoroughly established
before twenty-five years of age, it is less amenable to treatment; but
even in such cases a promptly vigorous treatment is sometimes followed
by recovery. I have already mentioned the case of a child twelve years
old in which complete recovery took place.

If tubercular phthisis supervenes, recovery is not to be expected,
while intercurrent disease, as pneumonia, which is rather prone to
occur, is very much more serious and apt to terminate fatally.

TREATMENT.--The treatment of the aggregate of symptoms known as
diabetes mellitus is conveniently divided into the dietetic, the
medicinal, and the hygienic, of which the first is by far the most
important. The efficiency of this treatment depends upon the successful
elimination from the diet of all articles containing grape-sugar,
cane-sugar, beetroot-sugar, and starch, it being universally recognized
that in the early stages of the disease these foods are the sole source
of the glucose in the urine. The normal assimilative action of the
liver, by which the carbohydrates are first stored up as glycogen, and
then gradually given out as glucose or maltose to be oxidized, being
deranged, such foods not only become useless as aliments, but if
continued seem to aggravate the glycosuria, and the excretion of sugar
steadily increases. There is, therefore, a double reason for excluding
them from the food. This is easiest accomplished by an exclusive milk
diet. The exclusive milk treatment of diabetes was suggested by A.
Scott Donkin in 1868. That he is correct in his assertion that in the
early stages of diabetes lactin or sugar of milk is quite assimilable,
and does not in the slightest degree contribute to the production of
glycosuria, I cannot doubt; that it is in this respect even superior to
casein, as claimed by Donkin, I am not prepared to state from actual
knowledge; but that casein itself resists the sugar-forming progress
immeasurably greater than any other albuminous substance, so that in
all but the most sure and advanced or complicated cases its arrest is
complete, I am also satisfied. Certain it is that in a large number of
diabetics the use of a pure skim-milk regimen results in a total
disappearance of the sugar from the urine. That in a certain proportion
of these cases a {219} gradual substitution of the articles of a mixed
diet may be resumed without a return of the symptoms is also true. In
other more confirmed cases the use of skim-milk results in a decided
reduction in the amount of sugar, with an abatement of other symptoms,
which continues as long as the diet is rigidly observed. In still other
cases, while the skim-milk treatment makes a decided impression upon
the quantity of sugar, it still remains present in considerable amount,
while the disease progresses gradually to an unfavorable issue. These
three classes of cases represent, ordinarily, different stages of the
disease, so that it may be said that as a rule cases recognized
sufficiently early may be successfully treated with skim-milk, although
it may occasionally happen that cases pursue a downward course from the
very beginning despite all treatment. Yet I have never seen a case
which, when taken in hand when a few grains of sugar only to the ounce
were present, failed to yield to this treatment.

As to the method of administration, my practice with adults is to give
eight ounces (an ordinary tumblerful) every two hours, beginning at
seven or eight o'clock in the morning, and continuing to the same hour
in the evening. Sometimes it is well to begin with half as much at
first, but rapidly to increase to the required amount. This method
ensures the ingestion of three to four quarts daily--a quantity
generally sufficient to maintain the body-weight of an adult person of
average size and taking moderate exercise, although a slight reduction
may take place at first. But if the individual is very active or of
large size, it will not be found sufficient. In such event the quantity
must be increased as demanded by a feeling of unsatisfied hunger. I
have known fourteen pints to be taken in twenty-four hours. But when
the quantity becomes thus large, the inconvenience in ingesting it is
very great, and it is much more convenient to coagulate the casein of a
part of the milk and use the curd thus obtained, while the second part
is drunk. Curd may be seasoned with salt to make it more palatable, and
should be thoroughly masticated before it is swallowed.

The milk should not be taken too cold, especially if the amount
ingested is large, else it is likely to reduce the temperature of the
stomach below the point necessary for gastric digestion. The
temperature should not be less than 60° F., nor much over 100°.
Something depends upon the idiosyncrasies of the patient, which must be
the guide as to temperatures intermediate between those named.

The chief advantage of the skim-milk over the unskimmed is simply that
it is more easy of digestion. Many persons who cannot take unskimmed
milk for any length of time without its deranging the digestion, or, as
is commonly said, making them bilious, can take with impunity milk from
which the cream is removed. Although Salomon[48] claims to have shown
that glycogen is produced in the liver of rabbits fed upon pure olive
oil, it is at least probable that fat is among the last of the
substances undergoing this conversion, and in ordinary cases of
diabetes it is rather its indigestible nature which renders it prudent
to remove from milk the greater proportion of fat by skimming it off.

[Footnote 48: _Virchow's Archiv_, Bd. 61, Heft 3, 1874, 18.]

Still more easily assimilable is the peptonized milk, in which the
casein is at least partially digested, and it should be employed where
there is any {220} difficulty in the way of using the ordinary milks.
Either skimmed or unskimmed milk may be used for peptonizing, the
latter peptonized being quite as easy of digestion as the former
unpeptonized. I have found the extractum pancreatis of Fairchild
Brothers & Foster most successful in the peptonizing of milk, and
according to the following directions: Into a clean quart bottle put 5
grains of extractum pancreatis, 15 of bicarbonate of sodium, and a gill
of cool water; shake, and add a pint of fresh cool milk. Place the
bottle in a pitcher of hot water or set the bottle aside in a warm
place, usually for three-quarters of an hour. When the milk has
acquired a slightly bitter taste, it has been completely
peptonized--that is, the casein has been completely converted into
peptone. After the process is complete the milk must be immediately put
on ice.

It is not always necessary to completely peptonize the milk, and if the
bitter taste is unpleasant the process may be stopped short of this by
putting the milk on ice, the degree of digestion depending upon the
length of time the milk is kept warm.

While I am confident that the promptest and most effectual method of
eliminating sugar from the urine is by a milk diet, it occasionally
happens that a patient cannot or will not submit to so strict a
regimen. In other instances, again, it is not necessary to resort to
it, because a less restricted diet answers every purpose.

A suitable diabetic diet would also be obtained by eliminating from the
bill of fare all saccharine and amylaceous and other sugar-producing
substances. Such a diet is, strictly speaking, impossible. For, apart
from the fact just mentioned that even fats, as well as albuminous
substances to a degree, are capable of producing glycogen, the monotony
of a pure meat diet soon becomes unbearable, to say nothing of other
derangements it may produce. Fortunately, it is not necessary that such
an exclusive diet should be maintained, for certain saccharine foods
seem capable of resisting the conversion into sugar more than others.
Sugar of milk, or lactin, has already been mentioned as one of these,
and to it may be added the sugar of some fruits, and probably also
inosit or muscle-sugar, mannite or sugar of manna, and inulin, a
starchy principle abundant in Iceland moss. It is found also that there
are many vegetable substances containing small quantities of sugar and
sugar-producing principles which may be used with impunity in at least
the milder forms of diabetes. This being the case, a bill of fare for
diabetics may be constructed quite liberal enough to satisfy the palate
of most reasonable persons by whom it is attainable.

FOOD AND DRINK ADMISSIBLE.--Shell-fish.--Oysters and clams, raw and
cooked in any way, without the addition of flour.

Fish of all kinds, fresh or salted, including lobsters, crabs,
sardines, and other fish in oil.

Meats of every variety except livers, including beef, mutton, chipped
dried beef, tripe, ham, tongue, bacon, and sausages; also poultry and
game of all kinds, with which, however, sweetened jellies and sauces
should not be used.

Soup.--All made without flour, rice, vermicelli, or other starchy
substances, or without the vegetables named below as inadmissible.
Animal soups not thickened with flour, beef-tea, and broths.

Vegetables.--Cabbage, cauliflower, brussels-sprouts, broccoli, green
{221} string-beans, the green ends of asparagus, spinach, dandelion,
mushrooms, lettuce, endive, coldslaw, olives, cucumbers fresh or
pickled, radishes, young onions, water-cresses, mustard and cress,
turnip-tops, celery-tops, or any other green vegetables.

Fruits.--Cranberries, plums, cherries, gooseberries, red currants,
strawberries, apples, without sugar. Or they may be stewed with the
addition of bicarbonate of sodium instead of sugar. (See below.)

Bread and cakes made of gluten, bran, or almond flour, or inulin, with
or without eggs and butter. Griddle-cakes, pancakes, biscuit,
porridges, etc. made of these flours. Where especial stringency is
required these should be altogether omitted.

Eggs in any quantity and prepared in all possible ways, without sugar
or ordinary flours.

Nuts.--All except chestnuts, including almonds, walnuts, Brazil-nuts,
hazel-nuts, filberts, pecan-nuts, butternuts, cocoanuts.

Condiments.--Salt, vinegar, and pepper in moderate quantities.

Jellies.--None except those unsweetened. They may be made of
calf's-foot or gelatin and flavored with wine.

Drinks.--Coffee, tea, and cocoa-nibs, with milk or cream, but without
sugar; also milk, cream, soda- (carbonated) water, and all mineral
waters freely; acid wines, including claret, Rhine, and still Moselle
wines, very dry sherry; unsweetened brandy, whiskey, and gin. No malt
liquors, except those ales and beers which have been long bottled, and
in which the sugar has all been converted into carbonic acid and
alcohol.

Vegetables to be especially Avoided.--Potatoes, white and sweet, rice,
beets, carrots, turnips, parsnips, peas, and beans; all vegetables
containing starch or sugar in any quantity.

The following list, including essentially the same articles, but
arranged in the shape of a true bill of fare, by Austin Flint, Jr.,[49]
will be found very convenient:

BILL OF FARE FOR DIABETES.--Breakfast.--Oysters stewed, without flour;
clams stewed, without flour. Beefsteak, beefsteak with fried onions,
broiled chicken, mutton or lamb chops; kidneys, broiled, stewed, or
devilled; tripe, pigs' feet, game, ham, bacon, devilled turkey or
chicken, sausage, corned-beef hash without potato, minced beef, turkey,
chicken, or game with poached eggs. All kinds of fish, fish-roe,
fish-balls, without potato. Eggs cooked in any way except with flour or
sugar, scrambled eggs with chipped smoked beef, picked salt codfish
with eggs, omelets plain or with ham, with smoked beef, kidneys,
asparagus-points, fine herbs, parsley, truffles, or mushrooms.
Radishes, cucumbers, water-cresses, butter, pot-cheese. Tea or coffee,
with a little cream and no sugar. (Glycerin may be used instead of
sugar if desired.) Light red wine for those who are in the habit of
taking wine at breakfast.

Lunch or Tea.--Oysters or clams cooked in any way except with flour;
chicken, lobster, or any kind of salad except potato; fish of all
kinds; chops, steaks, ham, tongue, eggs, crabs, or any kind of meat;
head-cheese. Red wine, dry sherry, or Bass's ale.

{222} Dinner.--Raw oysters, raw clams.

Soups.--Consommé of beef, of veal, of chicken, or of turtle; consommé
with asparagus-points; consommé with okra, ox-tail, turtle, terrapin,
oyster, or clam, without flour; chowder, without potatoes, mock turtle,
mullagatawny, tomato, gumbo filet.

Fish, etc.--All kinds of fish, lobsters, oysters, clams, terrapin,
shrimps, crawfish, hard-shell crabs, soft-shell crabs, (No sauces
containing flour.)

Relishes.--Pickles, radishes, celery, sardines, anchovies, olives.

Meats.--All kinds of meat cooked in any way except with flour; all
kinds of poultry without dressings containing bread or flour; calf's
head, kidneys, sweetbreads, lamb-fries, ham, tongue; all kinds of game;
veal, fowl, sweetbreads, etc., with curry, but not thickened with
flour. (No liver.)

Vegetables.--Truffles, lettuce, romaine, chicory, endive, cucumbers,
spinach, sorrel, beet-tops, cauliflower, cabbage, brussels-sprouts,
dandelions, tomatoes, radishes, oyster-plant, celery, onions,
string-beans, water-cresses, asparagus, artichoke, Jerusalem
artichokes, parsley, mushrooms, all kinds of herbs.

Substitutes for Sweets.--Peaches preserved in brandy without sugar;
wine-jelly without sugar, gelée au kirsch without sugar, omelette au
rhum without sugar; omelette à la vanille without sugar; gelée au rhum
without sugar; gelée au café without sugar.

Miscellaneous.--Butter, cheese of all kinds, eggs cooked in all ways
except with flour or sugar, sauces without sugar or flour. Almonds,
hazel-nuts, walnuts, cocoanuts. Tea or coffee with a little cream and
without sugar. (Glycerin may be used instead of sugar if desired.)
Moderately palatable ice-creams and wine-jellies may be made, sweetened
with pure glycerin; but although these may be quite satisfactory for a
time, they soon become distasteful.

Alcoholic Beverages.--Claret, burgundy, dry sherry, Bass's ale or
bitter beer. (No sweet wines.)

Prohibited.--Ordinary bread; cake, etc. made with flour or sugar;
desserts made with flour or sugar; vegetables, except those mentioned
above; sweet fruits.

[Footnote 49: "On the Treatment of Diabetes Mellitus," a paper read
before the American Medical Association at its meeting in Washington,
May, 1884, and published in the _Journal_ of the association July 12,
1884. I have so far modified the bill of fare as to permit the use of
milk, which Flint excludes.]

One of the foods the omission of which is most illy borne by the
diabetic, however great his previous indifference to it, is wheaten
bread, while the substitutes which have been at different times
suggested for it very imperfectly supply its place. Perhaps the best
known of these is the bread made of gluten flour. It was suggested by
Bouchardat in 1841, and is made by washing the ordinary wheat flour to
free it from starch.[50]

[Footnote 50: The Health Food Company, of 74 Fourth Avenue, N.Y.,
prepare a gluten flour by first removing the five bran-coats,
pulverizing the cleaned berry by the cold-blast process, stirring the
powder into iced water, and precipitating the gluten, cellulose, and
mineral matters, siphoning off the water holding in suspension the
starch, and drying out the precipitate. In this manner the salts of the
wheat are retained. A purified gluten made by the Health Food Company
is deprived of the cellulose walls of the cells in which the gluten
granules are held. Directions for making gluten bread and cakes of
various kinds are furnished by the company on application.]

Gluten flour, however prepared, contains some starch, as indeed it must
if bread is to be made out of it; and I confess to having been a good
deal disappointed in its use. I have known the sugar absent in a {223}
selected diet to return when gluten bread was permitted, and again
disappear on its withdrawal. Of course gluten flour contains less
starch than the ordinary wheat flour, and there may be cases where the
starch in the former can be assimilated when the quantity in the latter
cannot be. The gluten may be made into porridge.[51]

[Footnote 51: Gluten porridge is made by stirring the gluten into
boiling water until thick enough, and then keeping up the boiling
process for fifteen minutes. A little salt and butter are added at the
close to improve the flavor, and it may be eaten with milk or cream.]

A method of getting rid of the starch and sugar in bread, suggested by
Liebig and tried by Vogel, consists in converting the starch into sugar
by the action of diastase and dissolving out the sugar thus produced.
This is accomplished by treating thin slices of bread with an infusion
of malt. The bread is then washed, dried, and slightly toasted.

Another substitute for wheaten flour is the bran flour whence the
starch is removed by washing.[52] The bran itself, according to
Parkes,[53] sometimes contains as much as 15 per cent. of nitrogenous
matter, 3.5 per cent. of fats, and 5.7 per cent. of salts. It is
therefore not wholly innutritious, although the salts are washed out in
removing the starch. It is considered especially useful when there is
constipation, the slightly irritant properties of the bran aiding in
maintaining a proper peristalsis and action of the bowels. These
irritant properties are, however, inversely as the degree of
comminution. The bran flour may be made with milk and eggs into a
variety of cakes, of which the best known are those made according to
Camplin's directions.[54]

[Footnote 52: A very carefully prepared bran flour, as well as a
wheat-gluten flour, is prepared by John W. Sheddon, pharmacist, corner
of Broadway and Thirty-fourth street, New York City.]

[Footnote 53: _Practical Hygiene_, 5th ed., Philadelphia, 1878, p.
222.]

[Footnote 54: The following are Camplin's directions for making biscuit
of bran flour: To one quarter of a pound of flour add three or four
fresh eggs, one and a half ounces of butter, and half a pint of milk;
mix the eggs with a little of the milk, and warm the butter with the
other portion; then stir the whole together well; add a little nutmeg
or ginger or other agreeable flavoring, and bake in small forms or
patterns. The cake, when baked, should be about the thickness of an
ordinary captain's biscuit. The pans must be well buttered. Bake in
rather a quick oven for half an hour. These cakes or biscuits may be
eaten by the diabetic with meat or cheese for breakfast, dinner, or
supper; at tea they require rather a free allowance of butter, or they
may be eaten with curd or any soft cheese.]

Where extreme restriction of diet is not required the ordinary bran
bread of the bakers may be used. The unbolted flour of which this is
made of course contains the starchy principles, but in consequence of
the retention of the bran the proportion of starch is less. The
cold-blast flour of the Health Food Company is said to contain the
nutritious, but not the innutritious, parts of the bran.[55]

[Footnote 55: It is made by pulverizing the carefully cleaned wheat by
a compressed, cold air blast, which strikes the wheat and dashes it to
atoms.]

The almond food suggested by Pavy is another substitute for bread. The
almond is composed of 54 per cent. of oil, 24 per cent. of nitrogenized
matter known as emulsin, 6 per cent. of sugar, and 3 per cent. of gum,
but no starch enters into its composition. Theoretically, therefore,
the food should be everything that can be desired if the gum and sugar
can be removed. The latter is done by treating the powdered almonds
with boiling water slightly acidulated with tartaric acid, or soaking
the almonds in a boiling acidulated liquid which may form a part of the
process for blanching. The boiling and acid are necessary to
precipitate {224} the emulsin, which would otherwise emulsify the oil
of the almond. Pavy speaks well of biscuit made of almond flour and
eggs, which he says go very well with a little sherry or other wine,
although he admits they are found too rich by some for ordinary
consumption. One person only under my observation has used the almond
food, and found it unpalatable.

Seegen recommends an almond food made as follows: Beat a quarter of a
pound of blanched sweet almonds in a stone mortar for about
three-quarters of an hour, making the flour as fine as possible; put
the flour thus obtained into a linen bag, which is then immersed for an
hour and a quarter in boiling water acidulated with a few drops of
vinegar. The mass is thoroughly mixed with three ounces of butter and
two eggs; the yolks of three eggs and a little salt are added, and the
whole is to be stirred briskly for a long time. A fine froth made by
beating the white of the three eggs is added. The whole paste is now
put into a form smeared with melted butter and baked by a gentle fire.

Biscuits made of inulin, the starchy principle largely contained in
Iceland moss, were suggested by Kuelz. Although a starch, it is one of
the assimilable ones alluded to, of which small quantities at least may
be taken as food without appearing in the urine as sugar. The biscuits
are made with the addition of milk, eggs, and salt, and are
inexpensive.

To some persons sugar is almost as imperative a necessity as bread,
although to many it is not a very great sacrifice to omit it from
ordinary cooking, if not from tea and coffee. For the latter it is just
as well to dispense with sugar altogether. But where patients feel that
they must have some substitute for sugar, glycerin has been suggested
for this purpose, at least for sweetening tea and coffee. But Pavy has
noted[56] that under the use of glycerin the urine increased from three
and three and three-fourth pints to between five and six pints, and the
sugar from 1100 grains to 3000 grains per diem, in the course of three
days. Its withdrawal was followed by a prompt fall in both the urine
and sugar, a return to it by a second increase, and subsequent
withdrawal by another decline. Along with the increase of urine and
sugar came also more thirst and discomfort. An examination of the
chemical composition of glycerin would seem to confirm these results of
experience. Glycerin is represented by C_{3}H_{8}O_{3}, sugar by
C_{6}H_{12}O_{3}, and glycogen by C_{6}H_{10}O_{5}; whence it is
evident that a conversion of glycerin into sugar may take place in the
liver. These facts seem to show conclusively that glycerin is no
suitable substitute for sugar. I therefore do not use it.

[Footnote 56: _On Diabetes_, London, 1869, p. 259.]

From what has been said it may be inferred that sugar of milk, mannite,
and lævulose, or fruit-sugar, are admissible where sugar is demanded.
They may be tried, but the urine should be carefully examined under
their use, and if glycosuria occur or be increased they should be
promptly omitted.

Almost every purpose of sugar in the cooking of acid vegetables is
served by bicarbonate of sodium or potassium. As much bicarbonate of
potassium to the pound as will lie upon a quarter of a dollar will
neutralize the acidity of most fruits which require a large amount of
sugar to mask this property. In this manner cranberries, plums,
cherries, gooseberries, red currants, strawberries, apples, peaches,
and indeed {225} all fruits to which sugar is usually added in the
cooking, become available to the diabetic.

In the matter of drinks, where the patient is not on a skim-milk diet,
which usually affords as much liquid as is required by the economy,
little restraint need be placed upon the consumption of water, which is
demanded to replace that secreted with the sugar. Instead of water,
Apollinaris water, Vichy, or the ordinary carbonated water may be used
if preferred, and to many they are much more refreshing by reason of
the carbonic acid they hold in suspension. Apollinaris water is
particularly so, and one of my patients, who recovered completely under
a suitable selected diet with which this mineral water was permitted,
insists that it was that which cured her.

Where a simple selected diet is adopted, tea and coffee without sugar
are usually permitted. The propriety of the substitutes for sugar
already referred to must be determined by circumstances.

Of distilled and fermented liquors, moderate quantities of whiskey and
brandy, dry sherry and madeira, the acid German and French wines--in
fact, any non-saccharine wines--may be permitted. A medical friend who
reports himself about cured of diabetes writes me that he has consumed
eighty gallons of Rhine wine since he began to adhere closely to a
diabetic diet. On the other hand, the free use of the stronger
alcoholic drinks has been charged with causing diabetes, and I have
known such use to produce a recurrence of sugar. No malt liquors,
except those in which the sugar has been completely converted into
carbonic acid and alcohol, should be used. Bass's ale may be allowed
where no especial stringency is required.

HYGIENIC TREATMENT.--The patient should be surrounded by the most
favorable hygienic influences. He should sleep in well-ventilated
rooms; pass much time in the open air; bathe regularly, but not in
water that is very cold, and especially the body should not be long
submerged in cold water, as the liver must share the general internal
hyperæmia incident to prolonged cooling of the skin, and increased
glycosuria may result. I have known sugar to reappear after a prolonged
drenching of the skin of patients overtaken by a rainstorm. Perhaps the
most suitable time for the hot or tepid bath is on retiring in winter,
but in summer it may be taken on rising. Thorough friction of the
entire body should be practised after the bath or independently of it.
An ounce or two of sodium carbonate may be added to it with advantage,
as it softens the skin and facilitates the removal of the effete
epithelium. The bowels should be kept regularly open, as the effect of
their confinement is to produce torpor and congestion of the liver.

Certain natural mineral waters have always enjoyed a reputation for the
cure of diabetes, and notably those of Vichy and Carlsbad. The former
is an alkaline water with a slight laxative tendency, and the latter a
decided aperient alkaline-saline water; and it is not unlikely that
they owe a part of their good effects to an action upon the liver and
upper bowel. This seems the more likely because Carlsbad, which enjoys
the highest reputation, contains a far larger proportion of chlorides
and sulphates, which are purgative. Vichy water contains 35 grains of
carbonates to the pint, and Carlsbad 11, but the latter contains twice
the proportion of chlorides, or 8 grains to the pint, and ten times as
much sodium {226} sulphate, or 19 grains to the pint. They may be used
as adjuvants to the treatment, a pint of Vichy or half as much Carlsbad
in the morning. Being imported waters, they are comparatively
expensive, and I know of no American waters which closely approach them
in composition.

Of American waters, the Saratoga Vichy contains twice as much chlorides
as the Carlsbad, 17.7 grains to the pint, but no sulphates. It contains
about the same amount of carbonates as Vichy. It is therefore a
saline-alkaline water, and may be expected to serve the purposes of
Vichy and some of those of Carlsbad, for which it may be substituted.
Most of the American mineral waters vaunted as useful in diabetes will
be found, on comparison with these waters, to be chemically
indifferent, and therefore about as useful as so much ordinary
spring-water. Of the Crab Orchard Springs in Kentucky, the Sowder's
spring contains 25 grains of sulphate of sodium and magnesium and 7
grains of sodium chloride to the pint, therefore about the same
proportion of the two substances combined as Carlsbad; yet I am not
aware that these waters have any reputation in diabetes. The waters of
Bedford Springs, Pennsylvania, also approximate them in the proportion
of sulphates of sodium and magnesium.

Other Saratoga waters have an undoubted action on the liver through
their chlorides, and may be used in lieu of the European waters above
referred to, and of the Saratoga Vichy, when these cannot be obtained;
such are the Geyser spring, which contains 70 grains of chlorides to
the pint, and the Hathorn, containing 63 grains.

MEDICINAL TREATMENT.--While the dietetic treatment, and especially the
skim-milk treatment, of diabetes mellitus is much to be preferred for
its results over an exclusively medicinal treatment, and is of itself
sufficient to control, if not to cure, a large number of cases, yet
instances arise in which it is insufficient to complete the removal of
sugar from the urine, and there are others in which it is impossible
for various causes to carry out such treatment.

In my book on _Bright's Disease and Diabetes_, published three years
ago, I gave the preference of drugs to ergot; but since then extended
opportunities have convinced me that codeia is a far more efficient
remedy. Repeated comparative trials of this drug in the wards of the
Philadelphia Hospital and elsewhere have satisfied me of this. The
trials have been made while the patients were upon a mixed diet, which
I hold to be the only fair way of arriving at a knowledge of the true
value of a drug in the disease. Codeia was first suggested by Pavy in
lieu of opium and morphia, which had long been used, his reason being
that it did not produce the same narcotic effect. Favorable reports
upon its use have been made by Foster, Image, Brunton, R. Shingleton
Smith, Cavafy, Austin Flint, Sr., Harvey L. Byrd, and others. It may be
given in pill or solution. One should begin with ¼ of a grain three
times a day, increasing ¼ of a grain daily until the sugar disappears
or the remedy ceases to have any effect, or until drowsiness is
produced. Thus gradually increasing, I have reached as high as 47
grains in a day. Cavafy has given 15 grains three times daily.

Opium--which is said to have been used by Aetius for this disease--or
morphia might be used if codeia cannot be obtained, but they are less
efficient, more dangerous, and more apt to produce the troublesome
{227} symptom of constipation. MacGregor[57] gave in one case 60 grains
of opium, and in another 90 grains, in the twenty-four hours.

[Footnote 57: _London Medical Gazette_, 1837.]

While I have seen the most striking results upon the quantity both of
sugar and urine during the administration of codeia, and at the same
time have noted a gain in flesh and strength, I cannot say that I have
ever seen a case totally recover under its use. Such cases are,
however, reported by others. I have always used it in the very worst
cases, where dietetic measures had also failed to remove the sugar. As
to the mode of action of codeine, we can only speculate. It may be said
that it quiets the irritation of the vaso-motor centre, whence result
the glycosuria and other symptoms of diabetes.

Next to codeine in efficiency, of drugs, is ergot. The favorable
results of its use are more easily explained by its physiological
action--contraction upon the muscular walls of blood-vessels--than
those of codeine, but it is not so efficient a remedy. It may be used
by beginning with half a drachm, and increasing to a drachm, four times
a day. Larger doses than this, as much as half an ounce four times a
day, have been given, but the stomach rarely permits their continuation
for any length of time.

Bromide of potassium, an old remedy for diabetes, has recently been
revived and much lauded by the French physicians, but I have never
found any results from its use. I can understand, however, how in
certain cases of nervous origin it may be useful.

Comparatively recently, Clemens of Frankfort-on-the-Main has
recommended the use of what he terms brom-arsen or bromide of arsenic.
The dose is one-forty-eighth of a grain three times a day, gradually
increased by this same amount until one-sixth or one-fifth of a grain
is given daily.[58] Clemens, however, unites with its use a dietetic
treatment. I have used it in connection with an unselected diet, and
have not found the results claimed by Clemens. It is, however, both
tonic and sedative, and as such is to be recommended in conjunction
with other measures.

[Footnote 58: The late Mr. R. F. Fairthorne, with Mr. James T. Shinn,
apothecary, cor. Broad and Spruce streets, Philadelphia, prepared for
me a solution of bromide of arsenic in the following manner: 77 grains
of metallic arsenic in powder are added in small portions to 240 grains
of bromine, the latter being placed in a long test-tube immersed in
ice-water to control the otherwise violent reaction. One hundred grains
of the tribromide thus obtained are dissolved in sufficient distilled
water to make ten fluidounces. One minim will then contain
one-forty-eighth of a grain.]

Arsenic itself has some reputation in the treatment of diabetes, based
upon the observation of Salkowsky that glycogen diminishes in the
livers of animals poisoned with arsenic. It is at least a good adjuvant
tonic. Leube gave it in diabetes in doses of one-third of a grain three
times a day.

Strychnia is also very useful as a tonic, and may be used either alone
or in the shape of the sulphate, or combined with arsenic and iron, or
it may be given, perhaps preferably, in solution in combination with an
acid. Given in combination with phosphoric acid, I believe it the most
valuable tonic available in this disease.

To supply the phosphates, in which gluten bread is deficient, as well
as for their tonic effect, the various preparations of phosphates are
useful. {228} The well-known compound syrup of the phosphates, or
Parrish's chemical food, may be considered a type of these
preparations. Every fluidrachm, which is a dose, contains 2½ grains of
calcium phosphate, 1 grain of ferric phosphate, with fractions of a
grain of sodium and potassium phosphate with free phosphoric acid.
Similar is the solution of phosphates and phosphoric acid[59] known in
this country as solution of phosphoric acid with iron, or the latter
may be omitted.

[Footnote 59: Rx. Calcii phosphat. gr. iij;
                  Magnesii   "     gr. ss;
                  Potassii   "     gr. iv;
                  Ferri      "     gr. ss;
                  Ac. phosphoric   minim vj;
                  Aquæ q. s. ad.   fluidrachm i, which is a dose.]

Iodide of potassium has been used in some cases with satisfactory
results, and may be expected to be useful where syphilitic disease of
the nervous system is suspected.

Seegen has seen sugar disappear from the urine under a dosage of 20 to
30 drops of tincture of iodine daily, but the sugar reappeared after
the remedy was discontinued.

Lactic acid was recommended by Cantani on theoretical grounds as a
substitute for sugar. He supposes that in health the sugar ingested is
converted by the liver into lactic acid, and he would furnish the
latter already formed, and thus spare the liver this function. Senator
also favors the use of this acid for a similar purpose, but reasons
that in health sugar is converted into lactic acid in the small
intestine, while in diabetes this conversion is interfered with. Hence,
too, it should be given fully formed. Patients under its use are said
to gain in weight and to become stronger, while it is not claimed that
it alone diminishes the glycosuria; this must be brought about by a
selected diet. The lactic acid is simply an important force-producer
not otherwise obtainable, because sugar fails to undergo its usual
conversion. Cantani recommends that from 75 to 150 grains of the acid
should be taken daily in from 8 to 10 fluidounces of water. Diarrhoea
and pains in the joints are said to follow the use of large quantities
of the drug, but these again disappear on its omission. My experience
is limited to a single case, which recovered while taking 30 drops
three times a day in conjunction with Carlsbad water and a pill of
iron, quinia, and arsenic.

Senator suggested that the fatty acids--oleic, palmitic, stearic, and
butyric--be used on the same principle that lactic acid is given, that
their force-producing power may be availed of. To this end he
prescribed, with partially satisfactory results, soap in pills
containing 2-1/3 grains each, of which four were taken daily.

Cod-liver oil is especially suitable as a food where debility is to be
combated. Even those who claim that fats are convertible into sugar in
the liver admit that it is only in the most advanced stages of diabetes
that such conversion takes place. Cod-liver oil, therefore, in common
with other fats, may form part of a diabetic diet, and is especially
indicated where phthisis is present, as it so often is, in the latter
stages of the disease, or indeed whenever a good tonic is indicated.

In 1882, Moleschott[60] suggested the use of iodoform in diabetes. He
reported the effect of its use in five cases, giving .1 to .3 grm. (1.5
to {229} 4.5 grs.) in pill with extract of lactucarium and cumarin, the
purpose of the latter being to disguise the odor. His formula was as
follows: Iodoform, 1 gram (15 grs.); ext. lactuc. sat., .1 gram (15
grs.); cumarin, .1 gram (1.5 grs.), to be made into twenty pills. In
one case the sugar disappeared in twelve days; in the second, at the
end of six months; in the third case it had diminished from 14.4 to 1.6
grams in three months; in the fourth, from 28 grams to 1.6 in four
months; and in the fifth case, from 9.2 to 6.1 grams.

[Footnote 60: _Wiener Med. Wochenschr._, Nos. 17, 18, 19.]

The use of the remedy in Moleschott's hands produced no unpleasant
results, but Drasch,[61] who used the same treatment after Moleschott's
method in three cases, with the effect of diminishing the thirst, the
quantity of urine, and the proportion of sugar, found excessive itching
of the skin, diminished appetite, and diarrhoea to result in such
degree as to demand its disuse in the majority of cases. Iodoform has
been used by the Italian physicians De Renzi,[62] Bozzolo,[63] and
Silvestrini,[64] and by Sara E. Post[65] of New York, with varying but
generally favorable results, except in Silvestrini's case. These
results included diminution in thirst, quantity of sugar and urea, with
increase in weight. The drug deserves a trial in doses of from 1 to 2
grams (15 to 30 grains) a day, but due regard should be had to possible
toxic effects; and to this end the administration should be interrupted
at the end of one or two weeks, and the interruption continued for a
like period. It may be given in pill or in capsule, and in divided
doses or in a single dose at bedtime. The latter course is recommended
by Post, and is said to avoid eructations and anorexia. Theories of its
action based upon experimental use of poisonous doses ascribe its
effect to a primary stimulating and ultimately fatally degenerative
effect upon the protoplasm of cells, and especially those of the liver
and nervous system.

[Footnote 61: _Wiener Med. Presse_, 1882, xxiii. 1487.]

[Footnote 62: "Tre Storie di Diabete." _Gior. internaz. d. sc. med._,
Nap., 1882, N. S. iv. 913-917.]

[Footnote 63: "Sur l'action du iodoforme dans la diabète sucre," _Arch.
ital. de biol._, Turin, Feb., 1883, iii. 317-321.]

[Footnote 64: "Iodoforme dans le diabète," _La France Méd._, October,
1883, ii. 567.]

[Footnote 65: _Archives of Medicine_, April, 1884, p. 116.]

Transfusion of blood has been recommended by Dieulafoy,[66] and is
approved of by Ralfe,[67] especially to combat the symptoms of
acetonæmia, which, if due to a toxic agent, as seems most likely,
should be met by altering the percentage composition of the blood with
relation to the toxic agent.

[Footnote 66: "Étude sur la Transfusion du Sang dans le diabète sucre,"
_Bullétin et Mém. Soc. Méd. de Hôp. de Paris_, 1884, 4, S. 1, 38, 41.]

[Footnote 67: "Discussion before the Path. Soc. of London," _Lancet_,
Apr. 7, 1883, p. 592.]

Diabetic neuralgia yields generally to the treatment of the disease in
general correspondingly to the reduction in the quantity of sugar, and
at times to salicylate of sodium, while it does not respond to morphia
or other remedies for ordinary neuralgia.

The alkalies, which attained some reputation after Mialhe claimed for
them the power of destroying sugar in the blood and of neutralizing the
fatty acids which were thought to accumulate there in consequence of
the deficient action of the skin, are not often used at the present
day. Potassium carbonate was the favorite preparation, and in the hands
of Pavy its use seems to have been followed by good results. He gave it
in 10, 15, and 20 grain doses in combination with aromatic spirit of
{230} ammonia. Sodium bicarbonate was less satisfactory, as were also
potassium acetate, potassium citrate, and Rochelle salts. These were
given in doses of from four drachms to an ounce daily. In Germany, too,
the alkaline treatment has been used to some extent.

As is the case with so many diseases which are incurable by any special
treatment, a large number of remedies have at different times been
suggested for diabetes, mostly on a foundation which does not admit of
close analysis. One of these was the nitrate of uranium, suggested by
Dale of Lemont, Pennsylvania, who gave it in doses of 1 grain three
times daily, increased to 3 if necessary, in pill, powder, or solution,
by aid of a few drops of nitric acid. He appears, however, to have used
it in connection with a selected diet. I have tried it both with and
without a selected diet; in the latter case there was no effect, and in
the former there was none which the diet alone would not have produced.

Sodium phosphate, salicylic acid, salicylate of sodium, have all been
used, it is claimed, with good results, and the late Dr. Dougherty of
Newark, New Jersey, used with apparent advantage a mixture into which
all of these, together with sodium carbonate, entered, made up with
glycerin, tincture of cardamom, and water, the doses being 2½, 2½, 4½,
and 8½ grains respectively. Moleschott has also obtained good results
with salicylic acid.



{231}

SCROFULA.

BY JOHN S. LYNCH, M.D.


SYNONYMS.--Scrophula, Scrofulosis, Morbus scrophulosus, Struma, King's
evil, The evil, Quince, Cruels and Crewels (Scotice).

DEFINITION.--A morbid condition of the system manifested by a peculiar
liability to certain forms of nutritive disorders of the skin, mucous
membranes, joints, bones, organs of special sense, and especially the
lymphatic glands.

There is probably no disease of which it is more difficult to give an
exact and satisfactory definition than scrofula. The general tendency
of medical opinion within the last few decades has been to narrow the
significance of the term, and even to restrict it to those slow and
indolent inflammations and over-growths of lymphatic tissue which end
in caseation and finally imperfect suppuration. Formerly almost every
deviation from healthy functional activity in the young, as well as
every disorder of nutrition which could not be assigned to any definite
cause, was called struma; and thus, as Heule well remarks,[1] "Scrofula
became the receptacle into which one vaguely casts all the ailments
which afflict children under fourteen years, and of which we do not
know the cause."

[Footnote 1: _Handbuch der Rationellen Pathologie_.]

Before hereditary syphilis was understood all its manifestations were
classed as scrofulous, and at least one eminent authority in the United
States[2] has expressed the opinion that scrofula is only a
manifestation of the syphilitic poison in the second or third
generation. Rickets, chronic hydrocephalus, favus, lice, and worms
(Lugol), diabetes (Carmichael), and even scirrhus and cancer (R.
Hamilton), have all been classed as scrofulous diseases. Then there is
a large class of unhealthy persons whose morbid state can be no more
definitely expressed than by saying that they are "delicate" or of
"feeble health" or of "frail constitution," and by some all these are
included under the term scrofulous. But as knowledge advances, and
pathological knowledge as well as diagnostic acumen becomes larger and
keener, many of these affections and morbid conditions can be
eliminated from scrofula and assigned their true pathological and
nosological position.

[Footnote 2: S. D. Gross, _Transactions American Medical Association_,
1878.]

To many who have been educated in the more modern schools of medical
thought, therefore, our definition will appear much too broad, while to
others it may appear too narrow.

Scrofula is essentially and purely a diathetic, not a cachectic,
disease. {232} It is true that what may be called the manifestations or
lesions of the disease are often excited by some preceding dyscrasia,
and also that the long persistence of these lesions may excite a
cachectic condition which we might call the scrofulous cachexia; still,
as many children suffer from the lesions of scrofula who have never
exhibited any evidence of a precedent dyscrasia, but on the contrary
appear to be in perfect health, and many others, on the other hand,
show unmistakable evidence of ill-health and are decidedly dyscrasic,
yet are never attacked by scrofula, it is believed that every subject
of scrofula becomes so not because of any pre-existing dyscrasia or
cachexia, but because of some peculiar condition of the system--innate
or acquired--which constitutes a diathesis.

"The hypothesis," says Niemeyer,[3] "that scrofula depends upon a
faulty composition of the blood (dyscrasia), and that the lesions found
in scrofulous persons were due to a deposit in the tissues of a matter
circulated by the blood and called a scrofulous material, is almost
universally abandoned."

[Footnote 3: _Textbook of Practical Medicine_, vol. ii.]

But while insisting upon the peculiar and, so to say, specific origin
of the disease in some special condition of the system, without which
it will never exist, it is admitted that the lesions of scrofula do not
differ essentially from other similar lesions of the same tissues of a
non-scrofulous origin. They are mostly of an inflammatory nature, and
are only to be distinguished by the often trivial character of their
exciting causes--often, indeed, by the total absence of any known
exciting cause--and by their tediousness and intractability.

ETIOLOGY.--We believe, as already stated above, that the essential
cause of scrofula is some peculiarity in the constitution of the
tissues of the scrofulous subject; and we think it highly probable that
H. F. Formad of Philadelphia has pointed out what constitutes this
peculiarity. He declares--and the correctness of his observation has
been abundantly verified--that microscopic examination of the tissues
of certain animals characterized by their extreme aptness to be
affected by scrofula and tuberculosis, as well as of children known to
have been scrofulous or tuberculous, discloses the fact that the
lymph-spaces in these subjects are always more numerous, larger, and
more crowded with cells than in non-scrofulous subjects. The tissues of
the scrofulous are therefore coarser, less compact and solid, and there
is a greater tendency to undue cell-growth, than in the non-scrofulous.
And these are precisely the characteristics which they present
clinically, and such as we might have, a priori, expected to find.

This peculiarity of anatomical structure is in a large number of cases
undoubtedly inherited from the parents, but while heredity plays, as is
well known, an important part in the etiology of struma, it is not the
essential factor. Bad hygienic surroundings, overcrowding, and
consequent want of fresh air, improper food, consisting of a too great
proportion of starch, during the early months or years of life, will
cause the growing tissues to assume the peculiar anatomical arrangement
alluded to above. "A coarse diet, containing but little nourishment in
comparison with its bulk, is especially held in evil repute. The
earlier this injudicious feeding of an infant commences, so much the
greater danger that it will become scrofulous; hence the children fed
on pap furnish a very important contingent to the army of scrofulous
persons."[4] The {233} well-known fact that few children at the breast
suffer from scrofulous lesions, but that a large number do so within
the first two or three years after weaning, certainly tells in favor of
the belief that too much starch and an insufficiency of animal food
favor, if they do not actually produce, that faulty nutrition and
construction of tissues which we believe lies at the foundation of the
scrofulous diathesis. Independent, however, of improper food and the
other predisposing causes mentioned, it is quite probable that faulty
nutrition caused by accidental disease of the digestive or assimilative
organs during infancy may create a predisposition. How else can we
account for those not very rare cases in which from parents perfectly
free from any scrofulous taint a large family of children may be
reared, of which only one will suffer from any scrofulous lesions? Two
such instances have been brought to my notice, and as the children in
these cases lived upon a farm on the water-side, and enjoyed an
abundance of pure air and salt-water bathing, and were certainly not
stinted in food of proper quality, it is difficult to account for the
acquired diathesis except upon the hypothesis above. Among the general
predisposing causes of scrofula in addition to the special ones I have
mentioned may be added--

[Footnote 4: Niemeyer, _loc. cit._]

1. Locality and Climate.--It has long been believed that scrofula is
more common in the temperate zone than in the extreme north or in the
tropics. While this is probably true, it must be stated that a
sufficient amount of reliable statistics bearing upon this point have
not yet been collected to prove the fact beyond cavil. That we should
find that the disease prevails more extensively in cold and damp
situations than in warmer and drier ones is to be expected, since the
former conditions involve a greater confinement within dwellings, and
consequently a diminished supply of fresh air, which, as we have seen,
constitutes one of the predisposing causes of scrofula. Moreover, it is
in these situations we would encounter a greater number of catarrhs,
which, as we shall see, are known to be among the most active of the
exciting causes of the glandular affections of scrofula.

2. Season.--For the same reason we find that a large number of cases of
scrofula make their appearance in the early spring months, the results
of catarrh contracted during the previous winter or of the sudden
changes of temperature which accompany the transition of winter to
summer.

3. Age.--Scrofula is essentially a disease of early life, but not
exclusively so. As the diathesis can only be acquired directly from the
parent, or fortuitously by malnutrition during the period of active
growth, it follows that it becomes established, if at all, before the
age of twenty years. And as the predisposition seems to be quite strong
in most cases, and as the exciting causes are more apt to be applied
during the earlier years of life, it is not surprising that a very
large majority of the cases occur between the ages of three and fifteen
years. A few, however, escape during childhood, and only suffer from it
between twenty and thirty, while a small number only develop the
disease in old age. Rindfleisch mentions the period between twenty and
thirty as a common one for the development of hereditary scrofula; and
senile scrofula was first pointed out by Sir James Paget.[5] In all
these cases of deferred {234} manifestation of the scrofulous
diathesis--and they are not very numerous--it is to be presumed that
they have escaped the most active of the exciting causes of the
disease. Indeed, it is natural that a person having inherited the
predisposition should be more sedulously guarded--at first by his
parents, and afterward by himself--against the exciting causes of
scrofula during infancy and adolescence.

[Footnote 5: _Clinical Lectures and Essays_, London, 1875.]

4. Sex.--There is no reason to believe that sex plays any part in the
predisposition to this disease. Both sexes seem to be affected in about
equal proportions, but from the statistics bearing upon this point it
does seem to have some influence in determining the variety of its
lesions. Thus, females seem to be more frequently affected with
glandular disease, while males suffer from diseases of the joints in
the form of coxalgia, white swelling of the knee, and Pott's disease.

5. Condition in Life: Social Position.--If what we have said about the
predisposing influence of improper or insufficient food, overcrowding,
etc. be true, it will naturally be inferred that a large proportion of
the cases of scrofula will be found in the lower strata of society; and
this is true. Especially in cities, where the disease prevails most
extensively, we always find that the denizens of narrow streets, lanes,
and alleys furnish the largest contingent to the deaths as well as the
deformities from scrofula. It is here that the poor congregate to avail
themselves of the cheaper rents, and here will be found combined all
those predisposing causes which may be briefly summed up in one
word--poverty. It is true that cases of scrofula are quite numerous in
the country, and in a note to Sir Thomas Watson's _Practice of Physic_
(1851) D. Francis Condie quotes from a work on _The Nature and Causes
of Scrofula_, by Phillips, statistics which showed a greater
preponderance of deaths from scrofula in a given number of the rural
population than a nearly equal urban one. But at the time these
statistics were gathered in England (and perhaps now) it is probable
that there was a comparatively greater number of abjectly poor people
among the rural population than in London, where was congregated such a
large number of small tradesmen, artisans, and laborers, who, though
not well-to-do, were better paid, and consequently lived better, than
the agricultural laborers. Of course, a certain number of cases of
scrofula are found in the United States, and perhaps in all other
countries, among the children of the wealthy. These, however, are
almost invariably caused either by direct transmission from parents or
by some accidental injury to the digestive and assimilative organs in
early childhood, as we have already pointed out. When it is remembered
that in the constantly changing fortunes which are so frequently
witnessed in this age of excessive activities, and that in the grand
opportunities for obtaining wealth furnished by the liberal
institutions and rapidly-growing industries of the United States the
descendant of the pauper of the last generation may be the millionaire
of the present, it is not surprising that so many who are now wealthy
may possess the strumous diathesis as an inheritance from their parents
or grandparents, and which they in turn transmit to their offspring.

6. Consanguineous Marriage.--It has long been a popular belief that the
offspring of parents closely related by blood are more apt to be
scrofulous than when no such relation has existed. Indeed, not only
scrofula, but numerous other diseases, deformities, and imperfections
have been {235} ascribed to such unions. Idiocy or feeble-mindedness
has also been especially accredited to the production of such
marriages. But a thorough investigation of this point in England some
years ago demonstrated positively that no more idiotic, feeble-minded,
or insane children are born of such marriages than of an equal number
of marriages contracted between persons not related by blood to each
other. There is, however, this amount of truth in the popular belief:
if persons closely related to each other possess the scrofulous
diathesis, there will be a greater probability--almost certainty--that
the diathesis will be transmitted to their offspring. If one parent
only is tainted with scrofula, and the other is entirely free from it,
there is a possibility--even a probability--that some or all of the
children may escape.

7. Complexion and Temperament.--It has been stated by some observers
that scrofula occurred principally in the fair-haired, and with equal
positiveness by others that it was in the dark-haired that the disease
found the most of its victims. Such statistics as have been furnished,
however, upon this subject seem to show that there is no connection
whatever between scrofula and complexion. It will generally be found
that whenever in any country or locality more cases of scrofula occur
in persons of one or the other of the complexions, it is only because
that particular complexion is the predominant type among the
inhabitants of that locality.

8. Race and Nationality.--While it would seem that no race or nation is
entirely free from struma, yet there are certainly in the United States
two peoples who furnish an enormously disproportionate number of
scrofulous cases: these are the Irish and Jews. Among the first of
these both scrofula and tuberculosis abound with exceeding frequency,
while among the latter it is scrofula alone which seems to predominate.
The last, however, are not exempt from tuberculosis, but only exhibit
about an equal predisposition to it with their fellow-citizens. It is
not difficult to explain the special predisposition of these peoples to
scrofula when their past history is taken into account in connection
with what has been said about the bad influence of food and
surroundings in producing the scrofulous diathesis. The principal food
of the Irish peasantry--oppressed and ground into poverty by their
Anglo-Saxon conquerors for hundreds of years--have been bread and
potatoes, often potatoes alone. It cannot be surprising, therefore,
that Irish children fed upon this diet and reared in ill-ventilated
hovels should develop the scrofulous diathesis in legions. The Jews,
too, oppressed by all nations through ages, have been during many
generations reared in poverty and squalor. Even those of them who in
not very remote times had acquired by thrift the means of securing both
the comforts and luxuries of life dared not live according to their
means, lest a show of wealth should attract the unpleasant, often
fatal, attention of their rapacious and unscrupulous Christian or
Mohammedan neighbors. This condition, this mode of life, has existed
among them for many hundreds of years, and has so intensified the
strumous diathesis among them that almost the whole race may be said to
be patently or latently scrofulous. The negro or African race, however,
as observed by the writer in the Southern States of the American Union,
do not seem to have developed any special predisposition to struma,
notwithstanding their servile condition. This, at first sight, would
seem to {236} be contrary to our expectation based on what has been
said about Jews and Irishmen. But as my remark has been predicated only
on observation of the African in the Southern States, where the climate
is not favorable for the development of scrofula, the fact is not so
surprising. Besides, the food of these people consisted largely of
bacon or pork, fish, milk, and the succulent fruits and vegetables,
with a moderate quantity of corn bread, and very rarely potatoes. As
the rude cabins in which they dwelt were usually constructed of unhewn
logs and covered with rough boards, and cost almost nothing except
labor, overcrowding was unknown and ventilation always perfect. The
waiter practised medicine fourteen years in Wilcox county (S. W.),
Alabama, containing a population in 1870 of 28,377, of whom 21,610 were
colored, and during this time saw only two cases of genuine scrofula
and one of tuberculosis among the colored population.

Pork as an article of food has often been accused of producing a
tendency to scrofula, but evidently with great injustice, for we have
seen that the Jews, who never eat it, are almost universally
scrofulous, while the Southern negroes, whose staple animal food it
was, were conspicuously free from it.

9. Acquired Scrofula.--Although in perhaps a majority of all scrofulous
cases the diathesis has been inherited from the parents, the fact
cannot be too strongly emphasized that in a large number of cases the
disease may be developed de novo, independent of such heredity. To
scrofula developed from the influence of bad ventilation and
overcrowding, absence of sunlight, insufficient, bad, or unsuitable
food, cold and damp, imperfect clothing--in short, all those conditions
associated with poverty, squalor, and ignorance--Grancher has well
applied the term la scrofula a miseria. And it is only by a clear
comprehension that scrofula may be, and often is, developed under these
conditions that the medical profession in general, and municipal health
authorities in particular, may be induced to teach and enforce upon the
poor both the knowledge and the practice which may prevent it. Even in
the open country, where there is at least no lack of pure air and
light, the lesson can be enforced with equal profit; for the children
of the farm-laborer are likely to be imperfectly and improperly fed,
and lodged in apartments at night that in the matter of foul air and
filth could not be well surpassed in the purlieus of the dirtiest and
most overcrowded city.

EXCITING CAUSES.--The actual exciting causes of scrofula when the
diathesis already exists are too numerous to be mentioned in detail.
Indeed, almost any trivial injury or inflammation, any disease which
has produced a temporary cachexia, may rouse into activity the perhaps
hitherto latent tendency. How often do we see a slight blow upon the
knee-joint produce a white swelling which lames for life the heretofore
healthy and active boy or girl! A fall upon the hip which was almost
unnoticed at the time excites a coxalgia which either destroys life or
renders the child for life a cripple; or a slight jar of the spine
induces a disease of the vertebræ which, if recovered from at all,
produces a terrible deformity. A slight eczema of the face or scalp or
a catarrh of the mouth or throat will excite that slow and generally
painless enlargement and induration of a neighboring lymphatic gland
which always ends in its caseation and final destruction by
suppuration. A slight injury to the {237} periosteum may excite a
destructive caries or necrosis of the underlying bone, and a temporary
catarrh of the intestines a tabes mesenterica with all its fatal
consequences.

Speaking generally, it may be said that anything that produces a local
disorder of nutrition or impairs the health generally of a person
predisposed to scrofula is sufficient to bring about some
manifestations of the disease. They are especially apt to follow the
eruptive fevers. Measles and scarlatina are very commonly arousers of
the scrofulous process, not only by the temporary impairment of health
which follows them, but also through the catarrhs which are usually
present in both diseases. Vaccination has often been accused of
imparting scrofula; and, although this is untrue, since scrofula cannot
be imparted in the sense of transference from one person to another,
there can be no doubt that the predisposition may be roused into
activity by the slight impairment of health associated with vaccination
or by the slight injury inflicted at the point of introduction of the
vaccinal virus. In some cases the disease has manifested itself for the
first time during pregnancy or lactation, and there is no doubt that in
cases where the disease has existed in childhood these conditions often
cause it to reappear. In conclusion, it must be said that many cases
apparently occur spontaneously--"the disease came on of itself"--or if
there are any exciting causes they were so trivial as to have escaped
notice altogether.

Finally, it must be remembered that the eczemas, catarrhs, ophthalmias,
otitis, chilblains, erysipelas, and numerous other local disorders of
nutrition which are often the causes of graver manifestations of the
disease, are themselves very prone to run a peculiar course
characterized by chronicity and intractableness; and many regard these
disorders as themselves manifestations of scrofula. Indeed, Virchow,
basing his argument upon the fact "that scrofulous enlargement of the
lymphatic glands of the neck often follows upon certain diseases
affecting the throat, such as mumps, diphtheria, and scarlet fever,
maintains that scrofulous proliferation of these glands, like ordinary
inflammatory hyperplasia of the same organs, is always secondary to
some peculiar process going on at the mucous surface or other part
which is in direct relation with them by means of the lymphatic
vessels; that scrofulous disease of the glands of the neck is traceable
to some inflammatory condition of the throat, fauces, or contiguous
parts; of the bronchial and mediastinal glands, to pulmonary or
bronchial inflammation; and of the mesenteric and retro-peritoneal
glands, to similar conditions of the alimentary canal. But he considers
that there may be some special element or quality in the primary
inflammation, and a tendency in its products to undergo rapid decay
similar to that which characterizes the morbid products of the diseased
lymphatic glands, but that generally they are not recognizable, from
the fact that in this case the cells are mostly developed at a free
surface, and are speedily shed from it." He admits, however, "that
there may be some special aptitude or weakness, congenital or acquired,
in the lymphatic glands of certain persons, or of certain parts of
them, which makes their inflammations, induced by indifferent causes,
assume the scrofulous character."[6] From the last of these
propositions no one will be likely to dissent, but that there is "some
specific quality or {238} element in the primary inflammation," etc.
few will be inclined to agree. There is nothing peculiar in the
eczemas, ophthalmias, catarrhs, etc. spoken of, except that they occur
in persons possessing the scrofulous diathesis; and it is this alone
that gives them their special characters, if there are any. In other
words, we cannot say that these disorders occur in certain children
because they are scrofulous, but that they are specially intractable on
account of the scrofulous diathesis upon which they are engrafted.
Millions of children have catarrh and ophthalmia (indeed, few escape
these disorders throughout the first ten or twelve years of life) who
never show any other evidence of the scrofulous taint; and nearly all
have measles and scarlatina, but it is only the scrofulous who usually
suffer severely from the secondary effects of these diseases. But they
do not have measles or scarlatina because they are scrofulous, and we
can with no more justice say that they have catarrhs or other
inflammations because they are so. We do not believe, therefore, that
strumous children have cutaneous and catarrhal inflammations simply
because they are strumous; and if we speak of scrofulous catarrh or
ophthalmia or eczema, we use the terms in the same sense as when we
would speak of a scrofulous measle, scarlatina, or whooping cough.

[Footnote 6: _The Theory and Practice of Medicine_, by Jno. Syer
Bristowe, M.D., 1879, pp. 80, 81.]

Although we have few reliable statistics bearing upon the question, it
can scarcely be doubted, judged by the results of casual observation,
that scrofula is much less frequent in America than in Europe, and that
in the latter there is less of it than formerly. The cheapness of land
in America has prevented that excessive overcrowding that exists in the
older and more densely populated countries, and the abundance and
cheapness of animal food has prevented that excessive feeding on bread
and potatoes which constitutes such an important factor in the
production of the scrofulous diathesis in some other countries.

PATHOLOGY AND MORBID ANATOMY.--The most important and central
anatomical and pathological facts both in the causation and progressive
development of struma, according to the writer's views, are--

1st. That faulty anatomical--or rather histological--construction of
the tissues of the scrofulous individual already alluded to as having
been first brought to the notice of the profession by Formad of
Philadelphia, which consist of an unusually large number of
lymph-spaces (which are also unusually large), and consequently an
excessive number of lymph-vessels and lymph-glands.

2d. Excessive production of rudimentary lymphoid cells, and probably
also of lymphatic tissue.

3d. Diminished and insufficient number of the capillary blood-vessels;
and, as a necessary consequence of these,

4th. Diminished nutritive activity of all those processes, both
physiological and pathological, which depend upon a full supply of
nutritive blood.

The most striking feature in all scrofulous inflammation is excessive
cell-growth, but these cells show little tendency to differentiation
and organization, probably for two reasons: 1st, because they are
derived from the blood-vessels principally, and not from proliferation
of the proper connective-tissue cells of the part; and 2d, because they
are insufficiently supplied with nutrition from the scanty
blood-vascular {239} network, and this supply is too rapidly absorbed
into the lymph-spaces, and is carried off by the too numerous
lymph-vessels. The cells, therefore, speedily perish, undergo partial
or imperfect fatty degeneration, and finally caseation, unless the
process is going on at a free surface, in which case, of course, they
are shed and thus gotten rid of.

Virchow some time ago called attention to the predominant cellular
character of the scrofulous exudation and the low vitality of the cells
which compose it. Rindfleisch declares that the fresh scrofulous
exudations contain relatively large cells with glistening protoplasm,
and that the white blood-corpuscles have a tendency in scrofulous
persons to grow larger on their way through the connective tissue. He
adds that they swell up by the imbibition of albuminous substances, and
by this very swelling die and slowly degenerate.

It seems to the writer, however, that it is probable that herein lies
the reason why swelling and apparent hyperplasia of the lymphatic
glands in the neighborhood of a local inflammation occurring in a
scrofulous person always takes place. The swollen cells become arrested
at the first gland they reach, and block the channels through the
gland. Successive additions of cells continue to block these channels,
and finally the passage of lymph through the gland becomes impossible,
and then begins that secondary increase of the lymph-cells in the gland
resulting from their inflammatory proliferation.

"In scrofulous inflammation," say Cornil and Ranvier,[7] "there is a
remarkable tendency to permanent infiltration of the affected tissue.
In simple inflammation (_i.e._ inflammation in non-scrofulous persons)
the infiltration is a temporary condition which terminates in
suppuration, in organization, or in resolution." Now, the several steps
in this process of resolution are--contraction of the distended
blood-vessels, thus cutting off the excessive supply of blood which has
caused the exudation and cell-proliferation; fatty degeneration of the
new cell-formation; liquefaction of this fat by union with the alkaline
blood-plasma, converting it into a dialyzable (saponaceous) liquid
which can now be readily absorbed by the veins. In scrofulous
infiltration the cells are speedily attacked by fatty degeneration
(which seems to be strictly a physiological process), but instead of
becoming liquefied, it (the fat) remains, slowly dries and hardens, and
finally becomes converted into the so-called cheesy mass or cheesy
infiltration. It does not liquefy, because it does not receive a
sufficiently abundant supply of the alkaline blood-plasma from the
scanty blood-vessels, and that which is supplied too rapidly flows into
the numerous large lymph-spaces and is carried off by the
lymph-vessels. In the case of the infiltrated gland the supply of this
plasma is cut off in both directions. The passage of lymph through the
gland is blocked, when, of course, none can then reach it through the
lymph-vessel leading to it, while the swelling of the gland itself from
accumulated cells compresses the neighboring nutrient vessels and cuts
off the supply from this direction also. Hence the speedy death, fatty
degeneration, and caseation (not liquefaction) of the cells.

[Footnote 7: Ed. 1880, p. 114.]

"The newly-formed material not only interrupts the lymph-passages of
the gland, but also compresses the blood-capillaries in such manner
that the circulation completely stagnates. It is impossible by any
{240} method of injection to penetrate into the most swollen parts of
the gland. With the supply of blood the nutrition also self-evidently
ceases; the gland falls into caseous degeneration. Where this enters in
the gray mass first becomes opaque, then whitish-yellow,
non-transparent, dry, friable. If the whole gland has passed into the
caseous condition, it appears upon a section as a fresh potato, only
not quite so moist, but just as homogenously yellowish-white."[8]

[Footnote 8: Rindfleisch, _Textbook of Pathological Histology_, 1870,
p. 202.]

The subsequent fate of these glands seems to depend somewhat upon their
situation. In the mesenteric and bronchial glands almost always the
caseous mass is attacked by calcareous infiltration, and finally dries
into a solid chalky concretion. The writer counted seven of these
chalky masses around the primary bronchi of a boy about fifteen years
old whose body was brought into a dissecting-room in Baltimore City.
But the more common result of the caseous process in the glands of the
neck is softening. "The caseous dépôt melts from within outward into a
whitish-yellow, whey-like fluid, which holds a fatty granular detritus
suspended in smaller or larger fragments. If all the caseous material
has softened, the neighborhood of the gland is wont to inflame; this
inflammation facilitates the way for the scrofulous pus outward. This
is evacuated, and we have the scrofulous ulcer, with its overhanging,
bluish, hyperæmic, flabby edges. At length this opening also closes,
and a drawn-in, radiated cicatrix marks the place where the evacuation
took place."[9]

[Footnote 9: _Ibid._, _loc. cit._]

But it must be borne in mind that all so-called scrofulous hyperplasias
of lymphatic glands do not run this destructive course. Undoubtedly, in
a few cases there remains a sufficient nutritive supply to carry on the
liquefactive process which normally follows fatty degeneration, and
thus resolution of the affected gland takes place. We are, however, of
the opinion that Virchow was mistaken when he asserted that complete
resolution of the cheesy material could take place; and from what we
know of the dangerous and usually fatal consequences of the absorption
of this cheesy detritus, Rindfleisch is certainly in error in
describing this as the most desirable possibility of decomposition.

We have heretofore purposely avoided any mention of tubercle or
tuberculosis as a part of the scrofulous process. In the views of many
physicians the relations between the two processes are so close that to
them tuberculosis and scrofulosis mean one and the same thing. While
medical opinion as to the true meaning of the word tubercle was so
discordant and unsettled, while so many products of diverse
pathological processes were included in that term, and while many,
following the view of Burdon-Sanderson of England, believed that
tubercle always takes its origin in small, even microscopic,
collections of lymphatic tissue, such a belief in the identity of the
two processes was not only possible, but reasonable. But since, by very
many good authorities, the term tubercle is now limited to the miliary
or submiliary tubercle, since numerous inoculation experiments have
shown that tuberculosis can be induced in non-scrofulous animals, and
Koch of Germany has proved that there exists in decaying tubercle a
peculiar and distinctive bacillus which even when cultivated out of the
body of a tuberculous person will excite tuberculosis also if
inoculated upon a non-scrofulous animal,--a belief in the identity of
the two diseases seems to be no longer tenable. Certainly, it {241}
would seem that to Sanderson's view that tubercle always takes its
origin in lymphatic tissue it is only necessary to reply that the
subjects of miliary tuberculosis do not more frequently than those
suffering from other non-scrofulous diseases present those larger
glandular hyperplasiæ which are so distinctly characteristic of
scrofula, and to which many persons limit the term scrofulosis. It may
be said, perhaps, that the converse of this is not true, and that
scrofulous persons are more frequently attacked by miliary tuberculosis
than an equal number of non-scrofulous persons. But the extreme
susceptibility or liability of the scrofulous to be attacked by
numerous and even diverse morbid processes, and the profound cachexias
and dyscrasias which the scrofulous processes engender, amply account
for the apparent susceptibility of the scrofulous to be attacked by
miliary tuberculosis. The strumous are more susceptible to the exciting
causes of tuberculosis undoubtedly, but perhaps the same may be said in
regard to measles, scarlatina, and the various other exanthemata. It is
undoubtedly true also that among the lower animals (and probably also
in the higher ones) the introduction into the circulation of the
semi-purulent fluid resulting from the breaking down of a cheesy
scrofulous gland will produce that peculiar (perhaps specific)
dyscrasia which results in miliary tuberculosis. But as it has also
been abundantly proved that a similar fluid derived from a cheesy
pneumonia, or from the inflammatory products of any other disease which
have undergone the cheesy degeneration, will also excite tuberculosis,
the fact does not seem to tell in favor of the identity of, or even of
any close relationship between, the two processes. Still, as the
scrofulous more frequently than other people are the subjects of this
cheesy process, it is not surprising that they should more frequently
be poisoned by the entrance into their blood of the cheesy detritus.

We do not deem it necessary to adduce all the evidence or to state
authorities upon this subject, but we think we are justified in stating
the following doctrine in regard to the relation of scrofula to
tuberculosis as best supported by facts and by the consensus of medical
opinion:

Scrofula is a purely diathetic disease inherent in the individual.

Tuberculosis is a cachectic (possibly a purely dyscratic) one, not
inherent in the individual, but always caused by some morbid influence
from without. Tuberculosis may therefore occur in the non-scrofulous as
well as in the scrofulous.

But the scrofulous are more likely than others to have
tuberculosis--1st, because of their greater susceptibility to all
morbid influences; 2d, because the scrofulous processes are apt to
produce some cachectic condition which is always a condition precedent
to tuberculosis; and, lastly, because the products of decay resulting
from scrofulous processes may enter the circulation and directly
produce the tuberculous dyscrasia. These remarks of course apply only
to primary tuberculosis.

But while we thus deny anything else than a purely incidental relation
of scrofula to tuberculosis, we believe that there exists the very
strongest possible relationship of scrofula to pulmonary consumption.
We think we are justified in stating that fully 95 per cent. of all
cases of pulmonary consumption are of inflammatory origin, and of that
variety miscalled catarrhal pneumonia. Broncho-pneumonia or
catarrho-pneumonia more exactly describes the process. It begins as a
catarrh, {242} sometimes in the nasal passages or post-nasal fossæ,
sometimes in the pharynx, but most frequently in the trachea and large
bronchial tubes, and sometimes rapidly, but oftener more slowly,
travels downward and invades the lining membrane of the air-sacs, which
soon become packed with cells derived partly from emigration of
leucocytes, partly from proliferation of the epithelium lining the
sacs. These cells soon undergo the cheesy degeneration, and, finally
breaking up, as in the case of the scrofulous gland, cause the
formation of vomicæ attended with the familiar signs of pulmonary
consumption. Every step in this process is attended with that abundant
cell-production, and the process itself is marked by that inveteracy
and intractableness, which always characterize scrofulous
inflammations, or rather inflammations in the scrofulous. Occurring as
they most frequently do in young adults, these cases are often mistaken
for pulmonary tuberculosis; and as post-mortem examination generally
reveals a more or less abundant secondary tubercular eruption caused by
absorption of infective material from the centres of cheesy
degeneration and softening, the diagnosis is claimed to be confirmed.
But they are for the most part, nevertheless, cases of genuine
scrofulous inflammation of the bronchial membrane and lining membrane
of the alveoli, and should be called scrofulous pneumonia. It is true
that Mr. Phillips, Mr. Kiener, Villemin, Grancher, Mr. Treves, and
others have collected numerous statistics which would show that
comparatively few of those who had died of pulmonary phthisis bore any
evidence of previous scrofulous disorder. But as the principal evidence
relied upon to prove this fact was an absence of scars resulting from
suppurating glands, their statistics are inconclusive. Besides, it is a
well-known fact that there is a decided antagonism between scrofulous
diseases of all kinds, and a patient who has one severe or well-marked
manifestation of scrofula is not likely to develop another strumous
disease at the same time. The records of the Margate Infirmary for
Scrofula show this fact very strongly, and numerous writers--among whom
may be mentioned Holmes, Birch-Hirschfeld, Walsh, Mr. Treves, and
others--strongly express the same opinion. Indeed, some of them go so
far as to maintain that one form of the scrofulous manifestation
confers protection against others. The question may perhaps be more
clearly stated by saying that the scrofulous, like the non-scrofulous,
have their special predispositions and indispositions to certain morbid
affections, and while one scrofulous child may be specially predisposed
to affections of the bones, joints, skin, or other tissues, it may have
no predisposition whatever to affections of the lungs or lymphatic
glands, etc. This difference in vulnerability or invulnerability of
certain tissues or organs in individuals, whether scrofulous or not, is
so distinctly recognized as a controlling factor in determining the
special form of disease resulting from a given irritant that its
discussion is entirely unnecessary. It is argued against the identity
of scrofula and pulmonary consumption that the commoner manifestations
of the former occur in childhood for the most part, while consumption
is a disease of adult life. But this is readily accounted for by the
different morbid tendencies and exposures in the two periods of life.
"Scrofula tends to appear in early life on account of the unusual
activity of the lymphatic system at that period, and phthisis somewhat
later--at a time, indeed, when the lungs are in more active use, when
{243} sedentary and perhaps unhealthy pursuits are assumed in exchange
for the liberty of childhood, when the modifying influences of puberty
are active, and the structural responsibilities of adult life press
heavily on an organization never other perhaps than frail.... I would,
on the contrary, assert that scrofula and phthisis are as much
manifestations of the same morbid change as acute bubo, acute orchitis,
and acute pneumonia are outcomes of one single process--acute
inflammation."[10]

[Footnote 10: _Scrofula and its Gland Diseases_, by Frederick Treves,
F.R.C.S., Eng.; New York, 1882, p. 62.]

It is entirely unnecessary--and indeed it would be too tedious--to
describe the anatomical appearances of the almost innumerable lesions
met with in the scrofulous. Holding as we do that scrofula is not a
disease per se, but merely a condition resulting from malnutrition and
consequent faulty construction of the tissues during the early years of
childhood, no peculiar or distinct anatomical lesion can be ascribed to
it; and yet every lesion of nutrition as well as of function may have
certain specific characteristics impressed upon it by the scrofulous
diathesis. These may be briefly summed up as great slowness in
evolution, intractableness, incurability, and chronicity of all
pathological processes, and in all inflammatory processes abundant
cell-production and tendency to caseation.

SYMPTOMS, COURSE, DURATION, AND TERMINATIONS.--A great deal of fine
writing has been expended in describing the physiognomy of scrofula,
and for ages writers exercised their descriptive powers upon the type
of face and form supposed to be indicative of the disease. It is almost
needless to say that much of this has been evolved from the
imaginations of the writers, while many of these descriptions are not
pictures of those liable to suffer from scrofulous processes, but of
those who are already the subjects of these, and are simply types, not
of the scrofulous diathesis, but only of the scrofulous cachexia. Many
of these pictures, too, were drawn not from the scrofulous, but the
tuberculous patient, because they were considered identical. Scrofula
is not confined to the dark or the fair, the dull or vivacious, nor
even to the weak and puny or the strong and robust; but all these may
have this faulty and often fatal construction. Nor do we believe that
scrofulous children are either more brilliant or more stupid than other
children. At most we can only say that the scrofulous habit is marked
by a deficiency of blood and a bad nutritive state of the more
important and more highly organized tissues. In some an abundance of
fat is found, giving to the individual a certain amount of plumpness,
which might be thought to be inconsistent with a state of bad health;
in others there is an imperfect development not only of the
subcutaneous fat, but of the skin and muscles also, so that they appear
tender and delicate. In the first of these conditions there is supposed
to be an indolent state of the processes of constructive and
destructive assimilation; in the second, an unnatural activity of these
processes. These differences have led to a classification of scrofula
into the phlegmatic or torpid and the sanguine or erethistic forms,
which Canstatt has thus described: "An unusually large head, coarse
features, a thick chin, a swollen abdomen, enlarged cervical glands,
and flabby, spongy flesh." The erethistic form is said to possess "a
skin of remarkable whiteness, with a tendency to redden easily, and
through which the {244} rose-pink or bluish subcutaneous veins are
visible, a deep redness of the cheeks and lips, blueness of the thin
and transparent sclerotica, which imparts a swimming and languishing
look to the eyes. The muscles of such persons are thin and soft, and
their weight is light in proportion to their stature, indicating a
slightness of their bones. The teeth are handsome and of a bluish
lustre, though long and narrow; the hair is soft."[11] Although this
description may be characterized as diagrammatic, since it describes
rather the extremes and not the mean of the general appearance of the
scrofulous, and numerous cases will be met with that cannot be assigned
to either of the above categories, yet as quite a large number of cases
will be seen that obviously belong to one or the other of these types,
and as, moreover, we shall see that by this classification we shall
obtain valuable data for therapeutic indications, it may be well to
preserve this division of the scrofulous into the lymphatic and
sanguine types.

[Footnote 11: Niemeyer's _Text-book of Practical Med._, vol. ii. p.
741.]

The leading points in the physiognomies of each of these types were
admirably shown in the composite photographs exhibited by Dr. Mohamed
at the last International Congress in England. By some special process
a composite photograph of many faces was, as it were, condensed into a
single picture, in which all that is common remains, all that is
individual disappears. And although Mohamed's pictures were all of
phthisical patients, it must be admitted that the two types of coarse
struma and sanguine struma were strikingly illustrated, and were very
suggestive of Canstatt's descriptions as given above. But it must be
borne in mind that a large number of the strumous belong strictly to
neither of these types, but rather to a medium between the two. "Such a
type would include what is known as pretty struma. The general features
of the individuals so termed belong to the so-called phlegmatic type,
but the coarseness of the features is toned down; the lips would be
called full, not tumid; and a coarse flabbiness would subside into a
pretty, plump condition of the body. The limbs, if not actually
graceful, are at least prettily rounded. The skin may not be thin and
fine, but it is soft, white, and clear. The general expression is not
absolutely apathetic, but would be termed gentle and eminently
feminine. Excellent representations of this type of pretty struma were
also shown in the photographic series above mentioned."[12]

[Footnote 12: Treves, _Scrofula and its Gland Diseases_, p. 84.]

This matter of physiognomy of the scrofulous has this much at least of
practical importance--viz. that to the sanguine or erethistic type
belong those cases that show distinct heredity, while the phlegmatic or
torpid is usually the type assumed in the acquired forms. While there
are doubtless numerous exceptions, it will generally be found that
scrofula in the rich assumes the first, and in the poor the second, of
these forms. It has been asserted that the erethistic form is more apt
to develop tuberculosis or phthisis; and to a certain extent this is
doubtless true, but the torpid are by no means exempt from this grave
accident. The first are undeniably more liable to the more severe and
fatal forms of the disease, which run a more rapid course and are less
amenable to treatment, while in the second phthisis is more apt to be
chronic and incomplete recoveries are by no means rare. The first form
is said to {245} be more frequent in women, while the second is more
frequent in males; and this accords with my own observation and
experience.

There are certain features more or less peculiar to scrofula, besides
those appertaining to the general physiognomy already discussed, which
it may be well to call attention to, since these may aid us in
detecting the scrofulous diathesis even before the grosser
manifestations have declared themselves.

Allusion has already been made to the defective blood-vascular
capillary network in the scrofulous as a necessary consequence of the
excessive predominance of lymph-spaces and lymphatic vessels. Indeed,
there can scarcely be a doubt that the slowness of evolution of various
pathological processes, their chronicity, and the absence of tendency
to resolution and cure of inflammatory lesions, so prominent a feature
in all scrofulous manifestations, is due to this very condition. It is
especially in the coarser type of struma that these defects in the
circulation are most conspicuous. In these the pulse is often below the
average, soft, and wanting in vigor. The cheeks and limbs often assume
a bluish and mottled aspect, due perhaps to a tendency to stagnation of
the blood in exposed parts. The extremities appear swollen as if from
cold, and in the winter generally appear chapped. They are particularly
liable to chilblains, which persist far into the summer and often take
on a very unhealthy action. This last feature is so common as to
constitute an important symptom in scrofula. These defects in the
circulation also probably explain the frequent catarrhs and eczemas
with which such persons are affected, and account also for their
intractableness as well as the unwholesome character of their wounds.

For the same reason (deficient circulation) the temperature is
generally found to be a little lower in the coarsely strumous than in
healthy children, and even in their fevers a very high temperature is
rarely met with. Acute sthenic inflammations are rarely seen, and hence
these persons seldom have acute croupous pneumonias; it is rather the
catarrhal variety, and of this the subacute and chronic forms, which
they suffer from.

Opinions are completely at variance as to the influence of the
scrofulous habit in delaying or hastening menstruation. Lugol referred
to the frequency of dysmenorrhoea among the strumous, and there is no
doubt that the scrofulous as a rule often suffer from suppressed or
scanty menstruation. But it is improbable that the diathesis exerts any
influence whatever in determining the period of puberty in either sex.

We have already stated our belief that the strumous are neither more
intelligent nor stupid mentally than other people. An exception ought
perhaps to be made to this in the case of the exaggerated type of the
coarsely strumous. In these extreme cases we must confess that we have
generally found associated great slowness and dulness of the mental
faculties. If great intelligence and precocity are sometimes met with,
it is only in the erethistic or pretty struma, who, because it is the
delicate one of the family, is petted, has more notice taken of it, and
afforded every facility for the development of the points that make up
the precocious infant. The prettiness of these children, moreover,
attracts more attention to them than to other children or than the bulk
of the sickly would receive.

In young scrofulous children we often observe a considerable amount
{246} of close-lying downy hair upon the forehead, more abundant upon
the sides of the forehead. Upon the arms and back from the occiput to
below the shoulders also a like condition is often seen. Later the
eyelashes appear thicker and longer, and the eyebrows more abundant,
coarser, and longer, than in the non-scrofulous. The color of these is
also apt to be darker than the rest of the hair.

Constantine Paul, as quoted by Treves, has drawn attention to certain
changes in the ears, after they have been pierced for earrings, that he
considers to be diagnostic of scrofula. The mere weight of the earring
seems to cause the puncture to slowly ulcerate, and the ring thus cuts
its way out, either leaving behind it a linear scar or a slit in the
lobule. If the lobule be repaired the ring may cut its way out again,
and this may occur three or four times. These changes seem not so
frequently to be observed in England and America, and may be due in
part to the fact that earrings of greater weight, and more frequently
of base metal, are worn in France than in the countries named. But
still, from what has been said concerning the histology and minute
anatomy of the scrofulous, and the consequent less resistance of the
tissues, this cutting-out process by earrings is just what we would be
led to expect in strumous persons.

The thick upper lip is never absent from the older descriptions of the
physiognomy of the strumous. This is almost invariably present in the
coarse type of struma, and seldom absent even in the erethistic. It is
not always due to irritation from acrid discharges from the nose, as is
maintained by Treves, though doubtless the eczematous and herpetic
eruptions are often caused and maintained by these discharges, and
these may in time cause and increase this thickening.

The teeth in scrofula show nothing that is distinctive, though there is
undoubtedly a tendency to early decay. As this tendency to decay is,
however, so common in many persons who have at least shown no other
evidences of the scrofulous diathesis, no positive conclusions can be
drawn from this fact.

Clubbed fingers, too, so common in persons who have become cachectic
from the long persistence of scrofulous disorders, are not
characteristic. Clubbed fingers and incurvated nails will generally be
found in persons suffering from any disease characterized by slow
wasting. They are seen in phthisis of all varieties, as well as in
cancer, heart disease, aneurism, Bright's disease, empyema. They
therefore have no significance as far as struma is concerned.

GENERAL MANIFESTATIONS OF SCROFULA.--As, according to our view, there
is no such disease per se as scrofula, but simply a diathesis which
impresses its own malign influence upon every other disease with which
the strumous individual may happen to be afflicted, increasing perhaps
the general predisposition to be injuriously affected by all morbific
influences, or impairing the powers of resistance to these, and
especially intensifying any special predisposition which age, sex,
personal peculiarities, occupation, habits, mode of life, or heredity
may have created, we cannot describe any morbid processes as
specifically scrofulous. At most, we can only say that struma is more
apt to impress its malign influence upon certain diseases or upon
inflammations and injuries of certain tissues, that some diseases in
the scrofulous are more apt to be {247} attended by certain
complications and followed by certain sequelæ, and that all of these
are characterized by chronicity and incurability, by slowness of
evolution of pathological processes, and, in the case of inflammations,
by a tendency to profuse cell-production and to rapid caseation. Thus,
measles is apt to be complicated with or followed by otorrhoea, chronic
bronchitis, caseation of bronchial glands, phthisis, and even
tuberculosis; scarlatina by otitis, hyperplasia of the tonsils,
caseation or suppuration of the submaxillary and other lymphatic glands
about the neck, and by chronic catarrh of the renal mucous membranes,
causing dropsy and finally death; eczemas about the face or catarrhs of
the mouth and throat by hyperplasiæ and caseation of lymphatic glands
in the neighborhood. Boils and other subcutaneous inflammations of the
areolar tissue, so common in childhood and adolescence, do not run
their usual rapid course, ending in suppurations and cicatrization, but
become in the one case the scrofulous gumma, degenerating into the
scrofulous ulcer, or if more deeply seated become a cold abscess. A
single injury of a joint, whether mechanical or rheumatic, will
"sometimes take the form of a simple hydrarthrosis, sometimes that of a
so-called tumor albus, while at others it assumes the nature of a
malignant arthrocace, accompanied by suppuration, caries of the
articular surfaces, burrowing of pus, and the establishment of
fistulæ."[13] A slight injury inflicted in the sports of childhood and
soon forgotten--the prick of a pin perhaps--is followed by a disease
sometimes beginning in the periosteum, sometimes in the bone itself,
and presenting at one time the character of periostitis and ostitis,
and at another that of caries or necrosis, or of the two combined.

[Footnote 13: Niemeyer, _loc. cit._]

"As long as the existence of cheesy masses," says Niemeyer, "was
regarded as characteristic of the tuberculous nature of a disease, it
was of course necessary to ascribe many of the inflammations of the
joints and bones of scrofulous persons to a complication of scrofulosis
with tuberculosis."[14]

[Footnote 14: It is a well-established fact, however, that true miliary
tubercles are often found in the neighborhood of bone and joint
affections in the scrofulous, as well as in lupus, in cold abscess, and
in softening caseous glands, which last are considered by many as
specifically scrofulous diseases. It is suggested that an explanation
of this may be found in the probable fact that caseous pus may be
capable not only of producing a general tuberculosis when carried by
veins or lymphatics into the blood, but that it may also set up a local
tuberculosis by a morbid influence exerted upon the neighboring
lymphatics and blood-vessels with which it may come in contact. We are
aware that Wilson Fox (according to the _Medical Times and Gazette_),
captivated by the theory of Koch, has recently recanted his belief in
the inoculability of tuberculosis with anything except tubercle. But we
are afraid that Dr. Fox (who we believe was one among the first to
confirm Ferdinand Cohn's experiments in producing tuberculosis in
rabbits and guinea-pigs by inoculating them with caseous pus) is
suffering from that most active and virulent of all contagions, the
contagion of popular belief. Just now a belief in specific bacilli and
micrococci may be said to be riding upon the crest of a very high wave
of popularity, and we are afraid that many of those who are rushing
forward to mount this wave also will ultimately find themselves
stranded upon that shore which has been strewn with so many wrecks in
the past.]

A simple bronchitis, possessing nothing specific in its origin at
least, will persist and extend to the lobuli of the lung and excite a
catarrho-pneumonia which ends in consumption and death; a simple
intestinal catarrh will result in inflammation and caseation of the
mesenteric glands--a tabes mesenterica; or a simple dysentery,
persisting in spite of the most {248} approved treatment, causes
proliferation and caseation of the endothelial cells of the follicles,
terminating in that obstinate and intractable follicular ulceration
which wastes the strength and wears out the life of the little patient.

A simple conjunctivitis of the globe often will be followed by
ulceration of the cornea, giving rise to intense photophobia, and leave
behind it opacities of that organ which remain a perpetual evidence of
the scrofulous diathesis, if they do not shut out for ever the light
from the eye. Or if it is the palpebral conjunctiva that is affected,
the meibomian glands and follicles of the ciliæ become involved,
destroying the lashes and leaving the lids raw and everted or
inverted--a perpetual deformity. In short, there is no conceivable
disease or injury occurring in what we may call the intensely
scrofulous that does not have impressed upon it some one or more of the
malign characteristics which we have spoken of as indicative of the
scrofulous diathesis. But it is not probable that there is ever any
special disorder or lesion which can be said to be caused exclusively
by scrofula; or, in other words, there is no such disease as a
specifically scrofulous one. Lupus, cold abscess, and particularly
caseous glands, are especially attributed to struma, because they are
often thought to make their appearance independent of any assignable
cause; but as boils, eczema, impetigo, and numerous other affections of
the skin and areolar tissue affect children who are not scrofulous, and
equally independent of any known causes, the argument is not
conclusive. Besides, all these affections occur sometimes in the
non-scrofulous; and even caseation of a single inflamed gland quite
often occurs in children who are weak or in ill-health, but who show no
other evidences at that or at any other time of the scrofulous habit.

DIAGNOSIS.--The only affection likely to be mistaken for scrofula is
congenital or acquired syphilis in its later manifestations. In this
disease we see the same tendency to increased cell-production, the same
tedious, slow, and intractable inflammations and ulcerations, which are
characteristic of scrofula. And this apparent similarity has induced
many persons to believe that scrofula is nothing else than syphilis in
the second or third generations. But in congenital syphilis the lesions
usually make their appearance soon after birth or are present at birth,
and long before even hereditary scrofula begins to show its malign
influence. In most cases, too, a history of syphilis can be obtained,
and even when this is not obtainable a few inunctions or fumigations
with mercury, in connection with a few large doses of iodide of
potassium, will very quickly decide the question of diagnosis for us.

In the case of lupus, in which Erichsen admits there is no means of
positively distinguishing the syphilitic from the so-called scrofulous
varieties, the diagnosis is more difficult. But as this disease appears
later in life than the more ordinary scrofulous manifestations--when,
therefore, a history of syphilis can generally be obtained if there is
one, and when there would almost certainly be also a history of
scrofula if it existed--it would seem that the diagnosis even in this
case cannot be so difficult. Diagnosis here, however, is of little
consequence, since the treatment recommended for both forms is the
same.

PROGNOSIS.--This of course depends upon the nature of the special
lesion. The simpler lesions incident to childhood, such as glandular
{249} hyperplasiæ, catarrhs, eczemas, impetigoes, etc., usually do well
under appropriate treatment and proper hygienic conditions. Diseases of
joints, bones, mesenteric glands, etc. often terminate fatally or
result in serious deformities and permanent impairment of function. Not
infrequently diseases of the bones and articulations, attended with
profuse and protracted suppuration, cause amyloid degeneration of the
liver, kidneys, spleen, or other glandular organs, and, as a
consequence, death. Catarrho-pneumonia in a scrofulous subject almost
invariably causes phthisis sooner or later. Occasionally the caseated
cellular exudation in the air-sacs remains quiescent for months, and
even years, the patient remaining quite well except for a harassing
cough during the winter months; but sooner or later the caseous mass
will soften, the symptoms of active consumption ensue, with fever and
wasting, and death closes the scene. Far more frequently, however,
softening and suppuration follow swiftly upon the caseous degeneration,
and the whole process occupies a period of only a few months.
Tuberculosis especially runs a rapid course in these subjects, and
while a few perhaps only develop tuberculosis of the lungs--in which
case the duration of the disease may be a little longer--in by far the
larger number there is a generalization of the tubercular process which
puts a speedy end to their existence.

TREATMENT.--This may be most profitably discussed under two
heads--prophylactic and therapeutic.

Prophylactic.--Scrofulous persons who are closely related by blood
should be earnestly advised not to intermarry. We have so often seen
the deplorable results upon offspring of such marriages that we cannot
too strongly urge this upon the profession. Such persons should be
frankly and clearly told what are most likely to be the consequences of
such marriage, and all possible moral influences should be exerted to
prevent them. The canons of the Church wisely interdict such marriages,
but, unfortunately, its ministers seldom attempt to enforce them, or if
they do their efforts are made ineffectual by the facility with which
the marriage-rite can be obtained from civil officers in most of the
States of the American Union. The medical profession can do more than
any other class to diffuse knowledge and create a correct public
opinion upon this subject, but, unfortunately, it too often neglects
this important mission.

The children of scrofulous parents should be nursed (at the breast)
longer than other children, so as to ensure an abundance of animal food
during the first two years of life. Some advise scrofulous mothers not
to nurse their children, lest they should imbibe the scrofulous taint
through the milk. This fear is entirely groundless. We know of no
reason why such a mother should not nurse her offspring, unless it be
that it injures her. The child receives its scrofulous inheritance not
through the mother's milk, but from the ovarian or spermatic cell. Milk
can convey no disease or diathesis except on account of its deficiency
in nutritive properties. If, therefore, there is any special reason why
the mother should not nurse her infant on her own account, it may be
well to turn it over to a healthy wet-nurse; but the temptation to give
an infant raised on the bottle starchy foods prematurely is too strong
generally to be resisted. The numerous infant foods advertised consist
principally of starch, and young infants would infallibly starve on any
or all of them {250} if their venders did not always direct that they
should be taken with a large quantity of cow's milk. If the
circumstances of the parents do not enable them to obtain a wet-nurse,
then good cow's milk constitutes the best food for infants until they
have cut their canine and anterior molar teeth. The custom of weaning
infants at a certain age in every case is a pernicious one. Some
infants are as well developed as to their digestive organs at fifteen
months as others are at thirty, and the eruption of the teeth may
generally be taken as a safe guide as to that question. A moderate
amount of food containing starch after the period indicated may be
allowed, but always with a preponderance of animal food. It is not so
much the starch that acts injuriously upon the nutrition of children as
the excess of that substance; and if the food contains but little
nutrition in proportion to its bulk, it is so much the worse. Even milk
containing too little casein and fat in proportion to the watery
elements may be perhaps quite as injurious as potatoes. And hence if
the mother's milk should be poor in these elements, it ought to be
supplemented with cod-liver oil or other animal fat in small doses.

A practice existed among the Southern slaves (and to some extent also
among the whites) before emancipation which at first I was inclined to
condemn until I saw the excellent effects resulting from it. Within an
hour or so after birth a piece of fat salt pork or bacon was placed
between the child's lips, and it was permitted to suck this at all
times when not nursing. Tied to its wrist by a short string, so as to
prevent swallowing it, this piece of pork furnished both nutrition and
amusement to the infant for many hours while the mother was at work in
field or garden. The children throve well on it, and thus treated we
found them to be as well developed at twelve months as most other
children were at twenty. It was doubtless due in part to this practice
that there was so little scrofula among them.

An abundance of pure air is also a valuable factor in preventing the
establishment of the strumous diathesis. Strict regard, therefore,
should be had to ventilation, and overcrowding should if possible be
avoided. Children over twelve months of age should not even be
permitted to sleep with their parents, but should have in cold weather
a crib, cradle, or other bed to themselves; and in warm weather they
should be put to sleep in a net hammock, which is now so cheap as to be
within the means of almost everybody. This will not only secure to them
a better supply of air, but it will also prevent them from suffering so
much from the heat, which is so potent a factor in the production of
cholera infantum.

Bathing in proper season is also useful as a prophylactic. Sea-bathing
especially has long enjoyed great credit as a remedy for scrofula, but
we think this is often resorted to too soon and practised at improper
times. In warm countries a bath of cold water may be taken every day in
the year, but it should be given at the warmest hour of the day, not
early in the morning. In all climates due regard should be had to the
powers of resistance to cold and the promptness of reaction after the
bath. If children remain cold and pale for a long time after the cold
bath, the practice should be discontinued and tepid water substituted.
In colder climates tepid bathing should be practised once or twice a
day during the winter, and in summer a little lower temperature may be
used. Bathing children under three or four years in the sea at any time
is pernicious, {251} both because the temperature is too low and on
account of the fright which it always causes in these young children.
After four years a child will take to the water almost as instinctively
as a young duck.

Therapeutic.--Almost all of the so-called scrofulous manifestations
belong to the surgeon, dermatologist, or oculist and aurist, and we
shall therefore say nothing about the special and local treatment of
these manifestations, but refer the reader to works upon these several
departments of medicine. But as little success will be had in the
treatment of these special disorders unless due regard is had to the
general condition, and unless the local treatment is supplemented by
constitutional measures, we shall briefly give some directions for this
constitutional treatment of the scrofulous individual.

It is important in determining upon the proper treatment in any given
case to bear in mind the division of the scrofulous into the two types
of torpid or lymphatic and sanguine or erethistic already described. It
is true that in many cases it is not easy to determine to which class a
patient belongs, and many possessing some of the characteristics of
both certainly cannot be referred to either. Still, in many cases the
discrimination is easy, and then furnishes very clear and valuable
indications as to treatment. Iodine (and its preparations) has since
the time of Lugol, who first brought it into prominent notice, been
regarded as a useful remedy in scrofula. But burnt sponge (spongia
usta), which contained the iodides of sodium and potassium, had been
used to dissipate goitrous and scrofulous swellings many hundreds of
years before the time of Lugol. It is a valuable remedy in certain
cases, and if it is falling into disuse it is probably for the want of
proper discrimination in the selection of cases. In all cases in which
there seems to be an abundant production of fat, and therefore in
nearly all the cases of coarse struma where there is an indolent
process of assimilation and disassimilation, iodine and its
preparations will be found useful. Indeed, in the form of syrup of
iodide of iron we have rarely failed with it to cause strumous
enlargements of glands to disappear when the remedy was used soon after
their first appearance. Of course, neither iodine nor any other
medicine can have any effect in removing these enlargements after the
glands have become caseous. While good results may be obtained with the
syrup in all forms of scrofula, it is unquestionably in the sanguine
and neutral types that it is most useful. It should be given in doses
of 10 to 30 drops to children under five years of age, and to older
ones ½ to 1 fluidrachm three or four times a day may be administered.
We have given the latter dose to children four or five years of age for
a long time, with the best effect upon their scrofulous manifestations,
and without any injury whatever to their digestive organs.

In the torpid types preparations stronger in iodine should be used.
Here Lugol's solution or iodide of potassium or sodium will be found
very useful, either alone or in connection with the iron preparation
above mentioned. Indeed, as in these cases it seems to be
disassimilation that appears to be specially faulty, even very small
doses of mercury in the form of bichloride or biniodide will be found
useful. Donovan's solution may be prescribed in these cases along with
the active preparations of iodine with good effect, or if the arsenic
in that preparation is objectionable, one-fiftieth of a grain of
bichloride or biniodide of {252} mercury may be substituted. The
mercurial should not, however, be continued longer than one or at most
two weeks at a time, after which it should be suspended and the iodine
continued.

Cod-liver oil, which is too indiscriminately prescribed in all cases,
will be found to be of little use in the lymphatic types, if indeed it
is not actually injurious; but in those cases with pale, thin skin,
with deficient development of fat, and with small muscles--in short,
those in which emaciation or delicacy is prominent--it is a most
valuable remedy. It is almost surprising to see how rapidly
ulcerations, caries, eczemas, catarrhs, etc. occurring in this class of
subjects will disappear under the use of this medicine alone.

The hypophosphites and lactophosphates are also useful in this class of
cases, especially where there is disease of bone or joints, in
connection with the cod-liver oil. We have long been in the habit of
using the following formula, which we have found very useful:

  Rx. Pulv. Acaciæ,                  drachm ij;
      Ol. Amygdal. amar.,            gtt. vj;
      Syr. Calcii hypophosphit.,
        vel Syr. Calcii lactophos.,  fluidounce iv;
      Ol. Morrhuæ,                   fluidounce iv;
        Ft. mist.

S. Teaspoonful to tablespoonful three times a day according to age.
Syrup of iodide of iron may be added if desirable, though we prefer to
give this by itself.

Gentle exercise, passive or active, pure air, well-ventilated sleeping
apartments, a generous diet--in which wholesome animal food should
predominate--and bathing are of course as necessary and as useful in
the treatment as in the prevention of the scrofulous diathesis.

Alkalies should be given in all cases in which we are trying to
dissipate enlarged lymphatic glands, for the reason that caseation of
these glands occurs because of insufficient alkalinity of the blood to
effect reduction of fat, and because also the strumous almost always
suffer from excessive acidity of the gastric and other secretions. When
the iodides of potash or soda or the hypophosphites of lime and soda
are given, the additional administration of alkalies may not be
necessary; but if not, bicarbonate of sodium or potassium (which have
long enjoyed a good reputation in the treatment of struma) should be
added to the other remedies.

Since the appearance of Niemeyer's _Handbook of Clinical Medicine_ the
proper treatment of scrofulous glands that have undergone the caseous
degeneration has been a moot question. Some recommend the ablation of
these glands by the knife, some advise spooning out the caseous matter
through a small opening, while others prefer to await the natural
process of softening and the discharge of the caseous matter by
suppuration. There can be no question that the removal of these glands
by the knife, when this can be done without serious risk, will leave
behind a less unsightly scar, and will be attended with less fever and
consequent deterioration of the general health, than usually attends
suppuration. Spooning out the caseous matter will perhaps leave no
extensive cicatrix, but we can never be sure that by this operation we
have removed all the caseous matter, and it must certainly be more
painful than the knife. Mothers will generally object to either of
these {253} operations, and as the risks of infection by absorption of
the caseous pus during the suppurating process do not seem to be very
great, it is perhaps best to leave these glands to nature, unless the
vitality of the patient is so low as to give reasonable ground for fear
that the child may succumb to the effects of the natural process. If
any surgical interference is deemed necessary, we are decidedly in
favor of removing the caseous gland entire by the knife.



{254}

HEREDITARY SYPHILIS.

BY J. WILLIAM WHITE, M.D.


About a half century ago Mr. Abraham Colles, who had just resigned the
professorship of the Theory and Practice of Surgery in the Royal
College of Surgeons in Ireland, the duties of which, in the opinion of
the college, he had discharged for thirty-two years in an "exemplary
and efficient manner," wrote the following introductory paragraph to
his remarkable chapter on "Syphilis in Infants:" "Perhaps there is not
in the entire range of surgical diseases any one the contemplation of
which is more calculated to arrest our attention or to excite our
interest than syphilis infantum."

Although it was not then, and is not at the present day, strictly
relegated to the domain of surgery, hereditary syphilis, like its
parent disease, was generally treated of by the practitioner of that
branch of medicine. And yet in the great majority of instances the
management of such cases, especially as regards their family relations,
the relations of husband and wife, the management of the latter during
pregnancy, the delivery and subsequent care of the child, the necessary
attention to the safety of other members of the family--in fact, all of
the most weighty responsibility--falls upon the ordinary medical
attendant. It is therefore in every way proper that the condition
should receive some notice in a system of general medicine.

A proper presentation of the subject of hereditary syphilis involves a
consideration of the vexed question as to the mode by which the disease
is conveyed from parent to offspring. That it may be so transmitted has
been generally believed since the doctrine was first announced by
Torella at the end of the fifteenth century; and the facts in its
support are so numerous and convincing that, in spite of a few
distinguished opponents--among whom John Hunter was the most
conspicuous[1]--it has been unhesitatingly accepted by the profession
down to the present day. As regards the manner of transmission,
however, controversy has been and still is rife. Opposing theories have
been constructed and ardently supported, differing radically as to
essential points, often resting upon exceptional or anomalous, and
still oftener upon imperfectly observed, cases.[2]

[Footnote 1: _Works of John Hunter_, vol. ii. p. 383.]

[Footnote 2: Parrot, in a clinical lecture on syphilitic abortion (_Le
Progrès Médicale_, Nov. 3, 1877, p. 798), says: "The infection of
children was known, but its true origin was not suspected. The belief
of Gaspard Torella (1498) and Matthioli (1536) that it came from the
nurses through the milk was generally accepted."

{255} According to Diday, Paracelsus (1529) was the first to plainly
state the heredity of syphilis: "Fit morbus hereditarius et transit a
patre ad filium."

Others attribute the original announcement to Augier Ferrier (1553),
and it seems certain that he was first to specify the three modes of
infection of the product of conception: "La semence du père, celle de
la mère, et la contamination de la mère durant la grossesse."

Fallopius in a posthumous treatise on the Mal Français (1566) adds the
authority of his name to this view: "Præterea videbitis puerulos
nascentes ex foemina infecta, ut ferant peccata parentum, qui vedentur
semi cocti."

Ambroise Paré also acquiesced in the theory, saying, "Souvent on voir
sortir les petits enfants hors le ventré de leur mère, ayant ceste
maladie, et tost après avoir plusieurs pustules sur leur corps;
lesquels étant ainsi infectés, baillent la vérolle à autant de
nourrices qui les allaictent."

Subsequently, Mauriceau, Boerhaave, and Astruc sustained the same view,
which, with the single exception of Hunter, had no prominent
antagonist.

It was not, however, until the eighteenth century that it was described
with any attempt at detail or exactness by Rosenstein, and his essay is
loaded with errors. It was in the foundling hospitals of Paris at the
end of the last century, in the wards of Salpétrière and Bicêtre, and
in the hospitals of Vaugirard and in the Capucin convents of the Rue
Saint Jacques, where pregnant women and nurses attacked with syphilis
were admitted, that methodical and trustworthy observations were made
(1780-1810) by Colombier, Despenières, Doublet, Mahon, Cullerier, and
Bertin. Since then the history of the disease has been the history of
syphilis itself.]

A full consideration of these, or even a recapitulation of the
respective arguments pro and con, would far exceed the limits allotted
to the present article, and we will confine ourselves simply to stating
the questions which most nearly concern the practical physician, and
the conclusions which the accumulated observation and experience of the
profession seem to justify. The points bearing upon the general subject
of hereditary syphilis which exercise an important influence upon
advice or opinions of the utmost gravity as regards the happiness and
well-being of the individual or family may be enumerated as follows:

1. Is syphilis in all its stages transmissible (_a_) to the wife or
husband, (_b_) to the offspring? Or, in other words, is it ever proper
to consent to the marriage of a person who has had syphilis? If so,
under what circumstances?

2. By what means or through what channels can the disease of the
parents reach the child?

3. What are the pathology and symptoms of hereditary syphilis?

4. What is the treatment--(_a_) prophylactic, applied to the parents,
and (_b_) curative? We may now take these up seriatim.

No more important questions can be submitted to a medical man than
those pertaining to the marriage of syphilitics. Involving as it does
the welfare of many individuals, modifying or fixing the conditions or
circumstances of one or more lives, his opinion should be exceptionally
definite and well grounded. The responsibility of advising or
consenting to the marriage of a person who has once had syphilis is
undoubtedly great; the responsibility of prohibiting it is, however, no
less so. Matrimony is the natural condition for the majority of people.
Enforced celibacy, especially in males, brings with it not infrequently
a long train of attendant evils, moral and physical. It will not do to
assume that professional duty is properly discharged by telling all
patients to be on the safe side and to remain single for fear of
inoculating wife or offspring, unless it can be clearly shown that
there is a definite and unavoidable risk in every case, which continues
throughout life.[3]

[Footnote 3: "The surgeon who, on account of past syphilis, forbids
marriage to an otherwise {256} eligible man must remember that he
forbids it at the same time to some woman, who, possibly, if well
informed as to her risks, would willingly encounter them.... Respecting
a malady so common as syphilis, while it is often our duty to warn, it
is also not unfrequently our duty to encourage" (Mr. Hutchinson,
preface to the English translation of Fournier's _Syphilis and
Marriage_, p. vii.)]

There are two distinct methods of arriving at an answer to the question
under discussion: first, by considering the probabilities in regard to
the essential nature of syphilis; and, second, by carefully weighing
the clinical evidence in the matter. It seems evident that belief in
any particular theory of syphilis assigning it to this or that class of
disease must have an important influence in determining the opinion
which is held as to its curability, or at least as to its indefinite
transmissibility.

If the late symptoms, the so-called tertiary outbreaks of the disease,
are held to be evidences of the presence in the system of the specific
virus, which has simply remained for a long period, perhaps for many
years, latent or quiescent, and which is thus again manifesting its
power; if syphilis is believed, accordingly, to be a practically
unlimited disease, conforming to no known law as regards its duration,
corresponding to no other infectious or contagious malady in having a
period of termination--more or less delayed perhaps, or more or less
indefinite, but still invariably present--at which time either the
particular poison or the susceptibility of the system to its influence
has become exhausted;--if tertiary syphilis, in other words, is
regarded as simply a continuance or recurrence of the disease,
differing in no essential respect, except as to the particular tissues
involved, from the same disease in its early stages, it is difficult to
see how marriage can ever be conscientiously recommended to a person
who has once contracted it.

It is unfortunately true that in no given instance is it safe to assure
a patient of further complete immunity from the disease. In any case,
however mild in its course or under whatever treatment, there is always
an element of doubt as to the development of subsequent symptoms. The
probability of their appearance may be reduced to a minimum, the
character of the case and the thoroughness of the treatment may both
seem to give assurance that a cure has been completed, and yet both
patient and physician may be mortified and annoyed by an outbreak of
tertiary lesions. This, I think, would be admitted by every one of
large experience with the disease, and indeed furnishes the chief
argument to those who deny or are sceptical as to its curability. If,
then, it were impossible to predict with any sort of certainty that the
contagious and highly transmissible stage of syphilis would terminate
during the life of the individual, it would manifestly be unwise to
permit marriage, with its risk of inoculating the innocent partner and
the consequent double risk to the offspring.

If, however, syphilis is, as taught by Mr. Hutchinson,[4] one of the
exanthemata, having, like them, a period of outbreak, a period of
efflorescence or eruption, and a period of subsidence, and followed,
like them, by certain non-contagious sequelæ, which we call the
tertiary symptoms, but which are merely relapses or degenerations of
parts affected during the secondary stage, it becomes evident that the
risk of transmission to wife or husband or children _after the lapse of
a certain interval_ becomes greatly reduced or almost nil. No one
thinks of forbidding marriage on {257} account of a previous attack of
small-pox or scarlet fever or typhoid fever, even although there are
unpleasant consequences which sometimes follow these diseases.

[Footnote 4: _The London Lancet_, Feb. 5, 1876; Reynolds's _System of
Medicine_, Am. ed., p. 423.]

Or if another and still more plausible theory of syphilis is adopted,
and we look on the tertiary period as one of contraction or
obliteration of lymphatics due to long-continued irritation by the new
cell-growths of the secondary stage, which from the very onset affect
those vessels, our views will be materially modified. During the
primary period, then, when the new cells, which either constitute the
poison of syphilis or are its carriers, are accumulating at the site of
original inoculation, constituting the induration of the chancre, or
are slowly finding their way into the general system through the
lymphatic vessels, proliferating in their walls and thickening and
hardening them, or during the secondary period, when they are rapidly
multiplying in all the tissues of the body, the risk of inoculation or
transmission would be manifestly great. When, however, by destructive
metamorphosis and degeneration, either with or without the aid of
drugs, they have been eliminated from the body, the contagious element
disappears with them; and although here and there throughout the body
some important lymphatic trunk may have undergone irreparable injury,
and may have been contracted or obliterated, permitting of the
accumulation of waste products until the node or gumma or tubercle
which we call a tertiary symptom makes its appearance, yet the disease
has lost much of its terror, and has become dangerous only to the
patient himself.[5]

[Footnote 5: _Clinical Lectures on the Physiological Pathology and
Treatment of Syphilis_, by F. N. Otis, M.D.; _Syphilis_, by V. Cornil,
Am. ed., 1882, pp. 17-27.]

These theories are only alluded to by way of elucidation of the
statement that belief in one or the other of them has an important
bearing on the relation of syphilis to marriage, and because, whichever
is thought to be the most plausible, they equally lessen or altogether
do away with a certain proportion of the danger formerly thought to
surround the marriage of a syphilitic even after a most protracted
interval. It is not necessary to accept either the one or the other
implicitly. The essential point is the recognition of the fact that
modern syphilographers, as a rule, regard the tertiary or late symptoms
as indicative of damage done during the active period--as relapses or
sequelæ, and not as fresh outbreaks, of a highly contagious and
transmissible disease. Their time of appearance, their entire want of
symmetry, their non-contagiousness, their non-inoculability, all favor
this view, and we may now see what evidence corroborative of it may be
obtained from clinical facts.

It will be necessary, in the first place, to admit that there seems to
be but little doubt in the minds of most syphilographers that in rare
instances syphilitic children have been born to parents who had long
passed the limits of the secondary period. At least the great majority
of writers upon this subject speak confidently of the exceptional
occurrence of such cases, and assert that syphilis may be transmitted
during any of its stages.[6] If, however, we come to look for positive
evidence in this respect, we will find very little that is entirely
satisfactory. Cases are reported, to be sure, in which eight, ten,
twelve, or even fifteen or twenty, years after the primary sore,
syphilitic patients have become the parents {258} of children who
showed unmistakable indications of the disease. When we examine the
history of the cases, we find usually that many important points are
omitted without which it is impossible to be certain of its true
character. Were both parents originally infected? If not, has a recent
case of syphilis occurred in the one who at first escaped? If they were
both diseased originally, has either been subsequently re-infected?--a
much more frequent accident than has been commonly supposed.[7] On
applying these tests to the cases in question it will be found that few
if any of them are thoroughly convincing. Kassowitz's observations,[8]
made, it must be remembered, upon persons with whom no mercurial
treatment had been employed, seemed to show that the average limit of
transmissibility was about ten years, after which time healthy children
began to be born. His observations were, however, incomplete in many
respects, and, like all such investigations, are of course open to the
suspicion of intentional deception on the part of the patients.[9] Even
these cases, however, show unequivocally, as do all which have ever
been recorded, the steady diminution of the transmissive power under
the influence of time alone.

[Footnote 6: Belhomme et Martin, _Traité de la Syphilis_, p. 413.]

[Footnote 7: Cornil, _op. cit._, p. 20.]

[Footnote 8: _Die Vererbung der Syphilis_, Wien, 1876.]

[Footnote 9: See also Hutchinson, _British and Foreign Med.-Chir.
Rev._, Oct., 1877.]

I have said that the majority of writers seem to have no doubt of the
long continuance of this transmissive power in rare cases. There are,
however, a few notable exceptions. Fournier, whose immense experience
and acuteness of observation entitle his opinion to the utmost
consideration, says that in cases of paternal heredity the duration of
the force of transmission never exceeds at the maximum three or four
years.[10] In no case of the many hundreds he has observed has he known
a syphilitic father to infect a child--the mother being healthy--at a
later period than the one mentioned. And he is equally positive that
the gradual diminution and final extinction of the syphilitic reaction
of the parents upon the children is a veritable pathological law,
"absolutely demonstrated."[11]

[Footnote 10: _Syphilis and Marriage_, Am. ed., p. 87.]

[Footnote 11: _Op. cit._, p. 88. Of course when both parents are
diseased a somewhat longer period of activity is to be expected for the
poison.]

M. Mireur, a careful and accurate observer, records[12] a striking
instance in which in the history of a couple, both syphilitic and
untreated, eight pregnancies occurred. The first resulted in abortion
at fifth month; the second, in abortion at seventh month; the third, in
a stillbirth; the fourth, a syphilitic child dying in one month; the
fifth, in a syphilitic child dying in forty-five days; the sixth,
seventh, and eighth, in living, healthy children. To me the most
interesting fact in the whole relation is that during a portion of the
time, and immediately after the last three pregnancies, which resulted
in the birth of healthy children, both husband and wife manifested
grave tertiary syphilitic symptoms--gummata, tubercles, ulcers, etc.
This is direct evidence of the strongest kind in favor of the view that
syphilis ceases to be transmissible by heredity at the end of a certain
period, as we know that it ceases to be contagious or inoculable.

[Footnote 12: _Essai sur l'Hérédité de la Syphilis_, Thèse de Paris,
1867, p. 91.]

M. Ricord long ago,[13] and even before him Astruc and Doublet,[14] had
{259} promulgated the same theory, Ricord asserting that in the
tertiary stages the only effect of the disease upon the children was so
to modify their organization and temperament as to expose them to
developments of a scrofulous character--a view of the relation between
syphilis and struma which has been so ably supported in our own day by
Professor Gross.

[Footnote 13: _Traité pratique des Maladies vénériennes_ Paris, 1838,
p. 644.]

[Footnote 14: Legendre, _Nouvelles Recherches sur les Syphilides_, 1841
(quoted by Belhomme et Martin).]

M. Bazin also[15] denies absolutely that tertiary syphilis is any more
transmissible than it is communicable in other ways, although he fails
to give his reasons for this belief.

[Footnote 15: _Leçons sur les Syphilides_, 1859, p. 35.]

Hill and Cooper state[16] that the transmissive power continues as long
as the secondary eruptions are present, but usually ceases when the
tertiary stage is reached.

[Footnote 16: _Syphilis and Local Contagious Disorders_, London, 1881,
p. 62.]

Van Buren and Keyes[17] believe that fathers with tertiary syphilis
certainly, as a rule, procreate non-syphilitic children; and in
speaking of the fact that when the mother has syphilis the child is
generally infected, they except the later tertiary stages.

[Footnote 17: _Genito-urinary Disease and Syphilis_, 1874, p. 521.]

Bumstead[18] and Taylor say that without mercurial treatment the danger
of transmitting the disease to offspring usually persists up to the
fourth year of syphilitic contagion.

[Footnote 18: _Venereal Diseases_, 1879, p. 739.]

Mr. Lane says:[19] "It is certainly the rule that when the parents have
fully reached the tertiary stage the children born to them are free
from all signs of syphilis."

[Footnote 19: _Lectures on Syphilis_, London, 1881, p. 65.]

Mr. Hutchinson says:[20] "It is almost an acknowledged law that parents
in the late tertiary stages do not transmit taint."

[Footnote 20: _The Med. Press and Circular_, Aug. 2, 1882, p. 85.]

It will be seen from the foregoing extracts, which might be greatly
multiplied,[21] that there is a strong tendency on the part of many
authors to limit more or less strictly the period of transmissibility
of syphilis even when the disease is allowed to progress without
treatment. As to the facts that it becomes milder with time, both in
parents and offspring, that it ceases to be conveyed from husband to
wife or vice versâ, that with each succeeding year after the
termination of the secondary period the chances of escape of the
product of conception increase in a rapidly augmenting ratio,--there is
no difference of opinion whatever. Neither is it seriously disputed
that the length of time during which the disease remains active, as
well as the degree of its activity, may be markedly and beneficially
influenced by the administration of mercurial treatment. Under proper
medication patients who have rashly or disobediently married in the
height of the secondary period have been enabled to escape the danger
of transmission either to spouse or offspring--have, in fact, had
children born healthy and who never subsequently manifested any
symptoms of the disease.

[Footnote 21: M. Diday, _Traité de la Syphilis des Nouveau-nés_, Paris,
1854, p. 183; M. Bertin, _Traité de la Maladie vénérienne chez les
Nouveau-nés_, Paris, 1870, p. 142; M. Bazin, _op. cit._, p. 164; M.
Roger, _L'Union Médicale_, 1865, t. i. p. 147 (quoted by Fournier).]

I may add that my own experience seems to confirm the views which have
thus been set forth. I have notes of all my cases occurring in private
practice in a large city--some of them, I regret to say, among personal
friends or acquaintances, some of them in our own profession--and have
repeatedly {260} given permission to men to marry or to resume marital
relations after three years or three years and a half of mild mercurial
treatment, to which during the last six months or a year had been added
iodide of potassium. In many instances healthy children have been born:
in none, so far as I know, has the wife or mother been directly
infected. There have been a few doubtful cases in which premature
deliveries or stillbirths have occurred, but in nearly every such
instance there seemed to be other and entirely competent causes for the
accident; and in none of them, as I learned from the father or from the
obstetrician in attendance, were the children the subjects of
unmistakable syphilitic symptoms.

As to the exact time at which it is safe to permit marriage, and as to
the proper treatment before and after that event, it is hardly possible
in an essay like this to enter into many details. Yet so much is
involved in the answer to our first question that it may not be
altogether out of place here to indicate briefly the views of the
writer as to general methods of treatment. This is the more proper
because in every case of suspected syphilis in a new-born child, in
every case of threatened or actual abortion or miscarriage in the wife
of a man who has at some time in his life had syphilis, these questions
will present themselves, and the answers to them will greatly influence
not only the diagnosis and prognosis, but even the treatment, of such
cases.

1. In the first place, then, the diagnosis of syphilis should have been
assured. No venereal sore can with certainty be pronounced to be
syphilitic before the occurrence of general constitutional symptoms,
either the early cutaneous eruptions or at least the general glandular
involvement. Treatment begun prior to these developments leaves the
whole case open to the suspicion of mistaken diagnosis.[22]

[Footnote 22: "It is unsafe to predict confidently that any venereal
ulcer, even a soft sore attended with suppurating bubo, will entail no
further consequences. There is a strong probability that an indurated
sore will prove infecting; and there is a probability, though not
nearly so strong, that a soft suppurating sore will not; but exceptions
to both these general rules will be met with, and there is really no
absolute proof of the infecting nature of any given sore but the fact
of infection itself" (_Lectures on Syphilis_, James S. Lane, London,
1881, p. 23).]

2. The drug which should at once be begun when the character of the
case is fully recognized is mercury in one of its various forms. It may
be given by the mouth, by inunction, by vaporization, by hypodermic
injection, according to the preferences of the physician or patient;
but, however administered, it should be given in sufficient
quantity--_i.e._ in each case the full physiological dose of that
particular patient should be employed. To ascertain this the amount
used should have been gradually increased until commencing symptoms of
salivation are produced, when it should be diminished about one-half.

3. The quantity which has been thus determined should be given
continuously, or stopping only for the management of intercurrent
complications, for at least eighteen months. If during this time new
syphilitic symptoms make their appearance, the dose should be
temporarily raised until they have vanished, when it should be brought
down again to the original amount.

4. At the end of eighteen months or two years small doses of iodide of
potassium should be added to the mercurial, and this mixed treatment
should be persevered in for six months or a year longer, or should be
{261} still further prolonged if during that time any evidence of
syphilis is seen.

5. At the end, then, of from two to three years, if no symptom has been
seen for six months or a year, treatment may be stopped and the patient
kept under observation for a year; and if during that time no symptom
develops he may consider himself as in all probability cured. Any
course of treatment less thorough than this should be set down as
insufficient to afford any reasonable presumption of further immunity
from the disease.

There is evidence to prove, on the other hand, that this plan of
treatment, rigidly carried out, results in the majority of cases in
curing the disease, or at any rate in putting the patient in such
condition that he may with safety marry and may expect to have healthy
children.

To recapitulate: Syphilis after a certain period, not extending much
over four years where the disease is allowed to run its own course, and
probably much reduced by treatment, ceases to be a contagious disease;
and at about the same time or some time after loses, in the majority of
cases, its capability of being transmitted.

As there are probably exceptions to the rule that this power of
transmission disappears spontaneously within any specified time, it is
never safe to trust altogether to the unaided efforts of nature, but a
vigorous and sufficient specific treatment must be employed.

Given, however, the lapse of a sufficient time--say from three to four
years as a minimum--the history of a proper and continuous plan of
treatment, and the absence for a year or more of any specific symptoms
whatever, and the risks of marriage are so reduced as probably to
warrant a careful physician in permitting it.[23] And conversely, of
course--and this constitutes the reason for introducing the foregoing
matter into a paper on hereditary syphilis--in any doubtful case where
such a history can be elicited, and where all these precautions have
been observed, it is improbable that any taint of syphilis has been
transmitted.

[Footnote 23: This refers, of course, to an ordinary case of syphilis.
If the symptoms have been unusually grave, if the deeper tissues or the
viscera have been seriously involved, if cerebral or spinal
complications have occurred, the situation is of course much more
grave, and no step should be taken without the most thoughtful
deliberation. The work of Fournier already alluded to (_Syphilis and
Marriage_) furnishes an admirable guide under these circumstances.

Mr. Frederick Lowndes, surgeon to the Liverpool Dock Hospital
(_Lancet_, July 8, 1882), says: "Each case must be judged on its own
merits. When the constitution is good, and there has been sufficient
specific treatment, marriage may be permitted within a much shorter
period than M. Fournier suggests, and with safety. Syphilis alone and
syphilis combined with scrofula are two very different foes to contend
with, and if our patient be of a scrofulous temperament a delay even
longer than M. Fournier's may be desirable."

He quotes Dr. Thomas Edward Beatty in an address at the annual meeting
of the British Medical Association at Leeds in 1869: "Mercury given to
the man when first diseased would, I firmly believe, have prevented
this terrible calamity--_i.e._ the syphilitic infection of the wife;
and I would now humbly suggest to all who undertake the treatment of
venereal disease that if they have a certainty that their patients will
remain celibate all their lives, they may heal up their sores and
dispel their eruptions and sore throats in any manner they like, but
that they have no right to expose the pure, innocent, high-minded
females of society to contamination by marrying men treated without
mercury."]

Beyond this in positiveness of assertion it is not safe to go. There
may be exceptions to these as to most other hygienic or therapeutic
rules, but they will surely be of excessive rarity.

{262} Before considering the methods by which syphilis can reach the
child from one or the other of its parents, it may be well to mention
the modes in which they can infect each other.

The father can derive syphilis from the mother only in the usual
way--_i.e._ by contagion through a breach of surface permitting of the
direct absorption of the poison, the development of the disease being
attended by the usual phenomena--chancre, lymphatic enlargement, skin
eruptions, etc. The woman may--and in the vast majority of cases
does--acquire the disease from the husband in a similar manner. But
there seems to be good reason for believing that she may also become
infected through the medium of the child, who receives its syphilis
directly from the father, the mother up to the time of conception
having escaped contagion. More than this, it appears to be highly
probable[24] that no woman ever bears a syphilitic child and remains
herself absolutely free from the disease.

[Footnote 24: The strongest argument against the theory that every
woman who has had a syphilitic child has herself been infected lies in
the existence of cases like the following, several of which have been
observed: The wife of a man having active but untreated syphilis gives
birth to one or two syphilitic children, she herself developing no
symptoms. Later, the husband is placed on mercurial treatment. She then
conceives and gives birth to a healthy child. He stops treatment, and
she again bears a syphilitic child, which on his resuming his mercurial
course is followed by another healthy infant. Such cases certainly
indicate that the syphilis of the mother, if any exists, is incapable
of transmission to the child, as the effect of the treatment of the
father is too direct and unvarying to admit of doubt.]

The existence of this form of infection--syphilis by conception--has
been vehemently denied by many authors[25]--by some on the theoretical
grounds that as the essential elements or carriers of the syphilitic
virus have been shown to be cells or protoplasmic particles, and as,
after conception, the embryo is supplied with serum, but not with cells
of any kind, it is impossible that syphilis can be conveyed either to
or from it;[26] by others on reported observations of numerous cases in
which mothers who have been delivered of syphilitic children have shown
no evidences of the disease.

[Footnote 25: Kassowitz, Von Baerensprung, Bidenkap, and others.]

[Footnote 26: Bumstead and Taylor, _op. cit._, p. 742.]

It would appear, however, that, setting aside arguments based on
theoretical considerations, the weight of clinical evidence is
altogether in favor of the frequent, if not the invariable,
contamination of the mother through the medium of the foetus. No
physician of large experience in this class of cases can fail to have
seen some in which the husband, having had syphilis and having married
after an insufficient interval or an imperfect course of treatment, has
infected his wife with the disease, although at the time no
discoverable symptom is to be found upon his body--no abrasion, sore,
mucous patch, no lesion of continuity or suspicious point of any
description. An equally careful inspection of the woman will also in
such cases be attended by negative results--no initial lesion, no spot
of induration, no adenopathy being at all discoverable--and yet she
will be found with unmistakable evidences of constitutional
syphilis.[27] There is a clue to all such cases which will immediately
resolve the difficulty. In every instance, providing that no mistake
has {263} been made and that both husband and wife are really free--the
one from any contagious lesion, the other from any evidence of a
present or previous primary sore--it will be found that pregnancy has
occurred; that the woman has either been delivered of a syphilitic
child or has had an abortion or miscarriage at some time before the
outbreak of the symptoms of syphilis. Although I am firmly convinced
that this is a statement of facts based upon careful clinical
observation, and although this view has received the unqualified
endorsement of no less an authority than Fournier,[28] it is yet
strongly combated by many excellent authorities. They say in reply to
the above arguments that the reported cases are open to just criticism,
that trifling and unnoticed lesions of the father--chafes or abrasions
almost microscopic--suffice to transmit it on his part; while as an
explanation of the supposed absence of the primary lesion in the mother
they plead the well-known difficulty of discovering it in women under
any circumstances.

[Footnote 27: For a typical case see _Colles's Works_, New Sydenham
Society, London, 1881, p. 253. From that date to this hundreds of such
cases have been observed, and it would be idle to refer to them.]

[Footnote 28: _Op. cit._, pp. 26-30. He confesses to complete ignorance
as to the precise method by which this contagion takes place, whether
by poisoning of the fecundated ovule at the moment of conception (the
theory of Von Baerensprung) or by exchanges of the utero-placental
circulation, and regards the various hypotheses upon these points as
without practical value.]

Another argument, however, which seems to me to be unanswerable lies in
the application to the case in question of the well-known "law of
Colles," which from the date of its first enunciation by its
distinguished expounder in 1837 down to the present day has been found
to be absolutely without exception. I know of no other statement in
reference to disease which is at once so sweeping and comprehensive in
its bearings and so completely substantiated by clinical experience. It
may be given in his own words: "One fact well deserving our attention
is this: that a child born of a mother who is without obvious venereal
symptoms, and which, without being exposed to any infection subsequent
to its birth, shows this disease when a few weeks old,--this child will
infect the most healthy nurse, whether she suckle it or merely handle
and dress it; and yet this child is never known to infect its own
mother, even though she suckle it while it has venereal ulcers of the
lips and tongue."[29]

[Footnote 29: _The Works of Abraham Colles_, edited by Robert
McDonnell, the New Sydenham Society, London, 1881, chap. xiii. p. 287.]

As to the absolute and unvarying truth of this law there is no excuse
for a shadow of doubt. To quote Mr. Hutchinson: "It has received the
assent of every authority who has written on the subject since it was
announced. It has attracted attention both at home and abroad, and I am
not aware that a single exception to it has been recorded.[30] We have
all of us seen chancres on the nipples of wet-nurses. They are, indeed,
not very infrequent. We have, however, none of us seen such on those of
the mothers of infected children. Let us remember that it is very
unusual to put a syphilitic infant out to wet-nurse--a thing which no
prudent physician would ever permit--and that, probably, for one so
nursed a hundred are suckled by their mothers, and we can appreciate
the weight which this entire absence of proof that mothers ever suffer
{264} bears. It amounts, I think, to all but proof that they are
absolutely insusceptible."[31]

[Footnote 30: The cases of Cazenave (1847), Cocchi (1858), Müller
(1861), Ranke (1878), Guibout (1879), Scarenzio (1880), and Zingalès
(1882), are defective in important particulars, the first two so much
so as to render them valueless. Ranke's case is by far the strongest
yet recorded, but lacks fulness and rests upon his unsupported
testimony. (See Hill and Cooper, _op. cit._, p. 55, and _Nouveau Dict.
de Méd. et de Chir._, vol. xxxiv. p. 687.)]

[Footnote 31: Dr. J. N. Hyde suggests (_Archives of Dermatology_,
April, 1878, p. 103) that "the full weight of Colles's law is to be
estimated in connection with the question whether the child whose
hereditary syphilis is derived from the mother exclusively is capable
of infecting its healthy father; and if no instance of this latter can
be adduced a higher law becomes defined--viz. that the child whose
hereditary syphilis is transmitted by one parent only is incapable of
infecting either." He explains this hypothetical immunity, in which he
seems to believe, by saying that "it is probably due to the fact that
the syphilis-bearing cell-element cannot readily be implanted upon the
soil from which it sprang--a fact illustrated by the infecundity of
consanguineous marriages and the non-auto-inoculability in general of
the primary lesion of syphilis." When, however, we exclude the large
number of cases in which the father is already syphilitic, and remember
that in the others the contact between him and the child is slight,
infrequent, and fleeting--being usually limited to an occasional kiss
or caress--it does not seem strange that no instances of such infection
have been reported; nor does the fact seem at all worthy of being made
the foundation of a general law.]

There can be but one rational explanation of these facts--viz. that the
mothers who have thus acquired an immunity have done so by first
acquiring the disease. It may, in those cases where no secondary
symptoms appear, be in a modified form, due, as suggested by Mr.
Hutchinson, to some heteromorphism or alternation of generations on the
part of a specific fungus, or to the excessively small quantity of the
poison which finds its way from child to mother, or to some other
obscure cause with which we need not now concern ourselves. It is, at
any rate, no more mysterious than the protective influence of vaccine
in small-pox, and when one is rationally explained doubtless the other
will be found to be closely allied in its mode of action. The fact
which interests us at present is that it is in the highest degree
improbable that anything but some form of syphilis itself could afford
this entire protection, and that it is not in the least unreasonable,
but, on the contrary, logical and consistent with all the known facts,
to suppose that while in some cases no observable symptoms might be
produced, in others where the cause was the same, but more active, or
the powers of resistance less, the usual constitutional phenomena would
be developed.

We may conclude, then, that the husband may infect his wife--(1) In the
usual manner or by direct contagion; (2) through the medium of the
child, or at any rate by the production of conception.

The theory upheld by Von Baerensprung, that the syphilis of the mother
is imparted to her at the moment of impregnation, the disease being
impressed upon the fecundated ovule, does not materially conflict with
the above views, conception being in either case the essential factor,
but in the latter instance the intervention of the foetus itself not
being necessary. It has to support it the clinical fact that in those
cases where syphilis appears during pregnancy the outbreak of symptoms
occurs at about the ninth or tenth week after the date of conception,
or a period which closely corresponds to that of the appearance of
general symptoms after exposure to ordinary contagion--allowing about
three weeks for the so-called incubation of the chancre and six weeks
for the secondary incubation.[32]

[Footnote 32: This has been shown not only by Von Baerensprung (_Die
Hereditäre Syphilis_), but also by Diday, whose observations were
intended to prove the possibility of syphilis being derived from the
child by the mother--"choc en retour." In 24 cases the period at which
the first eruption appeared in the mother averaged sixty-five days
after conception; only once did the first signs appear after the fourth
month of pregnancy.]

{265} It may also be said to be rendered probable by the following line
of argument:

Colles's law, which is without exception, demonstrates that every woman
who has had a syphilitic child has been herself infected, even if she
has had no observable symptoms;[33]

Cases are recorded, however, in which a woman having given birth to one
or more syphilitic children, and therefore herself syphilitic, bears
healthy ones in consequence of specific treatment administered to the
father before and during the period of conception, she remaining
untreated;[34]

The determining cause, therefore, of the syphilis of the child is not
the syphilis of the mother, but the condition of the fecundating germ
of the father; and, as a corollary,

The determining cause of the syphilis of mothers in whom the disease
follows conception is not by infection from the foetus through the
utero-placental circulation[35] or otherwise, but is the diseased male
procreative cell which becomes blended with the female ovule.[36]

[Footnote 33: It is obviously no explanation of the law of Colles to
say that "it would seem to indicate that the escape of the mother is
due to some occult, undiscernible change in her system" (Bumstead and
Taylor, _op. cit._, p. 745).]

[Footnote 34: See foot-note, p. 262.]

[Footnote 35: On account of the absence of cellular elements in the
fluid interchanged.]

[Footnote 36: An elaborate paper by Fraenkel (_Archiv für
Gynaekologie_, 1873, vol. v. p. 1), based on twenty-one cases of
childbirth, was written to prove by the condition of the placenta that
direct infection of the child by the father was possible without the
participation of the mother, and that when the latter became infected
it was through the medium of the child (_choc en retour_). In fourteen
of his cases the specific change in the placenta began in the foetal
portion or affected it exclusively. These cases, however, as analyzed
by Hill and Cooper (_op. cit._, p. 57), are altogether defective in
important particulars. Of course to sustain his theory there should be
indubitable evidence that the fathers were syphilitic, and that the
mothers were not so. So far from this being the case, but one of the
fathers was known to be syphilitic, while two of the mothers had
evidences of constitutional syphilis at the date of childbirth, and
nine others had markedly suspicious histories. The lesions of the
placenta which are thought to be syphilitic consist in the development
of papular or gummatous growths which give rise secondarily to
inflammatory troubles affecting either the placenta itself or the
placenta and the uterine mucous membrane.]

If the premises are admitted the conclusions seem irresistibly to
follow.

There is no proof whatever that the semen of a syphilitic man is
contagious or can transmit the disease in any but the way above
discussed. On the contrary, it has been shown experimentally[37] that
it is entirely innocuous and non-inoculable.

[Footnote 37: Mireur, _Annales de Derm. et Syph._, 1876, p. 77.]

All other theories as to methods of contagion are so entirely
hypothetical and unsupported by trustworthy evidence that we can afford
to disregard them.

We may now consider the ways by which syphilis reaches the child, and
they may be broadly classified into--

1. By descent from the father.

2. By descent from the mother.

3. By direct infection.

As a matter of course, the influence of the father upon the child, so
far as regards heredity, ceases at the moment of conception; or, to be
more exact, no subsequent condition of the male parent, no development
or acquirement of disease, can exert any further effect. That the
existence of active syphilis in the father may result in the
transmission of the malady to the child can hardly be doubted. To be
sure, there are numerous {266} examples of cases where both wife and
child have escaped though the husband showed at the time of conception
very active secondary lesions. These, however, are exceptions, and do
not in the least invalidate the rule that it is possible for a father
to hand the disease directly to his child. There is no other possible
interpretation of the cases already alluded to in which successive
pregnancies in the same woman alternately resulted in healthy or in
syphilitic children according as the father was or was not kept under
specific treatment. Other arguments might be advanced, but that seems
to me conclusive.

The relative effect of paternal as compared with maternal influence may
be considered after we have described the latter.

Descent from the mother may occur theoretically in consequence of--

1. Infection of the mother previous to conception.

2. Infection of the mother at the moment of conception.

3. Infection of the mother during the period of utero-gestation.

As to the first of these methods of transmitting the disease there is
little if any difference of opinion. Even those who claim the most for
paternal influence[38] include among the conditions which may give rise
to syphilis in the child disease of the ovule, and it may be stated as
incontrovertible that recent or active syphilis in the mother at the
time of conception will almost certainly be followed by syphilis in the
child. As a rule, women who have borne syphilitic children, even when
they do not give unmistakable evidence of the disease, fail in health,
become anæmic, and often develop glandular or osseous swellings which,
according to Zeissl,[39] are only relieved by antisyphilitic treatment.
The bearing of Colles's law upon the alleged immunity in many instances
of the mothers of syphilitic children has already been shown (p. 264);
and there is other evidence, not perhaps so conclusive, but strongly
corroborative, of the same view--viz. that their escape is only
apparent, and that syphilis, either latent or active, always affects
such mothers.[40] There can be no manner of doubt that in every
instance at or about the time of childbirth there are not to be found
pathognomonic lesions of syphilis, nor do such lesions always make
their appearance in cases where the period of observation is a short
one; but it is claimed with much show of truth that prolonged and
patient inspection of such patients will in time result in the
discovery of some symptom which betrays the presence of the disease.

[Footnote 38: Bumstead and Taylor, _op. cit._, p. 745. Hutchinson in
Reynolds's _System of Medicine_, Am. ed., vol. i. p. 431: "In a large
proportion of the cases met with in practice the taint is derived from
the father only." (On this point see foot-note to p. 270.) If there
were any doubt as to the fact that syphilis in the mother only may be
transmitted to the child, it would be removed by the cases of Bardicet
and others mentioned in _Nouv. Dict. de Méd. et Chir._, vol. xxxiv. p.
688. In these cases nurses who had contracted the disease from their
sucklings subsequently became pregnant (without having infected their
husbands) and gave birth to syphilitic children.]

[Footnote 39: _Jahrbuch_, vol. ii. p. 303, 1872.]

[Footnote 40: Zeissl, Sigmund, Oewre, Flindt, Woodman, and others are
quoted by Hill and Cooper to this effect. Woodman, for other purposes,
gave the histories of 200 cases of infantile syphilis. In all of these
the mothers had suffered from typical secondary lesions.]

The cases in which treatment of the father has resulted in healthy
children, whereas without treatment he procreated only syphilitic
children, the mother being without either symptom or treatment, have
been urged as evidence of the direct descent of syphilis from the
father to the child without the intervention or participation of the
mother. Doubt {267} has been thrown upon them by those who uphold the
contrary view;[41] but after looking into them carefully I am
constrained to admit that some of them, notably those of Kassowitz[42]
and R. W. Taylor,[43] are convincing of the facts--(1) that treatment
of the father controlled the condition of the child, and (2) that there
was no evidence that the mother had syphilis. But we have seen that the
only proof of the universal infection of mothers of syphilitic children
is the law of Colles, and that in many cases the disease is for a long
time latent or unrecognizable. Let us admit that this was the condition
in the cases in question; it does not at all follow, necessarily, that
because the mother has latent or hidden syphilis she must infect her
child. Every case even of active syphilis in the parents is not handed
down to the children, a certain proportion of whom escape even when
both parents are in the height of the secondary stage at the time of
conception.[44] But the activity of the disease in the children, and
even more the likelihood of its reaching them, are in direct proportion
to its activity in the parents.[45] A mother, therefore, who under the
influence of active syphilis in her husband has given birth to two or
three syphilitic children, and has herself shown no symptoms of the
disease, may nevertheless have it in the latent form and have no strong
tendency to transmit it. Consequently, treatment of the father will
result in the procreation of healthy children, because it removes the
active and efficient cause of their infection. To take any other view
of these cases is to assume that every syphilitic parent must hand down
the disease to the children--an assumption which is not in consonance
with numerous well-attested clinical facts.

[Footnote 41: Hill and Cooper, _op. cit._, pp. 52, 53.]

[Footnote 42: "Die Vererbung der Syphilis," _Stricker's Med. Jahrb._,
1875, p. 391.]

[Footnote 43: _Archives of Clinical Surgery_, New York, Sept., 1876.]

[Footnote 44: Fournier, _op. cit._, pp. 35-37.]

[Footnote 45: Hutchinson, _op. cit._, p. 431.]

Leaving this interesting question, however, we may consider the other
methods by which syphilis descends from the mother to the child, having
seen already that it is beyond doubt that it may be handed down by
disease of the ovule due to syphilis acquired previous to conception,
and having seen that there is a high degree of probability that the
mother herself rarely, if ever, escapes the disease. The second method,
or that in which the mother becomes syphilitic at the moment of
conception, has already been sufficiently discussed. It is really,
strictly speaking, an example of paternal heredity, as the resulting
germ is syphilitic--not because the ovule of the mother was infected,
but on account of the disease of the spermatozoid of the father.

There remains for consideration the influence upon the child of a
syphilis acquired by the mother during some period of utero-gestation.
That under these circumstances the child can become infected has been
and is still absolutely denied by some very respectable
authorities.[46] All that is {268} necessary for proof of its
occurrence is, however, (1) freedom of both parents from syphilis at
the time of conception, or, in other words, syphilis must have been
acquired by both--not alone by the mother--after the beginning of
pregnancy; (2) that the syphilis of the child be unmistakably
pre-natal--that is, not acquired by some accident during or after
birth.

[Footnote 46: Bumstead and Taylor, _op. cit._, pp. 742, 744. They base
their denial, first, on the physiological fact (?) that no interchange
of cellular elements between mother and father is possible, and next on
the absence of satisfactory evidence of the occurrence of infection
during pregnancy. Zeissl's case seems sufficient answer to the last
assertion, and there is strong evidence that the first is without good
foundation. "The placenta is penetrated by the virus, and does not play
the part of a filter for the elementary particles of matter which, so
far as we know, represent the true active contagion of the disease. In
the absence of direct experiments, which it would be almost impossible
to institute, we may argue from the facts known to exist in certain
acute infectious diseases in which there are very interesting points of
resemblance.

"It has been known for a long time that small-pox occurring in the
mother may be transmitted to the product of conception enclosed in the
uterus, and it is supposed that the virulent particles traverse the
walls of the maternal vessels in order to penetrate the circulating
apparatus of the foetus. But what is only a supposition in the case of
small-pox seems to have been actually demonstrated in symptomatic
charbon (Arloing, Cornevin, and Thomas), bacteridian charbon, and
recurrent fever, in which the poisonous element is easily recognized.
The recent experiences of Strauss and Chamberland (1882) have shown
that the foetus participates in the 'infection charbonneux' of the
mother. Albrecht has shown (1880) the presence of numerous spirochoetæ
in the blood of the heart of a child born at seven months of a woman
with a second attack of relapsing fever. It may be supposed, therefore,
though not demonstrated, that the transmission of syphilis takes place
by the same method as that of relapsing typhus or of charbon" (_Nouveau
Dict. de Méd. et de Chir._, pp. 682, 683).]

The following case,[47] reported by a most accurate observer, seems to
combine both these requisites. Zeissl the younger reports that O. X.,
thirty-six years old, never having had syphilis, left his wife, to whom
he had been married two years, to go a journey on July 15, 1877. The
wife was then in the second month of her first pregnancy. On July 24th
O. X. had extra-marital intercourse. About twenty-one days after this
coitus he observed a small lump on the inner surface of the foreskin,
and on Aug. 22d he consulted Zeissl the elder. On Sept. 23d a
maculo-papular eruption of the skin with erythema faucium appeared.
Under treatment these symptoms completely disappeared. On Oct. 29th he
went home to fetch his wife to Vienna for her lying-in, and had
intercourse with her soon after his return, notwithstanding Zeissl's
strict prohibition. At the beginning of December a hard sore developed
on the left nympha of the wife, who was then in the seventh calendar
month of her pregnancy. At the end of December a maculo-papular
eruption spread over the body and was treated with mercury. On Feb. 14,
1878, a well-grown and apparently healthy female child was born at full
term. When eleven days old[48] a pustulo-scaly eruption came out on the
child's soles and toes, and soon afterward a maculo-papular eruption
over the body generally. A few days later the child died. No
post-mortem examination was permitted. In July, 1878, the wife had
iritis, and after that gummata on the leg. She miscarried in July,
1878, at the third month, and again in February, 1879, at the second
month.

[Footnote 47: Quoted by Hill and Cooper, _op. cit._, p. 60.]

[Footnote 48: Of course much too early for constitutional symptoms if
the disease had been acquired during or after birth.]

There seems to be no reasonable escape, after reading this carefully,
from the conclusion that in some manner the poison of syphilis found
its way from the mother to the child. The old idea that the latter was
directly infected in utero from the semen of the father is altogether
without foundation. Other cases equally satisfactory and complete have
been reported, and, unless the intelligence or the truthfulness of the
observers be impugned, establish without doubt the possibility of
infection during utero-gestation.

In the above case the contagion of the mother occurred in the seventh
{269} month of pregnancy; and this, I believe, is as late as it has
ever been known to be communicated to the child. The exact date at
which it becomes impossible so to transmit it is unknown, but as a
general rule it may be said that the earlier a mother is infected
during gestation the less likely is it that the child will escape.
Treatment of the mother--as of the father in cases where he is at
fault--very greatly modifies the whole problem and adds immensely to
the chances that the child will not be infected.

Direct infection of the child during birth could not properly come
under the head of hereditary syphilis. There is no possible reason why,
when the mother has contagious lesions of the genitals, acquired too
late to infect the child in utero, this should not occur, but as a
matter of fact no such case has ever been recorded. One explanation of
this circumstance may be found in the protective covering of vernix and
mucus which coats the infant's body and lessens greatly the risk of
absorption. This hardly accounts satisfactorily, however, for the
entire absence of such cases from medical literature, and it is fair to
suppose that in all but those cases in which the primary sore is
acquired during the last month of gestation--which for obvious reasons
are excessively rare--the infant acquires some immunity which protects
it from its mother, and is similar to that which, under Colles's law,
operates in her favor. In other words, even though apparently free from
syphilis at birth--a not uncommon event, as we shall see--it has a
latent or modified syphilis which protects it from contagion.

We may now briefly restate the conclusions at which we have thus far
arrived:

1. After a certain interval, not less than four years, and after
thorough specific treatment, a person who has contracted a syphilis not
especially severe or malignant in its type may be permitted to marry.
The assent to marriage will then be based on a belief in the curability
of syphilis or the cessation of its contagiousness, its inoculability,
and, in the vast majority of cases, its transmissive power at the end
of the secondary stage.

2. It may be inherited from either parent or from both, and the
probability that this will occur increases in a direct ratio with the
nearness of the time of conception to the date of their infection with
the disease. The severity of the inherited disease in the child
increases in the same proportion.

3. It is undoubted that, the father being healthy and the mother
syphilitic, the child may, and in all probability will, have the
disease.[49]

[Footnote 49: Dr. Sturgis, who disbelieves altogether in the
possibility of paternal heredity, concludes, after examining the
subject carefully, that (1) a mother begets non-syphilitic children as
long as she is not infected, even though the father is syphilitic; and
(2) the moment she is diseased the children are inevitably so (Paper on
"The Etiology of Hereditary Syphilis," _New York Medical Journal_,
July, 1871). This doctrine was previously supported by M. Cullerier,
whose views gave rise to the remarks of M. Voillemier (quoted by
Fournier) that if they were accepted "the father would be only the
accidental occasion of a child; one would be, in reality, the child of
his mother only." Cullerier's cases are invalidated by the fact that
the syphilitic fathers who had healthy children had been subjected to
mercurial treatment (_Mém. de la Société de Chirurgie_, Paris, 1854,
quoted by Taylor in _Archives of Clin. Surg._, vol. i. p. 83). The
theory is a very old one. Vassal has sustained this idea as long ago as
the end of the last century. Kostum (1804), and after him Hufeland,
were of the same opinion. Cullerier (1857) wrote: "In order that a
child acquire syphilis hereditarily it is necessary that the mother is
or has been {270} syphilitic." Notta, Follin, Charrier, Mireur (1867),
and Langlebert (1873) support this theory more or less earnestly. Oewre
wrote (1873): "Paternal influence is nil as regards hereditary
syphilis." Isseff (1879) wrote: "Where a man suffers or has suffered
from syphilis he cannot transmit the disease to his descendants without
infecting his wife; that is to say, in fewer words, there is no
infection from the father." Sigmund says: "The heredity of syphilis is
derived in its last analysis from the mother" (_Nouveau Dict. de Méd.
et Chir._, vol. xxxiv. p. 689).]

4. It is probable, but less so,[50] that, the mother being healthy and
the father syphilitic, the child will be infected.

[Footnote 50: This refers simply to the comparative probability of
infection, and does not conflict with the statistical fact expressed by
Hutchinson (Reynolds's _System of Medicine_, vol. i. p. 431) in his
words: "In the large proportion of cases met with in practice the taint
is derived from the father only." This numerical predominance of
paternal influence is very readily explained. There are many more
syphilitic men than syphilitic women, and especially among the couples
who contract fertile marriages the number of women who are infected
before becoming mothers is inconsiderable. On the other hand, it
frequently happens that men who have had syphilis, but have been
without symptoms for a longer or shorter interval, marry and transmit
to a series of children a disease which has ceased to be directly
contagious to their wives, the transmissive power continuing after the
possibility of ordinary contagion has disappeared. As in the majority
of such women the disease is latent, and may be only displayed in their
immunity from infection, it becomes evident that, history and symptoms
both being wanting on their part, the conditions justify the assertion
of Mr. Hutchinson. (See _Nouveau Dict. de Médecine et Chirurgie_, p.
684.)

That assertion (quoted above) has, however, been thought by several
writers to indicate his belief in the escape of the mother. That I have
not misinterpreted him is evident from the following extract from an
article on "The Transmission of Syphilis," written by him (_Brit. and
For. Med.-Chir. Rev._, Oct., 1877): "I take it for granted (although I
know that there are still some who doubt) that it is possible for a
father to transmit the taint, the mother being at the time of
conception wholly free. I believe, indeed, that in practice this is by
far the most common way in which syphilis is transmitted. Whether in
these cases it is correct to speak of the inheritance being paternal
only is, as we have just seen, another matter, since it is possible
that in every instance the mother derives an infection from the father,
and may thus in turn influence it."]

5. It is highly probable, though it can hardly be considered as proven,
that in all cases where a child becomes syphilitic through paternal
influence the mother is also the subject of syphilis, which may,
however, assume a latent form, the only evidence of its presence in a
few cases being the protection which it affords against contagion
through the medium of the child.

6. Syphilis may be transmitted from mother to child even when it is
acquired by the former as late as the seventh month of utero-gestation.

Since writing the above the thirty-fourth volume of the _Nouveau
Dictionnaire de Médecine et de Chirurgie_ has been published. In the
article on syphilis seventeen pages are devoted to the question of
heredity, which is reviewed in a most thorough manner and finally
summed up as follows (p. 698):

"The most definite views which we possess on the subject of the
hereditary transmission of syphilis may be thus expressed:

"Children may be infected by heredity, not only when the two parents
are syphilitic, but also when only one, either the father or mother, is
diseased at the time of conception.

"When both parents are diseased at that time there is more certainty
that the child will be infected, and infected gravely, than if only one
of them has the pox.

"The hereditary disease is not always fatal, even when both progenitors
have actual specific symptoms. The more recent the disease of the
parents the greater the chances of their transmitting the disease and
of its assuming {271} a serious form. There is no proof that inherited
syphilis is more grave when derived from the father than when coming
from the mother.

"It is altogether exceptional for the mother, healthy at the moment of
conception, not to participate in the disease transmitted by the father
to the child. If she escapes direct contagion--which is rare when the
disease of the father is active--she undergoes a species of infection
from contact with the contaminated foetus.

"Syphilis by conception, which is thus transmitted from the foetus to
the mother, may present the usual characters and evolutions of the
acquired disease; frequently, also, it is latent, and is betrayed only
by the existence of immunity from further contagion on the part of the
mother. It may finally manifest itself by tertiary symptoms or by
systemic troubles without specific characters.[51]

"The power of transmitting syphilis hereditarily decreases
spontaneously as the disease of the parent becomes older. The influence
of treatment is no less certain than that of time. When the two
progenitors are at the moment of conception free from syphilis, the
foetus may still be infected if the mother acquire the disease during
her pregnancy."

[Footnote 51: "This form of syphilis shows itself in the mother in
three ways:

"1st. By the usual signs of syphilis by contagion, with the exception
of the primary sore, appearing about the sixty-fifth day after
conception.

"2d. Appearing at a later date as secondary or even as tertiary
symptoms, and preceded merely by a little disturbance of the general
health, unhealthy appearance of skin, falling of hair, etc., but
nothing truly specific.

"3d. Showing itself some years afterward in a tertiary form, having in
the mean while given no indication of its existence save only in the
protection it afforded against contagion from the child" (_Nouveau
Dict. de Méd. et Chir._, vol. xxxiv. p. 696).]

       *       *       *       *       *

Syphilis of the placenta is of especial interest in its relation to the
abortions and stillbirths so frequent in syphilis. Until the elaborate
paper of Fraenkel upon this subject (see foot-note, p. 265) almost
nothing was known about it. He describes[52] the macroscopic changes as
consisting of increased size and weight of the placenta, closer and
firmer texture of the placental tissue, the presence of old and recent
extravasations of blood in all stages, opacity and thickening of the
decidual covering and of the amnion and chorion, which are in places
adherent to each other. Microscopically, it was found that the
placental villi were filled with small nucleated cells, which were
especially abundant in the centre of the villous spaces along the axis
where the vessels usually take their course. The ends of the villi were
enlarged with knob-like processes.

[Footnote 52: I condense here from the translation of Fraenkel's paper,
which constitutes the bulk of chap. xxvii. of Bumstead and Taylor's
excellent work.]

Fraenkel's explanation of these changes is as follows: Under the
influence of syphilis[53] cell-proliferation begins in the villi, which
are, normally, only sparingly supplied with cells. These new cells
excite proliferation of the cells of the connective-tissue stroma and
of the epithelium. This proceeds to such an extent that it leads to
compression of the vessels,[54] interfering with the circulation, and
finally obliterates them. The {272} vascular spaces into which the
villi dip become filled up and narrowed, and often disappear. In this
way, and also by reason of the thickening of the epithelium, the
interchange between the maternal and foetal blood is interfered with,
and at last is prevented.

[Footnote 53: Fraenkel took as his criterion of syphilis the presence
of the osteo-chondritis described by Wegner (see p. 286).]

[Footnote 54: "Hennig was the first who called attention to the
intimate relation of the cell-growths to the vessels. The obliteration
or compression of numerous vessels of the villi interferes with the
mutual interchange of gases between the maternal and foetal blood,
causing fatty degeneration of the villi and, if the process is
extensive, the death of the foetus" (article "Syphilis," _Ziemssen's
Cyclopædia_, vol. iii. p. 237).]

If this process is spread over the whole placenta, the foetus perishes
before it is complete. If it is limited to circumscribed areas, it may
continue to live for a shorter or longer period.

These observations require to be repeated and confirmed, but they have
served to open up a most interesting branch of syphilitic pathology.

Syphilis in the parents will manifest itself in the children in one of
several ways, which are determined chiefly by two factors--viz. first,
the length of the interval between the infection of the parent and the
date of conception; and, second, the thoroughness of the treatment of
the parents during that interval. To these may be added as subsidiary,
but still of definite importance, a third, the type of disease which
has affected the father or mother, whether mild or severe, benign or
malignant.

From what has already been said in reference to the question of
marriage, it will be at once understood that the danger to the
offspring in untreated cases, and in those where conception has
occurred during the early secondary period of the disease, is of the
most extreme gravity.[55] In such cases the usual result of pregnancy
is abortion at from the first to the fifth or sixth month,[56] the
foetus sometimes exhibiting the evidences of the disease in the shape
of large bullæ upon the palms and soles, or in the presence of
characteristic visceral lesions, but quite as often showing nothing
distinctive. It has generally undergone more or less maceration, and
the skin, which is readily detachable, is of a congested, purplish
color.

[Footnote 55: According to Kassowitz, in women who are not treated all
pregnancies occurring within the first three years of their infection
terminate either in abortion or in the birth of children who survive
for only a few weeks or months.

Weber (quoted by Parrot, _Le Progrès Médicale_, Nov. 24, 1877, p. 882)
treated thirty-five pregnant women by mercurial inunction, and they all
went to full term under normal conditions. Among those treated with
mercury and iodide of potassium, but who, by reason of intolerance of
the former drug, took chiefly the latter, 20 per cent. aborted; when
the mixed treatment was carried out regularly 15 per cent. aborted; and
when only iodide of potassium was given 36 per cent. aborted. His
studies were made on 109 syphilitic pregnant women. Parrot himself says
(_ibid._) that "abortion occurs in about one-third of all syphilitic
women. Syphilis should always be suspected when this accident occurs
several times successively." "The date of abortion depends on the age
of the syphilis of the woman. It is most to be feared during the
evolution of secondary symptoms, particularly when they are grave.
There is more probability that it will occur when infection has
preceded pregnancy than when it occurs during its course. Nothing is so
much to be feared as contamination of the ovule. The nearer the date of
infection of the mother approaches to full term, the fewer the chances
of abortion. It is not likely to occur even when the mother is infected
at five months."]

[Footnote 56: Many observers think that abortion results directly from
the death of the foetus. Babington (notes to Hunter's _Treatise on
Venereal_), Trousseau, and Von Baerensprung were of this opinion.]

Dr. Cory thinks that in many cases it is possible that the effect of
syphilis may be to effect so early an abortion that the case is simply
regarded as one of delayed menstruation or of menorrhagia. Such a
conception would, however, be competent to infect the mother, and might
seem to explain cases otherwise involved in obscurity.[57] At least
one-third {273} of all syphilitic children are dead born.[58] As time
goes on, however, and the intensity of the parental disease is
lessened, or in cases where appropriate treatment has been applied,
either the abortion occurs at a later period of pregnancy or the
children are brought alive into the world. Even then, however, and
although at birth they may show no evidences of the disease, their
chance of escape is but small. One-fourth of them die within the first
six months. If they survive that period, the chances for life are
slightly in their favor, but those for health or freedom from deformity
and disease are still overwhelmingly against them.

[Footnote 57: As, for instance, when a woman married to a syphilitic
man, but without issue, remarries a man with no history of syphilis,
and yet gives birth to a syphilitic child. In such a case there would
be no history of direct infection and none of pregnancy, the only two
modes by which she could have contracted the disease, and the
father--the second husband--might be unjustly suspected.]

[Footnote 58: Kassowitz, _op. cit._]

The course of inherited syphilis differs strikingly from that of the
acquired disease. It will hardly be necessary to do more than remind
the reader of the ordinary stages of the latter affection--the primary,
which includes the period of the chancre and of lymphatic engorgement,
lasting about sixty to seventy days; the secondary, or exanthematic,
characterized by copious cutaneous eruptions and extensive involvement
of mucous surfaces, lasting from one to three years; the
intermediate,[59] or the stage of latency and relapses, lasting for a
very variable period, from three to ten years, but under proper
treatment very much reduced or altogether abolished; and finally, the
tertiary period, beginning four or five years after contagion,
extending indefinitely throughout life, but often in cases properly
treated absent altogether.

[Footnote 59: So designated by Mr. Hutchinson, and for clinical
purposes a very valuable addition to the periods of syphilis. He
describes it as follows: The patient may be either wholly free from
symptoms and in good health, or he may remain pale and rather feeble,
and liable from time to time to slight returns of eruption on the skin,
sores on the mucous membranes, condylomata, etc. He is protected as
regards fresh contagion, and should he beget children they are almost
certain to suffer. The relapses during this stage are usually easy to
be distinguished from true secondary symptoms. There is little or no
febrile disturbance, the rash is not copious, and often not
symmetrical. Acute iritis, retinitis, etc. never occur for the first
time, though they may do so in the form of relapses.]

For purposes of description and of contrast we may similarly divide the
whole period of evolution of a case of inherited syphilis,[60] omitting
the primary stage, which has never been found to exist in true cases of
hereditary syphilis. Of course in congenital or infantile syphilis, in
which by direct contagion, either from the mother or from any one else,
the disease was acquired by the child, the course would not differ
materially from that observed in the adult. But as this stage in all
probability corresponds to the period during which the poison is
already finding its way into the system through the lymphatics, of
course it is not found in the child who is infected from the moment of
conception or who receives the poison from the mother directly into the
circulation.[61] For from one to three weeks the infants often show no
symptoms of the disease. In 158 cases collected by Diday, 86 manifested
symptoms of the disease before the expiration of the first month, and
60 of the remainder before the end of the third month.[62] When to
these are added the {274} statistics of Roger, we find that of a total
of 172 cases, 159 showed syphilitic symptoms before the end of the
third month.[63] When the symptoms are present at birth, they consist
largely in a general withered, atrophied, weazened appearance of the
child; a hoarse cry, due to swelling, with subacute inflammation, or
even ulceration, of the laryngeal mucous membrane; a coryza,[64] due to
a similar condition of the Schneiderian membrane; and certain cutaneous
eruptions, the most common of which at this early date is the large
vesicular or bullous eruption known as syphilitic pemphigus.

[Footnote 60: The idea that the character of the symptoms which first
appeared depended upon and corresponded with the stage of the disease
in the parent has now, I believe, no supporters. It was once thought,
at least by some syphilographers, that if the parents were in the
tertiary stage at the time of conception the child would develop
tertiary symptoms, omitting the other stages.]

[Footnote 61: If chancre were the first symptom of constitutional
syphilis, why should it not appear in cases of hereditary syphilis?]

[Footnote 62: _Infantile Syphilis_, p. 101]

[Footnote 63: About 1823, from 16,000 to 17,000 children were admitted
annually to the wards devoted to foundlings at Lyons. Clièt, recording
his experience with this disease, says that syphilis is one of the most
common of their maladies, but that "it exceedingly seldom shows itself
at birth by evident signs" (_Compte-Rendu Méd. Chir. des Observations à
l'Hôpital général de la Charité de Lyon_, 1823). Cristoferi, physician
to the foundling hospital at Bologna, says that syphilis generally
manifests itself between the ages of one and three months. Never once,
he states, was a newly-born infant admitted with the disease
unquestionably developed (_Gazette Medica di Milano_, 1844). Trousseau
says that it "rarely appears before the second week, and very
exceptionally after the eighth month" (_Lectures on Clin. Med._, vol.
iv. p. 331, London, 1871, ed. of New Sydenham Society).]

[Footnote 64: Usually appears later, but exceptionally at birth.]

Pemphigus.--With regard to the specific or non-specific character of
this eruption there has been much difference of opinion, and, as it is
often the earliest distinctive expression of syphilis, a diagnosis of
which could hardly be founded on the general appearance of the child,
or even on the hoarse cry and the coryza, it becomes important to have
definite ideas upon the subject. Nearly a century ago (in 1794) it was
denied[65] that this eruption was a manifestation of venereal disease;
and this view has been supported by many able and accurate observers
down to the present day. In 1851 a discussion upon the subject took
place in the French Academy of Medicine, which elicited the opinions of
the majority of those members who were entitled to speak with authority
in the matter, and which has since been referred to by most writers.
Cazeaux upheld the non-syphilitic hypothesis on the ground (1st) that
the so-called syphilitic pemphigus of children does not differ from the
simple pemphigus of adults, presenting none of the physical characters
which distinguish the specific cutaneous eruptions; (2d) that it
appears at birth or immediately after, while the symptoms of hereditary
syphilis generally show themselves later;[66] and (3d) that at that
time there had been seen no cases of pemphigus at the Lourcine
Hospital, where so many syphilitic children were born.[67] In this view
he was supported by Trousseau, Lasègue, Gibert, Bazin, and other
obstetricians and syphilographers of note.[68]

[Footnote 65: Ariander, _Mémoires de Méd. et d'Accouch._, quoted by
Diday.]

[Footnote 66: This is now known to be an unreliable distinction,
expressing perhaps a general rule, but one with so many exceptions as
to render it void of diagnostic significance.]

[Footnote 67: This may have been true at that time, but has certainly
not continued to be so. Cornil says: "We often see at the Lourcine
children born prematurely or at the full term with pemphigus, either
fully developed at the moment of birth or appearing a few days
afterward, and who commonly die with syphilitic cachexia, the sad
heritage derived from their maternal parents."]

[Footnote 68: Pemphigus may indeed be a specific affection, but no
characteristic sign has been discovered sufficient to distinguish it
from the ordinary form of pemphigus. "On the other hand, there is no
inconsistency in admitting that syphilis, which so deeply impairs the
constitution of the parent, may act like any other common cause and
excite non-specific pemphigus; for an infant is badly lodged and poorly
nourished in the womb of an enfeebled mother, apart from the influence
of the virus" (Ricord, note to _John Hunter's Works_, 1853).]

On the other hand, Dubois claimed a specific character for the
affection {275} on the ground (1st) of the fact that in the majority of
cases there was a syphilitic history in the parents; and (2d) that the
eruption often coexists with well-known syphilitic lesions. This was
supported by Cazenave, Danyan, Bouchut, Vidal, Ollivier, and
others.[69] Diday, who devotes several pages of his interesting work on
_Infantile Syphilis_ to this subject, regards the eruption as simply a
manifestation of a cachexia produced by syphilis,[70] founding this
opinion on (1st) the absence of specific characters in the eruption;
and (2d) that syphilitic pemphigus is a rare affection in the adult, if
it occurs at all, so that to recognize it in the child would be to make
a single exception to the general rule that "all the syphilitic
eruptions of new-born children have their equivalents in those of
adults." He explained the two cases which were then (1858) recorded of
cures of pemphigus by mercury[71] by saying that it was the treatment
of the diathesis, not of the disease, which caused the improvement. He
acknowledges, however, the very frequent association of pemphigus in
the child with syphilis in the parent, and says that it springs from
the latter affection, "specially, but not specifically"--a rather
wire-drawn distinction.[72]

[Footnote 69: Jullien (_op. cit._, p. 1005), after considering the
opposing views as to the character of this eruption, says: "We have no
hesitation in declaring ourselves in accord with Roger, Ollivier,
Ranvier, Parrot, and others, and in distinctly separating from the
specific affection the rare eruption known as simple pemphigus,
sometimes epidemic, occasionally febrile, and appearing most frequently
about three months after birth. We consider likewise that an evidence
of congenital syphilis which is by no means doubtful is found in the
bullous eruption seen at birth or within the first two weeks,
comparatively frequent, and involving by preference the palms and
soles. This opinion is based upon (1st) its appearance in children
whose parents are known to be syphilitic; (2d) its association with
syphilitic lesions of the lungs, liver, kidneys, thymus gland, etc.;
(3d) its partial disappearance under mercurial treatment, and its
reappearance when that treatment is discontinued."]

[Footnote 70: _Op. cit._, pp. 70-77.]

[Footnote 71: Depaul, _Gaz. Méd. de Paris_, 1851, p. 472, and Galligo,
_Gaz. Med. Toscana_, 1852, p. 123.]

[Footnote 72: Trousseau (_Clinical Lecture on Syphilis in Infants_),
after detailing a case in which there was some doubt as to the
existence of hereditary syphilis in a child born alive, and in which
case the previous pregnancy had resulted in a stillborn child at seven
months, the body of the latter having been preserved in alcohol and
exhibiting numerous traces of pemphigus, says: "So far as I was
concerned, this demonstration did not amount to more than the
establishing of a probability, and several physicians who participated
in this indecision finally accepted a compromise. They considered that
maternal syphilis had determined a sort of cachexia in the foetus which
had led to an eruption of bullæ which was not specific. By accepting
this too-facile hypothesis you will imprudently open a door which you
will with difficulty be able to close."]

As these differences of opinion have been perpetuated to the present
day, it has seemed to me proper to make this reference to their
history, although I am strongly convinced that the progress of clinical
and pathological knowledge enables us now to assert that although, as
an exception, bullæ may be due to a profound cachexia not dependent on
syphilis, yet that in the large majority of cases they are specific in
their character.

The argument which always seemed to me the strongest, the fact that a
similar eruption is almost--or quite--unknown in the adult, has been
removed by the observations of Cornil, who has shown that it belongs
properly with the papular rather than with the bullous eruptions, and
should be classed with the roseola and papules of early syphilis--just
where, from its clinical history, we should expect to find it. The
raising of the epidermic layers is due chiefly to their delicacy, their
slight resistance, and their previous immersion in the amniotic
fluid--_i.e._ to {276} conditions which are peculiar to the skin
shortly after birth.[73] He founds these very important opinions upon
the autopsy of a child stillborn a little before full term, the mother
being in the height of secondary syphilis. The child presented
characteristic bullæ on the soles and palms. After hardening these were
found to consist of the two layers of epidermis placed one above the
other. Fig. 6 represents a bulla about one centimeter in diameter which
was situated on the plantar surface of the great toe.

[Footnote 73: Cornil, _op. cit._, p. 203.]

[Illustration: FIG. 6. Pemphigus bulla from a new-born syphilitic
child. The superficial epidermic layer _e_ is elevated by a fluid
exuded between it and the rete mucosum. The rete mucosum, _c_, is also
partly raised, so that there exists a space filled with fluid between
it and the papillæ, _p_. The epithelial prolongations and the ducts of
the sudorific glands _m_, placed between the papillæ, and which run
between them into the derm, are broken and suspended from the rete
mucosum. _d_. Derm. _a_. Fibrous and muscular layers. _t_. Tendons and
fibrous tissue. _o_. Cartilage of ossification of the first phalanx.
_v_. Vessels. X 8.]

[Illustration: FIG. 7. Section of the rete mucosum and papillæ from the
same case of pemphigus as Fig. 6. _o_. Orifice of a sudorific gland.
_m_. Cells of the rete mucosum, some of which are excavated, _c_. _p_.
Papillæ. _v_. Their vessels. _n_. Prolongations of the rete mucosum
between the papillæ. X 200.]

If, then, we find an infant at birth or immediately after[74]
presenting on the soles, the palms, the fingers and toes, or on the
limbs, an eruption consisting of blebs more or less perfectly distended
with a liquid which may be clear, cloudy, or bloody, circular or oval
in shape, sometimes irregular, seated on inflamed, reddish skin, and
surrounded by trifling areolæ, we may strongly suspect the presence of
syphilis in an active and most menacing form. And this suspicion
becomes a certainty if, in combination with such an eruption, the
general cutaneous surface is yellowish or muddy in hue, is hard, dry,
wrinkled, without elasticity or softness--owing to the absence of
subcutaneous fat--and, for the same reason, is furrowed and wrinkled
about the face, imparting an appearance of senility; if the child has a
hoarse cry, a discharge from the {277} nostrils; and, of course, if
there are at the same time other and unmistakable syphilodermata. This
eruption is specially important, however, because upon the recognition
of its specific character in cases of stillbirth, or in those in which
the child survives only a few days--not long enough for the development
of further symptoms--will depend the opinion as to the cause of death,
which, whether expressed or not, will determine the future treatment of
both parents during the interval and of the mother during the next
pregnancy.

[Footnote 74: Non-syphilitic pemphigus is said to be never present at
birth, nor until the child has become considerably exhausted by wasting
from some defect of nutrition. It therefore does not appear until it is
several weeks old. It then attacks the trunk in preference to the palms
and soles.]

We may now consider the other symptoms of the secondary period in the
child.

Coryza is one of the most characteristic, and at the same time one of
the most important, of these in its influence on the health of the
child. It is due to the same condition of the mucous membrane lining
the nasal fossæ as manifests itself simultaneously or soon afterward on
the skin in the shape of erythema, roseola, or papules; in other words,
it is a hyperæmia with papillary infiltration. Now, on the skin this
condition, except in so far as it indicates the presence of a grave
constitutional disease, is of no special importance. In the nostrils of
a sucking infant, already debilitated and impoverished by the anæmia of
syphilis, and depending upon its nutrition for the continuance of the
miserable flickering life which was its original endowment, the same
condition assumes the gravest significance.

The excessive supply of blood to the parts induces a catarrhal
condition which shows itself in a thin, watery discharge, which, as the
child during sucking is compelled to breathe through the nose, is
rapidly dried into crusts. These become adherent, fill up and lessen
the channel for the passage of air, and in so doing add to the rapidity
and force of the respiration through the nose, and thus increase the
tendency to the deposit of these crusts. The peculiar nasal, noisy
respiration of the child has given the affection the popular name of
snuffles. As the child can no longer breathe, or can breathe only with
great difficulty, while sucking, it takes the breast only to drop it
again immediately on account of impending suffocation.[75] As the
disease progresses ulceration occurs beneath the crusts, and often
involves the entire thickness of the delicate mucous and periosteal
layers underlying the thin bones of the nose; perforation of these
bones results, sometimes with caries to such an extent as to cause an
entire loss of the nasal septum, with flattening of the nose--a symptom
comparable to one which sometimes occurs in the tertiary period of
adults, but produced, as we have seen, by other causes. In adults
syphilitic caries and necrosis are usually due to lesions seated
primarily in the osseous or subperiosteal tissues; in the child, at
least in this instance, these tissues are involved secondarily.

[Footnote 75: For an admirable description of the mechanism of this and
other symptoms of coryza see Diday, _op. cit._, pp. 78-83.]

Erythema, or roseola as it is differently called, is apt to present
itself about the second or third week[76] after birth. As in the adult,
it begins upon the abdomen in the form of little oval, circular, or
irregular spots, dull red in color and disappearing upon pressure.
Later the color becomes deeper, the eruption extends to the trunk and
limbs, and, as exudation and cell-proliferation succeed to simple
capillary stains, it {278} ceases to disappear when pressed upon. It is
often moist, owing to the thinness of the epidermis, sometimes
excoriated. Occasionally it is confluent, and covers large areas with
an almost unbroken sheet of deep-red color.

[Footnote 76: Bassereau gives an instance of its occurrence within
three days.]

The diagnosis in the early stage is often difficult on account of the
resemblance to the simple erythema of infancy. As the disease
progresses, however, maculæ form here and there; the cell-infiltration
involves the papillæ, several of which coalesce, forming flat papules;
the nutrition of the superficial layers of the epiderm is interfered
with, especially where it is thick, as on the palms and soles, and the
eruption in those regions becomes scaly, and then the diagnosis is not
difficult.

Papules and Mucous Patches.--In the ordinary evolution of the disease
the next manifestation is usually the development of papules upon the
general cutaneous surface and of mucous patches on the tongue, lips,
and cheeks--probably also on other mucous membranes not exposed to
examination. The papules are apt, for the reason already mentioned--the
thinness and moisture of the skin--to be of the broad, flat kind,
especially, as in the adult, in those regions where the elements of
warmth and friction are superadded to the moisture, as in the folds of
the skin about the genitalia, the neck, the flexures of the joints,
etc. They are then moist, covered with a grayish secretion or a thin
crust, and are in reality mucous patches. Occasionally they take on a
little hypertrophy and develop condylomatous excrescences which closely
resemble the simple acute condylomata of infants. In syphilis, however,
the growth springs from a previously existing papule, which is not apt
to be solitary, there being others in the neighborhood which will
probably establish the diagnosis. The syphilitic condylomata also have
a peculiar fetid discharge, resembling that of mucous patches and more
or less characteristic.[77]

[Footnote 77: Van Harlingen, article "Syphilis" in the _International
Encyclopædia of Surgery_, vol. ii. p. 560.]

Mucous patches in the infant are among the most important of the early
syphilitic lesions--not to the child itself, because they do not
materially affect its health, save in those exceptional instances where
they are accompanied by a marked degree of stomatitis, and thus
interfere with its nursing. Their importance is due to the fact that
they are almost constantly present, and they are thus by far the most
frequent vehicle of contagion from the child to its nurse or to others
with whom it may come in contact. At times they do not differ
materially from the same lesion occurring in the adult, but lose much
sooner their epithelial investment (on account of the delicacy and
comparatively slight attachment of the epithelium at this stage), and
they then appear as oval or irregular red, slightly depressed spots,
distinct or coalescing, ulcerating or oftener covered by a false
membrane. They especially affect the angles of the mouth and the sides
and dorsum of the tongue; and indeed their disposition to select the
former situation constitutes a diagnostic difference between them and
non-specific stomatitis which is to be found in the sulci between the
gums and cheeks and on the gums themselves--locations rarely invaded by
mucous patches.[78] When the latter are ulcerating or are concealed by
diphtheritic membrane, and are situated on the tongue, they may be
mistaken for either simple or parasitic stomatitis. The {279} diagnosis
can often be made by the presence of other syphilitic symptoms--coryza,
erythema, and especially papules. In their absence, however, it must be
remembered that in simple stomatitis, the inflammation not being
limited to special areas, the whole tongue is apt to be involved or a
much larger portion of the buccal mucous membrane; and as there is no
marked tendency to cell-proliferation in these cases, the accompanying
exudation is apt to be serous or watery and to result in
vesiculation--a condition never seen in syphilitic stomatitis. In the
parasitic disease, too, the inflammation is less localized, there is
more swelling and congestion, and the false membrane is said to be of a
whiter color.

[Footnote 78: Bumstead and Taylor, _op. cit._, p. 750.]

No child that has even been suspected of having a taint of hereditary
syphilis should be permitted to nurse at the breast of any one but the
mother, to share its cup or nursing-bottle with other children, to
receive the caresses of relatives or friends; and in this last
restriction we would include the father, even if the suggestion[79] be
true, that in the case of syphilitic children the protection from
contagion probably extends to the male as well as the female parent.
Paternity is sometimes a more doubtful problem than would seem
probable, and even if the father were protected the husband might not
be. The mucous patches, if any are found to exist, should be actively
treated both locally and constitutionally, and during their
demonstrable presence a most rigorous quarantine should be observed.

[Footnote 79: Hyde, _op. cit._ See p. 264.]

Syphilitic condylomata are due to hypertrophic changes in the papules,
which under the influence of heat and moisture in certain regions
coalesce and become more elevated. They vary in size from an eighth of
an inch to a quarter or even a half of an inch in diameter. Their
surface is flat and covered by a crust or by an offensive secretion.
They are found most commonly about the anus or at the angles of the
mouth.

Pustular Syphilides.--A little later in the secondary period, usually
at about the sixth week, but sometimes much earlier, the papules become
transformed into pustules, the change taking place slowly, so that if
examined at any time after it has begun the child will present an
eruption which is markedly polymorphic, showing here and there
yellowish or reddish-yellow maculæ left after the absorption of the
cell-element of certain papules, at other places beefy-red papules at
the height of their development, or papules crowned by a ring of
desiccated and desquamating epidermic scales, and in still other
regions pustules in various stages of formation. Or the various
formative stages of the pustules may be passed through so quickly that
the eruption will be almost entirely pustular, few if any unmodified
papules being discovered. The pustules may remain distended with pus
for a considerable time, after which they may wither and slowly
disappear or may rupture and leave ulcerated surfaces. A number of
these ulcers sometimes run together and make extensive patches covered
with thick, dark-colored crusts. These patches may resemble areas of
impetigo or of impetiginous eczema, but in those affections the crusts
are usually thinner and of a lighter color, and the skin beneath them
is generally on a level with the surrounding surface, bright red and
glazed; while under the crusts of the syphilide will be found a more or
less depressed or excavated ulcer, often covered with pus. The
diagnosis may indeed often be made by gently detaching and {280}
raising one of the crusts and noting the character of the surface
beneath. The erosion under the crusts of eczema heals over more readily
and without leaving a cicatrix.

A so-called furuncular eruption[80] is said to appear at variable
periods between the sixth month and the third year, but does not appear
to me to be clearly differentiated from the large pustular syphilides
with thickened and elevated bases on the one hand, or the ulcerating
tubercular eruption on the other.[81] They are all so rare in
hereditary syphilis, at any rate, as to have little clinical
importance.

[Footnote 80: Bumstead and Taylor, _op. cit._, p. 750.]

[Footnote 81: The distinction between the two forms is usually manifest
if the development of the lesions has been observed; but even this
fails in regard to the tubercular eruption. They both occur at the same
period; they both begin similarly, the furuncles as "small nodules in
the corium," the tubercles as "deeply-seated papules or nodules;" they
both run on to ulceration and pursue a chronic course (Van Harlingen,
_op. cit._, p. 561).]

Iritis.--Another symptom of the secondary period, but of later
development and of rarer occurrence than the syphilodermata which have
been described, is iritis. In spite of its rarity this is extremely
important, because it is frequently overlooked until it has reached
such a stage that occlusion of the pupil results, and also because when
it is recognized it constitutes an almost pathognomonic sign of
syphilis.[82] This statement may now be made unhesitatingly, although
for many years it was contended that iritis, and even the still more
characteristic symptom keratitis, were only associated with syphilis as
coincidences, the constitutional disease, when hereditary, having no
causative relation to the local condition.

[Footnote 82: "When primary iritis occurs in syphilis in young children
it is almost always due to syphilis" (Soelberg Wells, _Treatise on
Diseases of the Eye_, Philada., 1873, p. 173).]

To Mr. Hutchinson belongs the credit of having first clearly developed
the specific character of this trouble,[83] which, on account of the
mildness of the attendant symptoms, is often overlooked. The sclerotic
zone of congestion so marked in the adult, and therefore so valuable a
diagnostic sign to the general practitioner, is very slight, sometimes
absent; and as a consequence the attention of neither parent nor
physician is attracted to the condition until in the more serious cases
it has done irreparable mischief. In milder cases, particularly where
the child is under mercurial treatment for concomitant symptoms of
syphilis, it may run its course and escape notice altogether;[84] and
it is possible that owing to this fact the rarity of the affection has
been overestimated. It is also possible that in such cases changes
occurring at this time may in some instances lay the foundation for
some of the deeper-seated ocular troubles of later life.

[Footnote 83: _Med. Times and Gazette_, 1860, July 14; _Ophthalmic
Hospital Reports_, vol. i. pp. 191, 226; _A Clinical Memoir on Certain
Diseases of the Eye and Ear consequent on Inherited Syphilis_, London,
1863. In the introduction to this volume Mr. Hutchinson states that
acute iritis dependent on hereditary syphilis was first described in
connection with its true cause by Mr. Lawrence, but, as from the date
of that gentleman's first case (1830) up to 1863 but six cases had been
recorded, the announcement had made but little impression on the
profession.]

[Footnote 84: "In the cases of this form of iritis which are seen in
ordinary eye-practice much damage has often been done by occlusion of
the pupil and deeper mischief. Probably many of the slighter cases
escape the notice of the parents and are not brought to the surgeon"
(Mr. Edward Nettleship. See Hill and Cooper, p. 271).]

If, however, attention has been attracted to the eyes, the diagnosis is
not usually difficult. The pupil is irregular, especially under
atropia; {281} there are streaks of lymph, dulness, swelling, change of
color, and on very careful inspection a faint pink zone may be seen in
the sclerotic. The conjunctiva and cornea are generally clear.

Mr. Hutchinson's analysis of the twenty-three cases reported by him is
still of interest as furnishing reliable data for prognosis. The
average age at the time the iritis commenced was five months and a
half. The oldest was sixteen months at the time of the outbreak, the
youngest six weeks. In twelve cases but one eye suffered; in eleven
both were affected. In seven cases (ten eyes) the cure was complete; in
two or three other cases very slender adhesions remained; in twelve
cases, in nearly all of which the patients came under care only at a
late period of the disease, one pupil was permanently occluded by
organized false membrane. In nearly all, coincident symptoms of
syphilis of the skin or mucous membranes were present. Of the thirteen
cases in which alone a history of the family is recorded, the affected
infant was the only living child of his parents in twelve instances. In
the only case in the whole series in which it is stated that there were
other living children the mother had lost four infants out of seven
live births.

The prognosis depends on the stage at which they come under treatment.
The lymph if recent, no matter in what quantity, will probably be
absorbed under mercurial treatment, which will often be of great
benefit even in those cases in which a certain amount of organization
has occurred.[85]

[Footnote 85: It will not be uninteresting, perhaps, to append the
aphorisms regarding iritis in infants which Mr. Hutchinson at that time
enunciated: 1. The subjects of infantile iritis are much more
frequently of the female than the male sex. 2. The age of five months
is the period of life at or about which syphilitic infants are most
liable to suffer from iritis. 3. Syphilitic iritis in infants is often
symmetrical, but quite as frequently not so. (In his article in
Reynolds's _System of Medicine_, written in 1866, three years later,
but revised in 1870, he describes it as "usually symmetrical," vol. i.
p. 444.) 4. Iritis, as it occurs in infants, is seldom complicated, and
is attended by but few of the more severe symptoms which characterize
the disease in the adult. 5. Notwithstanding the absence of phenomena
of acute inflammation, the effusion of lymph and the danger of
occlusion of the pupil are usually very great. 6. Mercurial treatment
is most signally efficacious in curing the disease, and, if recent, in
procuring the complete absorption of the effused lymph. 7. Mercurial
treatment previously adopted does not prevent the occurrence of this
form of iritis. 8. The subjects of infantile iritis, though often puny
and cachectic, are also often apparently in good condition. 9. Infants
suffering from iritis should always show one or other of the
well-recognized symptoms of hereditary taint. 10. Most of those who
suffer from syphilitic iritis are infants born within a short period of
the date of the primary disease in their parents.]

We have now a group of symptoms characteristic of the secondary period
of syphilis, or that extending from birth, or much more commonly from
the age of three or four weeks to about the end of the first year. The
syphilitic child during this time has several or all of the following
symptoms: Coryza with snuffles; an erythematous, papular, or pustular
eruption on the skin; mucous patches on the lips, tongue, cheeks, etc.;
a marked tendency to general wasting; a hoarse cry or cough; senility
of aspect; iritis. The majority of syphilitic children born alive die
during this stage.

Before its termination, sometimes even at birth, other lesions have
been noticed (especially those affecting the liver), which, however,
may better be described in connection with the special organ or organs
involved.

Succeeding this stage--_i.e._ beginning in about a year or eighteen
{282} months--comes an intermediate period, which extends to second
dentition, to puberty, or even much later, and which is characterized
rather negatively--that is, by the absence of symptoms--than otherwise.
The evidence of the general diathesis will of course be present in the
shape possibly of malnutrition, stunted growth, or retarded
development, perhaps shown in the weazened or withered face, the sunken
nose, the pallor of the skin, the premature loss of the upper incisor
teeth or the malformation of the others if they have erupted.

There is but little tendency to recurrence or relapse of any of the
secondary symptoms; and in certain cases, not a very small proportion,
in which these symptoms have been light and have been well and
thoroughly treated, this stage extends throughout life; or, in other
words, as is frequently the case with the adult who has followed a
proper course of treatment, the disease appears to terminate with the
secondary stage. In other cases, however, it recurs, and the symptoms
which it then presents may be taken up in connection with the different
organs or tissues involved.

Syphilis of the ear is for obvious reasons not often discoverable until
the patient has reached an age at which interference with the function
of hearing becomes a noticeable phenomena. The only symptom likely to
attract attention during the stage of inherited syphilis which we are
now considering is a catarrh of the middle ear, which may have for its
starting-point some inflammation, ulceration, or mucous patch of the
pharynx, causing a temporary or permanent occlusion of the orifices of
the Eustachian tubes.[86] This may lead to perforation of the membrana
tympani, purulent infiltration of the mastoid cells, etc., and when
accompanied by an otorrhoea which attracts attention to the ear will be
easily discovered by the physician. These cases are, however,
exceptional, otorrhoea only being present in nine out of Hutchinson and
Jackson's[87] one hundred cases of inherited syphilis, and consequently
but little is known about the frequency or gravity of lesions of the
auditory apparatus in the secondary stage of this form of syphilis.[88]
The changes which occur later on are chiefly those which involve either
the nerves themselves or their distribution in the labyrinth.

[Footnote 86: Bäumler, _Ziemssen's Cyclopædia_, vol. iii. p. 226.]

[Footnote 87: Hutchinson and Hughlings Jackson, _Med. Times and Gaz._,
Nov. 23, 1861.]

[Footnote 88: Schwartze (quoted by Hill and Cooper) found also that
otorrhoea was a rare complication in deafness from syphilis.]

The affections of the middle ear and Eustachian tube are said to be
contemporaneous with the keratitis which appears in the neighborhood of
puberty,[89] while those of the nerve are somewhat later in point of
time, and are almost always conjoined with retinitis, choroiditis, and
optic neuritis. As usual when investigating or describing any subject
relating to syphilis, Mr. Hutchinson's opinion and observation must be
detailed. In 1863 he wrote[90] that it was only recently that he had
thought of specially investigating the disorders of hearing in
reference to hereditary taint, having had his attention called to a
peculiar form of deafness, usually symmetrical, passing rapidly through
its different stages and {283} unaccompanied by any marked degree of
pain or any external disease. He then reported eighteen cases of which
he had notes. The oldest of these patients was twenty-seven, the
youngest eight--the average time of development of the deafness from
twelve to fifteen. Although the membrana tympani was in no instance
quite normal, in none were there found adequate changes to account for
the deafness. In all the Eustachian tubes were pervious. In nearly all
the disease was symmetrical. This fact, together with the absence of
discoverable lesions of the external or middle ear, seems to point
conclusively to disease of the nerves themselves, or at least to a
central cause.[91] He adds: "With regard to the prognosis of
heredito-syphilitic deafness, I believe that it is very unfavorable.
When the disease was progressive I have rarely witnessed any permanent
improvement or arrest. In most it has gone on to total loss of hearing,
and this in several instances in spite of the cautious use of specific
remedies almost from the beginning. From six months to a year would
appear to be the usual time required for the completion of the process
and the entire abolition of the function."[92]

[Footnote 89: Purves, _Guy's Hospital Reports_, 1875, p. 564;
Pritchard, _British Medical Journal_, April 21, 1877.]

[Footnote 90: _Clinical Memoirs on Certain Diseases of the Eye and Ear
consequent on Inherited Syphilis_, London, 1863, pp. 182, 183.]

[Footnote 91: In the _Lancet_ for Jan. 16, 1875, he reports a case of
total deafness in a young woman of seventeen which had come on in ten
months without pain or otorrhoea. He believes the disease of the organ
of hearing to be parallel with those cases of choroiditis disseminata
or of optic neuritis in which blindness is produced without pain or any
external evidence of inflammation, and which are distinctly and
positively associated with inherited syphilis.]

[Footnote 92: Mr. Hinton, in his edition of Toynbee's work on _Diseases
of the Ear_, states that at Guy's Hospital, of his aural patients, one
in twenty is affected with deafness due to heredito-syphilis; that it
usually makes its appearance between the tenth and sixteenth years; and
that the great majority of the cases which he has seen have been
females. He adds: "Patients suffering from this disease may, as a rule,
at least when young, be at once distinguished by the amount of deafness
which they exhibit. I know no other affection except fever which in a
person under twenty brings on a deafness so rapidly and so nearly
complete. In the course of a few weeks a girl previously hearing well
will, without pain or known cause, become unable to distinguish words."
In one of Dalby's cases total deafness came on in three weeks, previous
to which hearing was normal. According to Pierce, the deafness is most
apt to manifest itself between eleven and eighteen years of age.
Troeltsch says that "l'audition du diapason par le vertex" is lost at
an early date after the beginning of the disease, and that there are
also often concomitant affections of the nose and pharynx.]

Dalby[93] is said to regard syphilis as, next to scarlatina, the most
fruitful cause of deaf-mutism as it occurs in children born with good
hearing powers. "The patient usually becomes deaf in early
childhood--after he begins to talk--or between this period and
puberty."[94]

[Footnote 93: _The Lancet_, Jan. 22, 1876.]

[Footnote 94: Bumstead, _op. cit._, p. 734.]

Syphilis of the Liver.--In 1852, Gubler published an account of the
general appearances in syphilitic disease of the liver in new-born
children, which was distinguished especially by increase in size and
weight. This increase depended, as might be expected in this
stage--that of general cell-proliferation--upon a proliferation of
cells from the connective tissue between the acini, or from the
adventitia of the interlobular vessels, this growth becoming
transformed into connective tissue.[95] The change is quite analogous
to what is taking place at the same time in the skin, the mucous
membranes, and other tissues. Wilks has also described[96] a form of
syphilitic disease of the liver which corresponds to that of Gubler,
and in which the whole organ is infiltrated by a new fibrous tissue,
producing a uniform and general hardening.

[Footnote 95: Bäumler, _op. cit._, p. 186.]

[Footnote 96: _Trans. Path. Soc._, vol. xvii., 1866.]

{284} [Illustration: FIG. 8. Section of an old gumma of the liver. _a_,
_a_. Central caseous tissue of the gumma. _v'_, _v'_. Its vessels. _l_.
Boundary between the central portion and fibrous zone; this line of
demarcation is marked in places by an opening or cleft. _t_, _t_.
Connective tissue of the fibrous zone which entirely surrounds the
central part. _v_, _v_. Small vessels of this zone. _c_. An arteriole
of the fibrous zone. _f_, _f_. Quite large biliary vessels included in
the fibrous zone. _t'_. Fasciculi of connective-tissue fibres running
parallel with the surface of the caseous part. At _b_ and _d_ the
fasciculi of fibres of the fibrous zone penetrate into the central
caseous part. _e_, _e_. Tissue of hepatic cells interrupted by bands of
fibrous tissue, _m_, _m_. X 12.]

As described by Gubler,[97] the liver in such children is
hypertrophied; hard, resistant to pressure, so that it cannot be
indented; elastic, so that it rebounds; creaks, but does not bleed,
when it is cut into, and presents the yellow color and the
semi-transparence of flint. There are seen on a yellowish ground a
number of small white granulations like grains of wheat, which a
histological examination shows to be formed by an accumulation of
embryonic cells in the spaces which separate the hepatic acini.
Injections reveal the fact that the vascular network has become almost
impenetrable, the capillaries obliterated, the larger vessels
diminished in calibre. Fibro-plastic matter is found throughout the
organ in large quantity. In consequence of these conditions--the
compression of the hepatic cells and the destruction of the
vessels--the secretion of bile is stopped, and the gall-bladder is
found after death to contain a pale-yellow liquid consisting of bile
mixed with an excess of mucus. This form of hepatitis has thus far been
observed almost exclusively in infants. Cornil {285} says[98] that he
has had frequent occasion to examine such cases of hepatic syphilis,
and describes them as follows: "The hepatic acini, in the normal state,
are in contact except at the prismatic spaces which are formed by their
union--spaces in which the capsule of Glisson forms an envelope to the
afferent portal vessels of the lobuli. It is in these spaces that the
round lymph-cells form and collect into small nodules representing
microscopic gummata. The cells at the centre of the new formation are
sometimes granular. This neoplasm is seated about the ramifications of
the portal veins, which in consequence also present thickened walls
with newly-formed cells in their external tissues. The small granules
above mentioned are not always visible to the naked eye, and in their
places are only seen, about the perilobular capillaries of the portal
vein, an excessive number of embryonic cells." In addition to this
interstitial sclerosis or interstitial infiltrating hepatitis there is
an inflammation of the liver depending upon the presence of
gummata--gummous hepatitis--which occurs in two forms: one in which
very small and very numerous nodules are present, situated along the
course of the fibrous seams, the prolongation of the capsule, and
another in which there are two or three large circumscribed tumors.
This form of hepatitis is always accompanied by the interstitial form,
although the latter may be only slightly developed.[99] The gummata,
though not infrequently found in the liver of new-born children, are
more likely to develop later, at from about the eighth to the twelfth
year.

[Footnote 97: _Mémoires sur une nouvelle Affection de Foie_, and _Gaz.
Méd. de Paris_, 1852.]

[Footnote 98: _Op. cit._, Am. ed., p. 370.]

[Footnote 99: It does not differ essentially, either pathologically or
clinically, from the same lesion in adults.]

Rochebonne[100] describes the following symptoms of syphilitic
hepatitis in infants: A deep wine-colored venous stain and oedema of
the lower extremities, often accompanied by pemphigus; ascites due to
mechanical obstruction of the circulation, as in cirrhosis; a more or
less pronounced chloro-anæmic appearance of the face; and the presence
in the urine of albumen and hæmato-globulin. Vomiting may occur, and
constipation alternating with diarrhoea has been observed. Icterus,
symptomatic of the affection, has not been observed.

[Footnote 100: Quoted by Bumstead and Taylor, p. 758.]

Bäumler says:[101] Implication of the peritoneal coating of the liver
may be recognized by the pain in the hepatic region. In new-born
children--unless, possibly, there may be some enlargement of the
liver--the only local symptoms, often, are those due to
peritonitis--screaming, drawing up of the legs, vomiting. In those
cases it is not rare for the peritonitis to become diffuse.[102]

[Footnote 101: _Op. cit._, p. 194.]

[Footnote 102: In an article on "Inherited Syphilis" in the _British
and For. Medico-Chirurgical Review_, 1875, p. 28, it is said: "Of the
liver the lesion consists in enlargement and induration of the organ in
whole or in part, due to the development of fibro-plastic material
between the cells of the acini, with obliteration of the vessels and
interference with the secretion of bile. This condition is generally
doubtful during uterine life, and is rapidly fatal. The symptoms are
vomiting, diarrhoea, and tympanitis, but, strange to say, no jaundice.
The enlarged and indurated organ may be felt by palpation. It is
probably in this connection that the peritonitis described by Simpson
as occurring in inherited syphilis is found."]

Hill says:[103] "The symptoms are mainly those of functional
derangement of the organ, with alteration of its bulk."

[Footnote 103: _Op. cit._, p. 163.]

Hutchinson[104] has described cases in which in young persons the
subjects {286} of hereditary syphilis there has been great hepatic
enlargement which has subsequently wholly disappeared. He finds it
difficult to believe that there is any kind of gummous growth in such
cases, and feels obliged rather to fall back upon the hypothesis of
mere vascular turgescence. In one such case the liver occasionally was
so large as to be visible as the patient lay on his back in bed.[105]

[Footnote 104: _Path. Transactions_, 1877, p. 309.]

[Footnote 105: Illustrative cases of this condition may be found in the
_Med. Times and Gazette_, Dec. 22, 1877.]

It seems much more likely that the enlargement is due to an
exceptionally active cell-proliferation, which does not, however, go on
to organization, but may be just as susceptible of absorption and
resolution as are the papules or maculæ of the skin. A portion of the
enlargement may be due to a passive congestion caused by the presence
of this cell-accumulation.[106]

[Footnote 106: Barlow (_Path. Trans._, 1877, p. 355) has suggested that
the engorgement is only a preliminary stage of the fibrous thickening,
and may disappear either with or without leaving permanent contractions
or adhesions in its wake.]

As to the diagnosis of hepatic syphilis in infants, I am disposed to
agree with Cornil, who says:[107] "The symptoms are null, or they are
identical with those of local and general troubles so often observed in
children who have poor or insufficient nourishment. The only physical
sign which properly belongs to hepatic syphilis is, when it exists at
all, increase in the size of the liver."

[Footnote 107: _Op. cit._]

Syphilis of the Bones.--Until the publication in 1870 of the researches
of S. Wegner,[108] an assistant of Prof. Virchow, diseases of the
osseous system due to hereditary syphilis were either ignored or denied
by the various writers upon this subject.[109] Valleix, Bargione,
Ranvier, and Guéniot had indeed recorded cases of bone disease
occurring at the points of junction between the epiphyses and diaphyses
and in the costal cartilages, but it remained for Wegner first fully to
describe the pathological changes which occurred there, and to
differentiate them from those due to rickets or scrofula. His memoirs
recognized three stages of alteration in the long bones:[110] 1st.
While in the normal state the boundary of the hyaline cartilage is
distinctly marked by a line which indicates the direct transformation
of the cartilaginous tissue into a spongy tissue, the unaided eye being
unable to distinguish a spongio-calcareous layer, in new-born
syphilitic children, on the contrary, the bones are seen to have a
spongio-calcareous layer interposed between the bone and cartilage,
measuring two millimeters in thickness. This is a zone of calcifying
cartilaginous material more extensive than in the normal state. 2d.
These same changes become more distinct and more extensive. The
unnaturally thick layer of calcareous material continues to grow. There
is proliferation of the cartilaginous trabeculæ, abundant calcification
of the cartilage, too early and irregular ossification of the
intercellular substance {287} of the cartilage, and at the same time an
arrest of the normal formation of bone which should be going on from
the epiphysial cartilage. 3d. There is now added, by extension of these
processes, a thickening of the perichondrium and periosteum at the
extremities of the long bones and at the junction of the ribs with the
costal cartilages. In consequence of the interference with nutrition
occasioned by these changes atrophy and fatty degeneration of the
cartilage-cells occur, and they form between the epiphysis and
diaphysis a necrosed mass which irritates the living bone. This causes
osteo-myelitis, which frequently results in a separation of the
epiphyses. Occasionally pus is produced in such quantity as to
perforate the periosteum, escape into the surrounding tissues, and
become superficial. He terms the entire process an osteo-chondritis.

[Footnote 108: _Virchow's Archiv_, 1870, B. 50, S. 305: "Ueber
hereditäre knochen Syphilis bei jungen Kindern."]

[Footnote 109: Diday says: "Affections of the bones are so rare in
children with inherited syphilis that the annals of medicine scarcely
offer five or six well-authenticated cases of caries or periostitis"
(_op. cit._, p. 83). Referring to this statement, Mr. Hutchinson
remarks: "So different has been my own experience from this that I may
say that we are scarcely ever without a severe example of it in the
wards of the London Hospital" (_Illustrations of Clinical Surgery_,
London, 1875, p. 47).]

[Footnote 110: Cornil, _op. cit._, p. 282 _et seq._]

Waldeyer and Köhner,[111] after examining twelve cases, confirm in the
main these investigations of Wegner, but interpret the changes as
arising rather from the formation of a gummous tissue between the
epiphysis and diaphysis than from an osteo-chondritis. The tissue-death
which occurs later, the atrophy of the cells, etc., they compare with
the same modifications observed in syphilomata.

[Footnote 111: "Beiträge zur Kenntwiss der hereditäre knochen
Syphilis," _Virchow's Archiv_, B. 55, S. 367.]

Parrot[112] in a number of exceedingly valuable papers has repeated and
greatly extended these observations. He places especial importance upon
the formation of osteophytes, which, he says, in the first stage
envelop the diaphyses of the long bones, especially at their inferior
extremities. In the succeeding stage the new bony layers are more
porous; a gelatinous degeneration affects the epiphysial cartilage and
the spongy bones at a point where they are in contact; the epiphyses
tend to separate from the diaphyses. This solution of continuity
results in a characteristic pseudo-paralysis, with curvatures, abnormal
twistings, and preternatural mobility of the bones, with loss of the
power of locomotion. Then the osteophytes increase in size by the
formation of several layers, thus enlarging the inferior extremities of
the long bones. He describes the general process as consisting, first,
of a periosteo-genesis--a formation of osseous tissue from the
periosteum; next of a chondro-calcosis--a calcareous incrustation of
cartilage; and finally of a gelatiniform degeneration and softening of
the bone, with diaphyso-epiphysial disjunction.[113]

[Footnote 112: _Société de Biologie_, June 1, 1872; _Société
anatomique_, 1873, p. 92; _Archives de Physiologie_, 1876, vol. iii.
pp. 138, 139; _Revue mensuelle de Médecine et de Chirurgie_, 1877;
_Pathological Trans._, 1871, vol. xxx. p. 339, etc., etc.]

[Footnote 113: Cornil (_op. cit._) coincides in the main with this
description.]

Taylor[114] sums up the results of his observations as follows: "In the
first stage we have a simple hyperplasia of cells with irregular
deposition of lime salts; in the second, an intensification of this
condition; and in the third, a new element--namely, the abnormal
proliferation of all the elements of the tissues, with an infiltration
of granulation-tissue into the medullary spaces following the
vessels."[115]

[Footnote 114: _Syphilitic Lesions of the Osseous System in Infants and
Young Children_, New York, 1875, p. 134.]

[Footnote 115: Verraguth (_Archiv für Path. Anat._) describes the first
step as an excessive formation of vessels in the cartilage and a
corresponding overgrowth of the cellular elements. This becomes
inflammatory, and constitutes a primary syphilitic chondritis, the
changes in the medulla of the bone being degenerative and secondary to
the affection of the cartilage. Still other observers have described
the process, each with minor modifications; but as they are of no
clinical importance, it does not seem worth while to quote them.]

{288} We see, then, that, setting aside minor points of difference,
these observers all coincide in describing this condition as one
essentially of the nature of syphilitic bone troubles with which we are
familiar in the acquired form of the disease, consisting primarily and
throughout of an unnatural accumulation of cell-elements, which in the
later stages by their pressure produce various degenerations of
surrounding structures, and which, as they occur during the process of
bone-formation, are accompanied by irregular and abnormal deposition of
lime salts. They especially affect the regions mentioned--the junctions
of the epiphyses and diaphyses--because at that time those points are
the seat of great physiological activity. Syphilis, indeed, throughout
its entire course is notably subject to similar influences, as one
example of which I may instance the preference displayed by the
periostitis which results in nodes or in caries for the subcutaneous
bones, the tibia, clavicle, cranium, etc.; or, in other words, for
those which are subject to frequent traumatisms--trifling, perhaps, but
sufficient to determine a slight hyperæmia, which is followed by
abnormal cell-proliferation or accumulation.

The symptoms which obtain in this condition of syphilitic
osteo-chondritis are as follows: The child may be attacked during
intra-uterine life, and in that event the osseous lesions will probably
be coincident with other syphilomata and with placental disease of
sufficient gravity to destroy life.[116] If the child is born alive,
the first development of the disease will probably be noticed as a
swelling at the diaphyso-epiphysial junction of one of the long bones,
which in the emaciated subjects of hereditary syphilis is often
visible, and can always be discovered by palpation. The bones most
frequently attacked are the humerus, radius and ulna, tibia and femur,
but the clavicle, ribs, sternum, and bones of the metatarsus and
metacarpus are also often involved, and much more rarely the frontal
and parietal. The more pronounced the syphilis of the parents, or the
nearer the date of conception to the time at which their infection
occurred, the more probable is it that several bones will be affected,
and the more unfavorable the prognosis as respects the life of the
child. Indeed, it has been noticed that "in stillborn infants and in
those dying soon after birth the majority, or even all, of the long
bones are affected."[117]

[Footnote 116: Pollnow found osteo-chondritis in 35 out of 50
syphilitic foetuses (_Der Hydrops Sanguinolentes foetus_, Berlin, 1874,
quoted by Hill and Cooper, _op. cit._, p. 352).]

[Footnote 117: Bumstead and Taylor, _op. cit._, p. 767.]

The swelling is found to consist of a ring or collar which more or less
completely surrounds the bone, is apt to be smooth rather than
irregular, and when two bones situated near to each other are
simultaneously affected may conjoin them. This condition persists
during the first stage of pathologists, and passes with greater or less
rapidity into the second stage, in which the swelling, the
cell-proliferation, reaches its height. This may take, in cases
uninfluenced by treatment, several weeks or even months. Under the use
of mercurials and iodide of potassium they usually subside rapidly.
During this second stage, however, owing to the proximity of the
swellings to the joints, a moderate amount of synovitis is often
present. This affects chiefly the elbow and the knee, but may appear in
any joint. It is also readily influenced by specific treatment and
well-regulated pressure.

{289} When the third stage is reached, or that of the formation of
granulation-tissue, with degenerative changes of the cartilages and of
the bones themselves, deformity often becomes more marked. There are
unnatural curves or angles in the bones, with more or less complete
separation at the point of junction. Where many bones are affected in
this way, the resulting deformity is extreme and the patient may be
absolutely powerless, a condition of pseudo-paralysis supervening in
which the limbs lie motionless or swing about like the arms or legs of
a doll when the child is carried.

When the swelling does not undergo absorption, the superjacent tissues
sometimes become involved, abscesses form and make their appearance
externally, extensive necrosis of the shaft of the affected bone takes
place, and the little patient usually dies of hectic, pyæmia, or
exhaustion. When the cranial bones are involved, the disease is apt to
limit itself chiefly to the stage of osteophytic formation, the
immovability of the bones probably favoring the organization of the new
cell-growth rather than the production in it of inflammatory changes.
The growths are met with chiefly in older children than those affected
with the form of osteo-chondritis just described; they affect the
periphery of the liver, and are found most usually around the anterior
fontanel, and later on the parietal and frontal eminences. The sutures
are sometimes completely soldered together.[118] The osteophytes vary
in thickness from a quarter of an inch to an inch, or are even
larger.[119]

[Footnote 118: In a case reported by Barlow it was not possible at the
autopsy to discover the point of union (_Path. Transactions_, 1879, p.
339).]

[Footnote 119: These conditions may all result in a child the subject
of acquired syphilis, but are apt to be milder, to involve fewer bones,
and to yield more readily to treatment. This would of course be
expected, inasmuch as the same difference in favor of the acquired
form, as compared with that which is inherited, extends to all the
lesions. As Diday succinctly expresses it: "In the one case the poison
vitiates only the elements of nutrition; in the other it vitiates at
the same time those of formation and those of nutrition." It would
exceed the limits of the present article to describe acquired syphilis
in children.]

The most important differential diagnosis to be made in these cases is
between the rachitis of young children and the form of syphilis in
question. Much difference of opinion still exists as to the relation
between these diseases, syphilis being claimed, on the one hand, as
having in the majority of cases a definite causative influence, while,
on the other, the existence of this relation is denied. When we come to
contrast the pathology of the two diseases, we can readily understand
why they should be confounded, the minuter changes which occur being
essentially the same--viz. cell-proliferation and accumulation, with
subsequent inflammatory changes, associated with irregular deposits of
lime salts.

Compare, for example, the description of the pathology of bone diseases
in inherited syphilis already given (pp. 287, 288) with the following
terse summary of the changes which take place in rickets in cases where
no suspicion of syphilis exists, either ancestral or acquired: "The
changes are more distinctly noticed at the epiphyses than in the
diaphyses. Instead of the regular stages and distinct boundaries
observed in the normal development of bone, there is a singular
disorderly commingling of the exaggerated cartilage-proliferation and
transition substance, with calcification. The cartilage-cells,
stimulated to excessive multiplication, are transformed, some into
bone-corpuscles, some into medullary cells, {290} and others into
connective-tissue forms. The same process is in active operation in the
deep periosteal layers, the material accumulating to such a degree as
to add much to the thickness of the shaft."[120]

[Footnote 120: Agnew's _Surgery_, vol. i. p. 1030.]

The points of resemblance are manifest, just as they are between a
syphilitic and a variolous pustule, but they end in both cases when we
come to study the evolution of the phenomena either from an anatomical
or from a clinical standpoint. They may be expressed as follows in
tabular form:

  OSSEOUS LESIONS DUE TO INHERITED |       RICKETS.
          SYPHILIS.                |
                                   |
  The swellings, particularly those| Rarely appear before six months,
  of the long bones, show          | generally still later.
  themselves at or soon after      |
  birth.                           |
                                   |
  A history of syphilis or evidence| No such history necessarily.
  of existing syphilis in one or   |
  both parents.                    |
                                   |
  Preceded or accompanied by       | No such prodromata.
  snuffles, coryza, and cutaneous  |
  and mucous lesions.              |
                                   |
  No such prodromata in most cases.| Pallor, restlessness, sweating,
                                   | nausea, diarrhoea, etc.
                                   | constitute a combination of
                                   | symptoms which often precede the
                                   | bone disease.
                                   |
  Cachexia absent or moderate.     | Cachexia marked.
                                   |
  Physiognomical peculiarities of  | Not present as a group.
  syphilis present.[121]           |
                                   |
  Circumscribed tumors on frontal  | Cranial bones thickened in spots,
  and parietal bones, rarely on    | usually upon the occiput.
  occiput.                         |
                                   |
  Ribs not markedly affected.      | All or nearly all involved.
                                   |
  Swellings on long bones or       | Extremities symmetrically
  extremities irregular.           | enlarged.
                                   |
  Disease of ribs, when existent,  | Nearly always so.
  not ordinarily coincident with   |
  that of other bones.             |
                                   |
  Fontanels close at usual period. | Closure delayed.
                                   |
  Other syphilitic symptoms        | Syphilitic symptoms absent.
  present; enlargement of          |
  phalanges, metatarsal bones, etc.|
                                   |
  Often accompanied by sinuses,    | Little external or surrounding
  synovitis, abscesses, cutaneous  | involvement.
  ulcers, etc.                     |
                                   |
  Generally disappears by          | Usually leaves some bending of
  resolution, without leaving any  | shaft and distortion of the
  permanent change.                | neighboring joint.
                                   |
  Mortality among children in whom | Much less.
  many bones are involved is very  |
  great.                           |
                                   |
  Specific treatment useful.       | Of no benefit.
                                   |
  In the first stage there is an   | This is less marked. There is
  exuberant calcification of the   | formed, instead, a soft and
  ossifying cartilage, causing     | non-calcified osteoid tissue.
  necrosis of the new-formed tissue|
  and a consecutive inflammation,  |
  which terminates in the          |
  separation of the epiphyses.[122]|

[Footnote 121: See p. 313.]

[Footnote 122: This table is founded on one which I added to the
translation of Cornil made by Dr. Simes and myself, and is compiled
chiefly from the excellent work of Dr. Taylor already alluded to.]

The diagnosis of the bone lesions of hereditary exostosis can readily
be recognized in a short time by noting the fact that they are
stationary, {291} even if their later appearance, larger size, the
absence of syphilitic history or symptoms, and the resistance to
specific treatment left us in doubt.

The diagnosis from accidental separation of the epiphysis, or from
fractures, may be made from the history of the case.

In cases of separation of the epiphysis, complicated with suppuration,
sinuses, etc., the trouble may be mistaken for a similar condition due
to non-specific inflammation. In all the recorded instances, however,
the latter has occurred much later in life, is attended with much more
acute inflammatory symptoms, lymphangitis, etc., and is of course
without concomitant symptoms of syphilis. In both these cases there is
a decided osteo-periostitis, and as so much depends on the early and
vigorous use of specific treatment, it may be worth while to contrast
the two forms of the disease.

  SYPHILITIC OSTEO-PERIOSTITIS.    | NON-SPECIFIC OSTEO-PERIOSTITIS.
                                   |
  Occurs in infants under three    | No instance of its occurrence in
  months of age.                   | children under one year of age.
                                   |
  History of syphilis in child and | No history of syphilis; sometimes
  its parents.                     | a history of traumatism.
                                   |
  Implication of other bones.      | Usually confined to one bone.
                                   |
  Coincident with the development  | Coexists with the ossification of
  of the shaft of the bone.        | the epiphyses.
                                   |
  Other lesions of syphilis: nodes,| No such symptoms.
  skin eruptions, etc.             |
                                   |
  All the local symptoms           | Pain, redness, and swelling very
  comparatively mild.              | marked.
                                   |
  Disease sharply localized.       | Involves neighboring parts.
                                   |
  Lymphatics of limb unaffected.   | Lymphangitis present.
                                   |
  Beneficial effect of specific    | No such effect.
  treatment if employed early.[123]|

[Footnote 123: Cornil, _op. cit._, p. 274.]

Syphilitic dactylitis in the inherited variety of the disease, as in
the acquired, consists of two varieties. The one of these which usually
appears earlier involves chiefly the periosteum and the fibrous and
integumentary structures surrounding a joint, usually a metacarpo- or
metatarso-phalangeal articulation, involving a phalanx, and is
characterized by slow, almost painless, swelling and discoloration of
the affected member. (Fig. 9.) This is due to a gummous infiltration
which, after absorption under proper treatment, leaves the toe or
finger temporarily stiff, but not permanently disabled.

The second form is a specific osteo-myelitis, with periostitis, coming
on later, and often destroying the bone or the articulation involved.
(Fig. 10.)

[Illustration: FIG. 9. FIG. 10. From Bumstead on _Venereal Diseases_,
illustrating Syphilitic Dactylitis.]

The absence of acute inflammatory symptoms in the first variety
distinguishes it from paronychia, whitlow, and gout. Rheumatoid
arthritis begins in the joints, is associated with other symptoms;
deformity of the fingers comes early in the disease, and there is a
teno-synovitis with contraction.

The second variety might be taken for enchondroma or exostosis, but
these growths increase much more slowly, involve only a limited portion
of the bone, are of greater density, and are much more strictly
circumscribed.

As a rule, especially in cases which are recognized early and treated
{292} actively, the prognosis is good. Iodide of potassium should be
used in combination with mercury.

Syphilis of the Teeth.--Syphilis of the teeth has its chief interest to
the general practitioner from its very important bearing on diagnosis.
As manifesting itself at an age when the child is not apt to present
the active and unmistakable cutaneous and mucous lesions of the
disease, and when, consequently, its recognition is often extremely
difficult, this diagnostic importance is greatly increased.

The teeth of the first dentition, although exhibiting the usual signs
of interference with nutrition in their irregular development, opaque
and chalky enamel deficient in quantity and unevenly distributed, soft
and friable dentine, incongruity of size individually and relatively,
and proneness to decay, do not often display any distinctive evidence
of syphilis. {293} The same conditions may, and often do, depend on
other causes, and are commonly associated with various cachexiæ--the
strumous, gouty, rheumatic, rachitic, etc.--and even with other
slighter ailments tending to produce imperfect assimilation and
malnutrition.

In the permanent teeth, likewise, the same condition may be due to the
same causes. Stomatitis, however produced--by mercury, by
gastro-intestinal derangements, by local irritation of any kind--is apt
to result in imperfectly organized dental structures. Mercurial teeth,
for example, are usually irregularly aligned, horizontally seamed,
honeycombed, craggy, malformed, of an unhealthy dirty yellow color,
separated too widely, and deficient in enamel.[124] The diseases of
childhood, especially the eruptive fevers, eclampsia, typhoid fever,
etc., by temporarily arresting or greatly interfering with nutrition
during the developmental period of the teeth, often cause horizontal
furrows across their crowns, which are, of course, persistent
throughout life, and mark indelibly the influence of such disorders on
all the formative processes.

[Footnote 124: The latter defect is particularly noticeable on the
cusps of the sixth-year molars. (See note on "Syphilis of the Teeth,"
by Dr. James W. White, in Am. ed. of Cornil, pp. 287-290.) The
discussion as to the effect of mercury in producing the condition of
the teeth known as honeycombed is still going on, but the evidence
seems to point clearly to a direct connection with the administration
of mercury in infancy, either for syphilis or in excessive doses as a
purge, or in some of the teething powders, which often consist of
calomel and opium. Mr. Hutchinson, at a meeting of the Odontological
Society (see _Proceedings_ for 1877, p. 249), gave an interesting
description of the way in which the supposition was arrived at.
Lamellar cataract is a disease which affects the eyes of children who
have suffered from convulsions: it was noticed that in cases of that
form of catarrh there were also honeycombed teeth, and it was thought
that the convulsions, the cataract, and the honeycombed teeth were all
due to the same unknown cause. At last a few exceptions were
found--patients with cataract, but with good teeth, and then some who
had had convulsions only, and yet had honeycombed teeth; lastly, it was
noticed that most of the patients had been treated with mercury. So it
came to be recognized that the honeycombed teeth were only accidentally
associated with the cataract, and that they were, in fact, the result
of the mercury which had been given to cure the convulsions. The same
gentleman figures a case of this disease in his _Illustrations of
Clinical Surgery_ (London, 1875), and thus describes it (p. 55): "The
present state of his permanent teeth is so characteristic as to deserve
more detailed description. The change about to be mentioned affects all
the incisors, canines, and first molars of both upper and lower jaws,
the bicuspid being scarcely implicated at all; the second molars are
also quite healthy. In the first molars the alterations consist of
deficiency of enamel on the upper surface of the crown and the presence
of spines of uncovered dentine. In the case of the incisors a
considerable portion of the crown of each tooth is totally devoid of
enamel, and its dentine is also deficient to some extent, so that the
teeth are thin, sharp-edged, and of a dirty yellowish color. The
transition from the enamel-covered to the diseased part occurs suddenly
in a horizontal line at some little distance from the crown of each
tooth; the position of this line or step being in each tooth nearly at
the same distance from the gum. The general effect when all the teeth
are seen together is as if a string had been tied around them when soft
and the distal part had withered."]

None of these conditions, however, are in the least degree
characteristic of syphilis, the special expression of which in the
mouth is to be found only in the permanent upper median incisors. For
the recognition and description of the peculiarities of these teeth in
the subjects of inherited syphilis we are indebted, as we are for so
much else of inestimable value in the study of the disease, to Mr.
Hutchinson. In 1863, in a memoir on _Syphilitic Diseases of the Eye and
Ear_, he wrote as follows[125] {294} concerning the symptoms which, in
a suspected case, would aid in determining the diagnosis: "By far the
most reliable amongst the objective symptoms is the state of the
permanent teeth if the patient be of age to show them. Although the
temporary teeth often, indeed usually, present some peculiarities in
syphilitic children of which a trained observer may avail himself, yet
they show nothing which is pathognomonic, and nothing which I dare
describe as worthy of general reliance.[126] _The central upper
incisors of the second set are the test teeth_, and the surgeon not
thoroughly conversant with the various and very common forms of dental
malformation will avoid much risk of error if he restricts his
attention to this pair. In syphilitic patients these teeth are usually
short and narrow, with a broad vertical notch in their edges and their
corners rounded off. Horizontal notches or furrows are often seen, but
they, as a rule, have nothing to do with syphilis. If the question be
put, Are teeth of the type described pathognomonic of syphilis? I
answer unreservedly that when well characterized I believe they are. I
have met with many cases in which the type in question was so slightly
marked that it served only to suggest suspicion, and by no means to
remove doubt; but I have never seen it well characterized without
having reason to believe that the inference to which it pointed was
well founded."

[Footnote 125: Chapter on "The Means of Recognition of the Subjects of
Hereditary Syphilis during the Tertiary Stage," p. 204. Before this,
however, he had called attention to the same peculiarities in a paper
on "The Means of Recognizing the Subjects of Inherited Syphilis in
Adult Life," _Medical Times and Gazette_, London, Sept. 11, 1858, p.
265.]

[Footnote 126: So far as I know, the only recorded instances by
reliable observers of the temporary teeth presenting the peculiar
characteristics of syphilis are as follows: In the _Transactions_ of
the Odontological Society of Great Britain, vol. ix., 1877, p. 258, Mr.
Oakley Coles described a case--without, unfortunately, giving
details--in which the "peg-shaped temporary teeth were very
characteristic of syphilis." Mr. Coles's abilities are well known, and
the fact that at the previous meeting the society had been addressed by
Mr. Hutchinson on this very subject would seem to indicate that he was
quite familiar with the importance of his statement.

M. Fournier calls attention (_Archives de Derm. et Syph._, Sept. 25,
1883) to a cast (No. 48) to be found in the collection of M. Parrot in
the museum of the Foundling Hospital at Paris. This displays the
dentition of a child twenty-seven months old, in which the two upper
median incisors are typical Hutchinson teeth. M. Fournier adds that
later researches show unmistakably that the temporary teeth may be
affected by hereditary syphilis in the same manner as those of the
second dentition, although not more than one case of the former is
recognized to fifteen or twenty of the latter. He thinks, however, that
this proportion would be greatly modified if in the autopsies of young
children the alveoli were opened to examine the embryonic teeth. M.
Parrot by this means claims to have often discovered lesions of the
milk teeth.]

As a matter of course, so positive a statement in a matter of such
gravity and importance excited considerable criticism, and the views of
Mr. Hutchinson have never been without earnest and often able
opponents; but it is safe to say that time has only served to place
them on a surer foundation and to enhance their value in the eyes of
the profession. That they have undergone no material change in the mind
of their distinguished author is shown by his expressions of opinion
during the debate on syphilis in the London Pathological Society in
1876,[127] and still later by the following memoranda which he gives as
a guide in diagnosis:[128]

"1. No special peculiarities are to be looked for in the first set of
teeth.

"2. There can be no more serious blunder than to imagine that bad teeth
in proportion to their badness of form are to be suspected of syphilis.

{295} "3. The upper central incisors are the only teeth which are
positively characteristic. The others may afford corroborative
testimony, but are not to be relied upon alone.

"4. The chief peculiarity is a general dwarfing of the tooth, which is
both too short and too narrow, and, from its sides slanting together,
presents a tendency to become pointed. This tendency to pointing is
always defeated by the cutting off of the end, the truncation being
usually effected in a line curved upward, so as to produce a single
shallow notch. At the bottom of this notch the enamel is deficient and
the dentine exposed, but there is no irregular pitting, as in
stomatitis teeth.

"5. The malformations are unusually symmetrical and affect pairs of
teeth. The two central incisors resemble each other, and the two
laterals are also alike. If any defect passes horizontally across all
the incisors at the same level, and affects them all alike, it is
probably not due to syphilis.

"6. In syphilis the lateral incisors usually show little or no
malformation.

"7. The occurrence of the peculiarities due to syphilis and those due
to mercury in the same mouth are exceedingly common."

[Footnote 127: _London Lancet_, 1876, pp. 56 and 535.]

[Footnote 128: _Illustrations of Clinical Surgery_, fasciculus xi.,
London, 1878.]

The great importance of the subject seems to me to justify one more
quotation, as showing the opinion at a very recent date of men well
qualified to judge of the correctness or inaccuracy of these
statements. Mr. C. Macnamara and Dr. Thomas Barlow[129] say: "The
characters of the teeth are so valuable when present that it is
important to have them clearly noted--the more so that, in spite of Mr.
Hutchinson's clear description, they have been much misrepresented. It
may be pointed out--(1) That only the upper median permanent incisors
are characteristic, and sometimes only one of them is typical, of the
disease; (2) that these teeth are generally a little apart, instead of
being in apposition, and are more or less dwarfed; (3) that in a
typical specimen the width of the cutting edge is narrower than the
width of the tooth as it emerges from the gum; (4) that a typical
syphilitic tooth presents a single notch, not a serrated margin; and
that occasionally, if the notch has not been actually scooped out,
there is a little lunula-shaped area which may readily become a notch;
(5) finally, that although such teeth, when present, are absolutely
pathognomonic, the existence of normal permanent upper median incisors
by no means excludes the existence of hereditary syphilis."

[Footnote 129: On behalf of the Collective Investigation Committee, who
have issued a circular designed to elicit information as to the effect
of syphilis on the civil population of Great Britain. This circular has
been sent to physicians, and contains queries as to various points
relating to the symptoms of inherited syphilis; among others as to the
existence in any given case of "notched, dwarfed upper median
incisors," which, with or without other symptoms, would establish the
diagnosis of that case. The observations above quoted are explanatory
of this question (_The British Medical Journal_, Dec. 16, 1882).]

I believe this may fairly be taken to represent the general belief at
the present day among those best qualified to pronounce upon the merits
of the case; and I may say that it is unqualifiedly my own opinion,
arrived at after some experience and considerable investigation into
the literature of the subject. It is not uncommon, however, to hear
doubts expressed as to the value of this sign in the diagnosis of
syphilis, and at intervals articles are written or papers read to prove
that it is not of the uniform and distinctively conclusive significance
that has been attributed to it.[130]

[Footnote 130: "I cannot say more in favor of the diagnostic values of
these teeth than that, when {296} present in typical form, they have a
certain weight in favor of the existence of hereditary syphilis in the
given subject" (Van Harlingen, article "Syphilis" in _Internat. Encyc.
of Surgery_, vol. ii. p. 565). "It has been the custom from time to
time since Mr. Hutchinson made his observation to question the validity
of his views, both as to the fact of interstitial keratitis being due
to hereditary syphilis, and as to the diagnostic values of the
so-called characteristic teeth. Thus, it has been asserted, not only in
England, but on the Continent, and especially in Germany, that the
disease may be the result of malnutrition in scrofulous and rickety
subjects; and it has been maintained that the malformation of the teeth
is the simple arrest of development in a perverted constitution from
other causes than syphilis" (Bumstead and Taylor, _op. cit._, p. 701).
Garretson says (_Oral Surgery_, p. 316): "Observations will be found to
greatly vary concerning the existence of any constancy in phenomenal
expressions of the teeth in this relation."

M. Magitot, who has for some years been supposed to pay especial
attention to this subject, has recently written an elaborate paper
(_Gazette des Hôpitaux_, Sept. 29, Oct. 4, 11, and 18, 1881) to prove
(1st) that dental erosions, as he calls them, are not due to syphilis;
and (2d) that they are due to, or are almost invariably associated
with, infantile convulsions. He has collected a number of interesting
facts, but a very careful study of his article and inspection of his
diagrams have convinced me that he does not recognize at all the
special peculiarities of the Hutchinson teeth, but includes under his
title of "erosion" a variety of widely-differing conditions. He has
altogether misinterpreted Mr. Hutchinson's views as stated in his
_Memoirs on Certain Diseases of the Eye and Ear due to Inherited
Syphilis_, and represents him as at that time (1863) believing that the
cause of the change in the incisors was a mercurial stomatitis. The
quotation on p. 294 sufficiently refutes this absurdity. Of course the
paper as an argument against the syphilitic origin of these teeth is
without the slightest value.]

Corroborative evidence--which, to my mind, is very strong as coming
from men whose opportunities for observation of dental peculiarities
are almost unlimited--is found in the writings of those gentlemen who
have devoted themselves to dental and oral surgery exclusively. Mr.
Henry Moon[131] says:[132] "My observations on this subject extend over
some twelve years, and include some hundreds of cases; and although, in
some details as to the manner of causation, I may differ from the view
published by Mr. Hutchinson (before knowledge on tooth-development was
advanced as it is at present), yet I must coincide entirely with his
general conclusions." "The question really is this: Is there one
peculiar conformation of the teeth due to inherited syphilis and not
produced by any other cause? The evidence in favor of an affirmative
answer to this question appears to me to be so strong that I think the
onus of disproof rests with the sceptics."[133] These views were
coincided in by the majority of the gentlemen to whom they were
addressed, all of them dentists and surgeons of experience and repute,
and who included men so well known to the profession as Mr. Oakley
Coles, Mr. Samuel Cartwright, Mr. Charles Tomes, and others.

[Footnote 131: Author of the section on "Surgery of the Teeth"
incorporated in Bryant's _Surgery_.]

[Footnote 132: _Proceedings of the Odontological Society of Great
Britain_, vol. ix., 1877, pp. 238, 239. In the same journal for 1875,
vol. vii. p. 17, Langdon Down says that whenever he has discovered
syphilitic teeth he has "never failed to find confirmatory evidence of
the syphilitic history of the case."]

[Footnote 133: He says elsewhere (Bryant's _Surgery_, 3d Am. ed., p.
429) that the most characteristic change in these teeth is "the
lessened breadth of the cutting edge as compared with that of the neck,
the vertical groove on their anterior face being often absent, and the
notch on their cutting edge not being an absolutely constant feature,
and being also subject to obliteration through wear."]

It may be considered as well established, then, that these peculiar
teeth--stunted, abnormally narrow at the cutting edge, crescentically
rounded with the convexity upward, and the surface inclined upward and
forward instead of backward as in normal teeth, widely separated, but
converging at their lower edges--are pathognomonic of hereditary
syphilis.[134] They are {297} often described as pegged, having been
likened to a row of pegs stuck in the gums. This appearance is due to
the fact that they are shortened, often projecting not more than half
the normal distance from the gum, and are also widely separated; which
abnormalities often affect the adjoining teeth as well, and sometimes
the entire dentine. It has been asserted that other specific
peculiarities are to be found associated with those of the incisors,
and Mr. Moon describes as characteristic, and figures[135] small
dome-shaped first molars with suppressed angles and absence of enamel
from the masticatory surfaces. He believes also that when the upper
incisors are typical it is exceedingly rare for the lower incisors to
be altogether unaffected.

[Footnote 134: Mr. Hutchinson stated in 1877 that in spite of the fact
that many years previously he had challenged any one to bring forward a
patient with well-marked syphilitic teeth in whose history no evidence
of syphilis could be found, none had come.]

[Footnote 135: _Proc. of Odont. Soc. of Great Brit._, vol. ix. pp. 241,
242; Bryant's _Surgery_, Am. ed., p. 429.]

[Illustration: FIG. 11. Serrations of Normal Incisors.]

[Illustration: FIG. 12. Syphilitic Incisors.]

A mistake which I know, from observation, to be frequently made is the
confusing of the normal serrations of the cutting edges of
recently-erupted normal incisors with the peculiar crescentic edges of
the syphilitic teeth. It seems worth while to call especial attention
to this, on account of the unpleasant consequences which often follow
injudicious questioning based on such supposed syphilitic phenomena.
Indeed, the space which has been devoted to this subject of syphilis of
the teeth is well warranted, I am sure, by the fact that not only do
diagnosis, prognosis, and treatment in cases of great severity, and in
both children and parents, often depend upon a recognition of these
peculiarities, but that in addition to the clinical and therapeutic
problems there are others the solution of which is dependent upon the
same knowledge on the part of the practitioner, and which may involve
reputation, marital relations, and personal honor and happiness.

The approximate cause of these peculiarities in the incisors can hardly
be said to have been demonstrated. Mr. Hutchinson thought at one
time[136] that they were due to a stomatitis or an alveolar
periostitis, but he has since changed his mind as to that point,
believing now[137] that the syphilitic tooth is the result of an arrest
of development in the central or first-formed portion of the dentine.
The incisors being made up of these lobes or denticles, and dwarfing of
the middle one taking place, the two lateral ones fall together. This
accounts at once for the small size of the tooth, its shape of an
inverted truncated cone, and its crescentic edge.[138] If it were due
to stomatitis, it would be more likely to be equally {298} distributed,
syphilis in its late manifestations being notably unsymmetrical; there
would be no rational explanation of the involvement of one or two teeth
while those on either side so frequently escaped; if it were mercurial
stomatitis, the enamel too would be involved, as is not usually the
case in the syphilitic teeth. It is possible that the central incisors
are chiefly affected because they, with the first molars--also affected
according to Mr. Moon--and the lower incisors--not infrequently
involved--are the first-formed teeth.

[Footnote 136: "The physiognomonical, dental, and other peculiarities
by which we recognize the subject of inherited taint when advanced
beyond the period of infancy are all of them the direct consequences of
special inflammations from which the patient has suffered at former
periods; _e.g._ the synechiæ and lustreless iris of iritis; the
malformed teeth of periostitis of the alveolus and dental sacs; the
protuberant forehead of hydrocephalus; the flattened nose of snuffles;
the pale, earthy, opaque skin of cutaneous inflammation and eruption"
(_Aphorisms respecting Constitutional Syphilis_, 1863).]

[Footnote 137: _Proc. of Odont. Soc. of Great Britain_, vol. ix., p.
248. See also _ibid._, pp. 241, 242, remarks of Mr. Moon; also _Monthly
Review of Dental Surgery_, June 15, 1877.]

[Footnote 138: The denticle theory of formation is not necessarily
opposed by the fact that there is only a single undivided pulp-cavity
in these incisors. Instances of the separate formation of processes of
dentinal pulp while others are being used and worn away, all of them
finally to unite in a common pulp-chamber, have been observed in lower
animals, as in the molar of the elephant.]

The most elaborate article upon syphilitic teeth which has appeared
since Mr. Hutchinson's original memoir is one by Fournier,[139] in
which, after a very broad and comprehensive consideration of the
subject, he arrives at the following conclusions: The hereditary
influence of syphilis shows itself in the dental system in two ways,
very unequal in point of diagnostic value--viz. first, by a retardation
of evolution; second, by the arrest of growth and modifications of
structure. The phenomena belonging to the second class may be grouped
as follows: First, _dental erosion_. This is due to imperfect formation
of the tooth, the result of a temporary stoppage in its development;
but as it produces an appearance like that of worm-eaten wood, it has
been called erosion, though in so far as the word conveys the idea of
the wearing of a surface which has been previously normal, it is
incorrect. The tooth affected with syphilitic erosion has never been
normal. The different forms of erosion can be subdivided into groups
according as they affect the face or the free edge or grinding surface
of the tooth. Of those involving the face there are four types:
Erosions _en cupule_, consisting of small excavations or cups in the
surface of the crown; erosions _en facettes_, in which the surface
presents a series of small planes, as though they had been filed;
erosions _en sillon_ when there is a linear excavation in the crown of
the tooth in the shape of a transverse groove; and erosions _en nappe_,
in which the whole surface is discolored, disorganized, and
honeycombed.

[Footnote 139: _Archives de Derm. et Syph._, Sept. 25, Oct. 2, Oct. 9,
1883. A translation made by the writer may be found in the _Dental
Cosmos_ for January and February, 1884.]

A second group of erosions affects the free edge of the tooth, and
includes the Hutchinson teeth, with several less important varieties.
Dental erosions are multiple, symmetrical, maintain the same level on
the crowns of corresponding teeth, and are situated at different
heights on the crowns of teeth of different classes. It is evident,
therefore, that they are the result of a morbid influence of a general
character. There are three theories as to their etiology: (_a_) that
they have no relation to syphilis,[140] but are always connected with
infantile eclampsia; (_b_) that they are exclusively the result of
hereditary syphilitic influence; and (_c_) that they are simply
ordinary lesions originating from syphilis with marked frequency, and
even in one form--the Hutchinson tooth--appearing to originate only
from it. This latter view is the one adopted by Fournier himself.
Continuing to group the symptoms due to arrest of growth and
modification of structure, we have, second, _microdontism_, or dwarfing
and stunting of the teeth--pegged teeth; third, _dental amorphism_, in
{299} which the teeth are strangely distorted or even transformed in
type; fourth, _dental vulnerability_, or extreme susceptibility to all
traumatic or disintegrating influences.

[Footnote 140: M. Magitot, _Treatise on the Anomalies of the Dental
System_, Paris, 1877; _Clinical Studies on Erosion of the Teeth
considered as a Retrospective Sign of Infantile Convulsions_, Paris,
1881; Castanié, Paris, 1879, Thesis No. 384; Rattier, Paris, 1879,
Thesis 569; and others.]

Interstitial Keratitis.--The frequency of this form of diffuse
inflammation of the cornea, and the diagnostic significance which has
been so positively attributed to it--and has been as positively
denied--render it of special interest to the general practitioner, who
is almost certain to meet with occasional cases, and should be prepared
to recognize its possible relation with other, and often graver,
conditions.

It begins, commonly, as a slight, diffused haziness situated in the
substance of the cornea itself, usually not far from the centre, and at
first affecting only one eye. This depends at this stage on the
presence of a number of little distinct dots of inflammation, limited
to circumscribed, almost microscopic, areas, but later, in a few days,
these coalesce, and at the end of a few weeks the whole cornea will
probably have become nearly or quite opaque, looking like ground glass.
There is no ulceration, and but little congestion as compared with that
seen in other inflammatory diseases of the eye, although in the
majority of cases there is a fulness of the ciliary vessels and a
little photophobia with pains around the orbit. This condition may
persist for one or two months, after which the other cornea is nearly
always attacked,[141] and is similarly affected, although the disease
is apt to pass through its different stages rather faster than in the
first eye.

[Footnote 141: In 91 out of 102 cases in Mr. Hutchinson's series--in 6,
the left alone; in 5, the right alone (_op. cit._, p. 123).]

When the height of the disease is reached the corneæ are nearly opaque,
a bare perception of light remaining, so that the patient is just aware
of the difference between its presence and absolute darkness. Then the
cornea which was first involved begins to clear; this is soon followed
by improvement in the other one, which in the course of a year or two
results in a return to fairly good sight, although in most cases there
remain a slight haziness and an abnormal expansion of the cornea.

This favorable result is much influenced by the character of the case,
which is sometimes very mild from the outset, and by the thoroughness
of the treatment. It is sometimes complicated with iritis,
kerato-iritis, cyclitis, posterior choroiditis, secondary glaucoma,
etc., in which cases of course the prognosis is most unfavorable. Even
in ordinary cases it should be guarded in respect to perfect
restoration of function, as clearing of the corneæ may reveal adhesions
from iritis or spots of choroiditis disseminata, which could not, of
course, have been previously detected. In very mild cases, however,
without much evidence of involvement of the other tunics, and which
have been submitted to treatment early, it is not uncommon for the
cornea to regain almost perfect transparency.

The diagnosis of this condition may generally be made with ease. The
ground-glass appearance in the earlier stages and the dull pink or
salmon color in the more vascular stage are very characteristic. The
vascularity differs from that attending other chronic forms of
keratitis, granular lids, etc., in which the vessels are large and
superficial, in that in the syphilitic keratitis they are much deeper
and very closely interwoven, so that the effect is almost that of an
ecchymosis. In other cases both eyes are not so apt to be affected, nor
is the tendency to spontaneous cure {300} so marked. The absence of
ulceration and the very slight degree of accompanying sclerotic or
ciliary congestion are also valuable features.

The conditions which Mr. Hutchinson has known to be most frequently
mistaken for it[142] are certain forms of inflammation following
small-pox and very superficial ulcers in a healing stage, together with
cases of vascular conjunctiva.

[Footnote 142: _Op. cit._, p. 128.]

There is also said[143] to be some difficulty in distinguishing it from
a form of non-syphilitic relapsing cyclitis with corneal opacities and
iritis. This, however, is a disease of adults, often limited to one
eye, with a strong tendency to relapse, the opacities being more
abruptly defined and limited to the region near the circumference.

[Footnote 143: Edward Nettleship in Hill and Cooper, _op. cit._, p.
267.]

The chief point of interest, however, in the diagnosis of interstitial
keratitis is its association with other symptoms of syphilis, upon
which, for the general practitioner at least, the diagnosis will
usually depend.[144] Mr. Hutchinson's conclusions, drawn from an
analysis of 102 cases of interstitial keratitis, bear strongly upon
this point, and are as follows:[145] A large proportion of all cases
occur in patients between the ages of eight and fifteen, the disease
being comparatively rare in early childhood, and still more so after
adult age has been reached. He never saw it begin after the age of
twenty-six. The patients presented the physiognomical peculiarities of
inherited syphilis (see p. 313). In more than half the cases the
previous history, especially as regards infancy, was one of hereditary
syphilis, and in many instances there was a clear history of infantile
syphilis in brothers or sisters. In half the cases no questions were
asked as to the existence of venereal disease in the parents prior to
the birth of the child. In 29 out of the other half such disease was
freely admitted. All the patients had lost in early life nearly half
their brothers and sisters. Omitting miscarriages and premature births,
it was found that 77 mothers had borne 547 children, of whom only 284
remained alive--an excessive rate of mortality. It appeared in the
large proportion of cases in the eldest children in their respective
families--a circumstance to be expected in view of the usual behavior
of hereditary syphilis in families. Undoubted syphilitic lesions, such
as nodes, ulceration of the palate, etc., are not infrequently
associated with the keratitis.

[Footnote 144: "To those who have not had opportunities for observation
at an ophthalmic hospital I would recommend that the diagnosis should
be held to be doubtful if the patient does not present the
peculiarities of teeth and physiognomy which I have described, since we
find that the latter are the almost invariable concomitants of the true
disease" (Mr. Hutchinson, _op. cit._, p. 128).]

[Footnote 145: _Op. cit._, pp. 109-129.]

Examination of large numbers of cases has, I believe, established the
general accuracy of these statements; and although there is still much
difference of opinion as to the exact relation between syphilis and
this form of keratitis--whether, for example, it is a symptom of
syphilis itself or of a cachexia frequently produced by syphilis, but
often by other systemic diseases affecting nutrition--yet, on the
whole, so far as I am able to judge of the question outside of its
purely technical and ophthalmological relations, I think the weight of
modern authority is chiefly on the side of a distinct and practically
invariable relation of cause and effect between inherited syphilis and
the corneal inflammation.

{301} Mr. James Dixon,[146] for example, proposes to call the disease
syphilitic keratitis, and says it is met with exclusively as a sequel
of an inherited taint. He adds: "We may meet with some syphilitic
keratitis in patients with healthy physiognomy and deformed teeth, or,
still more rarely, in those with faultless teeth and the syphilitic
cast of features; but to find the true form of keratitis in connection
with both good teeth and good complexion is, I think, next to
impossible." Many other ophthalmologists express themselves to the same
effect more or less strongly. Nettleship,[147] Noyes,[148]
Förster,[149] Macnamara,[150] De Wecker,[151] and Carter[152] may be
mentioned as having ranged themselves upon this side. On the other hand
we have Schweiger,[153] Maunther,[154] Sæmisch,[155] Soelberg
Wells,[156] and others who are not convinced that syphilis is the sole
nor even, in the opinion of some of them, the principal cause of this
disease.

[Footnote 146: Article on "Diseases of the Eye," Holmes's _System of
Surgery_, Am. ed., vol. ii. p. 71.]

[Footnote 147: _Op. cit._]

[Footnote 148: _Text-book of Ophthalmology_.]

[Footnote 149: _Handbuch der gesam. Augenheilkunde_, vol. vii. p. 186,
1876.]

[Footnote 150: _Op. cit._]

[Footnote 151: _Ocular Therapeutics_, trans. of Forbes, 1879, p. 124.]

[Footnote 152: He even asserts the converse to be true, which is going
beyond what I believe can be established in regard to the invariable
connection between the two diseases. He says: "The subjects of what we
call simply inherited syphilis are liable--nay, are almost sure--to
suffer from a peculiar form of interstitial keratitis."]

[Footnote 153: "Hutchinson's view, that this form of keratitis is to be
regarded as the direct consequence of congenital syphilis, has not been
generally accepted in Germany" (_Handbook of Ophthalmology_, p. 298).]

[Footnote 154: Zeissl's _Jahrbuch der Syphilis_, 1875, p. 288.]

[Footnote 155: Graefe and S.'s _Handbuch d. Augenheilkunde_, 1875, vol.
iv. p. 264.]

[Footnote 156: _Diseases of the Eye_, p. 138.]

Probably the conclusions of Jullien[157] most nearly express the views
of those who do not accept in its entirety the syphilitic theory of the
disease. He concludes--(1st) that interstitial keratitis coincides
frequently, but not invariably, with syphilitic derangement of the
dental apparatus; (2) that it is associated with a feeble constitution
and with malnutrition, and is thus produced indirectly by scrofula,
rheumatism, or syphilis, each of which by its debilitating influence
may give rise to a diathesis which favors such morbid developments.

[Footnote 157: _Op. cit._, p. 1013.]

As a specimen of the evidence which is produced by observers other than
Mr. Hutchinson--who, as he himself observes, may be suspected of "that
bias which almost necessarily warps more or less the judgment of one
who supposes himself to have noted something new"[158]--the statistics
of Förster,[159] who apparently takes an unbiassed view of the
question, may be noted: In a total of 214 cases of interstitial
keratitis evidence of hereditary syphilis other than the corneal
disease was noted in 146 (68 per cent.). This number was made up as
follows: _a_, evidence of syphilis in parent, 17; _b_, evidence of
hereditary syphilis in other members of the family, 14; _c_, evidence
in the patient other than keratitis, 115; characteristic teeth, 69;
evidences in physiognomy, bones, skin, palate, or choroid, 46. In the
remaining 68 cases (32 per cent.), though inheritance of syphilis was
not proved, there was, almost without exception, strong suspicion of
that disease apart from the keratitis.

[Footnote 158: _Op. cit._, Preface, p. x.]

[Footnote 159: Graefe and Sæmisch's _Handbuch_, vii., Part I.]

The condition of the permanent central upper incisors was noted in 138
of the cases; of these they were typical in 73 (53 per cent.);
suspicious in 34 (24 per cent.); normal in 31 (23 per cent.). In 15
cases {302} the permanent teeth had not been cut, and in the remaining
61 the state of the teeth was not recorded.[160]

[Footnote 160: Note by Mr. Nettleship, Hill and Cooper, _op. cit._, pp.
263, 264.]

This seems to me such direct and such unequivocal clinical evidence
that it is safe to say, as of the question of syphilitic teeth, that
the burden of disproof rests with the doubters, and that we may venture
the assertion that interstitial, diffuse, or parenchymatous keratitis
is a symptom of inherited syphilis, and that the unmistakable presence
of the former disease is sufficient proof of the existence of the
latter.

Syphilis of the Nerve-Centres and Nerves.--Until a comparatively recent
period our only guide to the course and progress of the nerve diseases
of inherited syphilis was to be found in analogy. We knew, for
instance, that in acquired syphilis three forms of cerebral disease
could be recognized in a general way--one characterized by sudden
attack of paralysis, in which the lesion was usually thrombosis from
specific endo-arteritis; one in which the symptoms of brain tumor were
present, and in which gummata were the cause of the difficulty; and one
in which pain, headache, and various functional or convulsive
disturbances--chorea, epilepsy, paralysis of single nerves, etc.--were
the customary phenomena, and in which periosteal, meningeal, or
neuroglial thickenings constituted the pathological basis. The last two
are often intermingled both symptomatically and histologically.

Heubner[161] divides cerebral syphilis into three groups, two of which
very closely resemble those I have described. In one, however, he
includes both the general physical disturbances, incomplete paralysis,
and final coma characteristic of tumor and the epileptiform attacks so
often due to peripheral or meningeal irritation. This combination is
explained by the results of his autopsies, which disclosed in 26 cases
in which these symptoms were conjoined a gummous growth in the pia
mater of the convexity of one of the cerebral hemispheres, either
limited and superficial or involving more or less of the cortex and
forming a distinct tumor. The epileptiform attacks were present in 19
out of these 26 cases, while in 20 other cases where the growth was
limited to the white substance at the base of the brain they were
present only twice. This second form is the apoplectic, followed by
general hemiplegia, and depending on disease of the cerebral arteries.
His third division is a very ill-defined one, depends much for its
limitations upon subjective symptoms, and is of no special interest as
applied to the subject of inherited syphilis.

[Footnote 161: _Ziemssen_, vol. xii.]

Althaus[162] also makes three divisions, two of which are as follows:
(1) Cerebral tumor--a gumma either hard or soft. There are then
nocturnal headache, sleeplessness, epileptiform attacks, the various
phenomena produced by involvement of the cerebral nerves, etc. (2)
Disease of the arteries, apoplexy, or softening, followed by
hemiplegia.

[Footnote 162: _Medical Times and Gazette_, Nov. 10, 1877.]

Hutchinson makes a similar division of lesions and symptoms,[163] and
the observations of Jaksch, Wilks, and Hughlings Jackson more or less
closely coincide with this general classification.

[Footnote 163: _Ibid._, Feb. 17, 1877.]

Now, in spite of certain striking differences--more apparent than real,
however--between inherited and acquired syphilis as regards cause,
{303} duration of stages, etc., the essential pathological changes are
the same. When syphilis in its later periods attacks the brain or
spinal cord or nerve-trunks or vessels of a foetus, it proceeds just as
in the adult, the same characteristic accumulation of cells taking
place and setting up an arteritis or a meningitis, thickening the
sheaths of nerves, or constituting a pericranial node or a gumma
according to their number and their situation. We would accordingly
expect to find in subjects of inherited syphilis manifestations closely
allied to those observed in the adult; and the observations of
Barlow,[164] Graefe,[165] Jackson,[166] Heubner,[167] Dowse,[168] and
Hutchinson,[169] though comparatively few in number, have already
demonstrated the correctness of this supposition.

[Footnote 164: _Trans. of Path. Soc. of London_, vol. xxviii., 1877.]

[Footnote 165: _Archiv für Ophthalmologie_, Bd. 1, Ab. i.]

[Footnote 166: _Journal of Mental Science_, Jan., 1875.]

[Footnote 167: _Ziemssen_, vol. xii.]

[Footnote 168: _Syphilis of the Brain and Spinal Cord_, London, 1879,
chapter on "Hered. Syph.," p. 67.]

[Footnote 169: _Med. Times and Gazette_ (? Feb. 17, 1877).]

We find, thus, that in these patients meningitis, growths, and arterial
disease constitute the three clinical divisions of the disease which
have thus far been distinctly differentiated, and the reported cases,
with or without autopsy, fall naturally into these classes.

These cases are naturally few, and to make them absolutely reliable it
is necessary to have unmistakable evidence of hereditary syphilis in
some other form and the demonstration of syphilitic lesions at an
autopsy. The case of Dowse, however,[170] includes these requirements.
A child twelve years of age, of syphilitic parents, with a history of
coryza, sore eyes, and a tubercular syphilide, was attacked with
epilepsy, diplopia, facial paralysis, etc., and finally died. At the
autopsy three gummatous growths of the surface of the brain were found,
and the vessels of the base were found to have undergone the special
changes described by Heubner. Their lumen was in some places nearly
occluded by an accumulation of spindle-shaped cells between the tunica
fenestra and the epithelial lining; and interspersed with them, but
particularly in the muscular and adventitious coats, were to be seen
enormous quantities of round cells which in many parts seemed actually
to replace the normal structures. Dowse's other cases are not at all
conclusive in their clinical histories; even the diagnosis was not
established by autopsy.

[Footnote 170: _Op cit._, pp. 71-75.]

Barlow's two cases were both very young children, and are extremely
convincing.[171] A child four months old, with snuffles, serpiginous
ulcers, etc., and with a syphilitic father, had epileptiform attacks,
followed by laryngismus, carpo-pedal contraction, and changes in the
choroid. She died aged about fifteen months, and the autopsy disclosed
thickening of the pia mater, evidently not tubercular, and changes in
the arteries, which in the gradual narrowing of the lumen of the
vessel, the absence of ulceration or disintegration or calcification,
and the continuity and extent of the cell-proliferation are as
different as possible from ordinary atheroma, but correspond precisely
with the description of Heubner's cases, which were undoubtedly the
subjects of acquired syphilis.

[Footnote 171: _Transactions of the Pathological Society of London_,
1877, vol. xxviii. pp. 287-291.]

In the second case the symptoms were associated chiefly with the
cranial nerves. These were nystagmus, paresis of facial muscles,
laryngeal spasms, etc. He died at the age of fifteen months. The
vessels of the base were extensively diseased as in the other case, and
the fourth, {304} fifth, sixth, seventh, and eighth pairs of nerves
were smaller by a new growth of a gummatous nature which had produced
almost entire atrophy of the nerve-cylinders. There were cicatrices of
the liver and spleen. Cases in which the diagnosis rested upon the
history, upon the co-existence of undoubted syphilitic symptoms, and
upon improvement under specific treatment are by no means rare.

Berkely Hill reports[172] a typical case of syphilitic epilepsy. A girl
aged nine was the first child of the family that had lived, two having
previously been born dead. She presented characteristic teeth, traces
of choroiditis, and, while under treatment, suffered from both
keratitis and iritis. Her first fit was when she was four years of age,
and the attacks had recurred frequently since that time. The convulsion
was confined to the left side. The left arm was very weak, the weakness
having come on gradually, and being especially great in the extensors
of the wrist. Under specific treatment the attacks ceased entirely. In
this case there was certainly organic disease, probably a gumma on the
surface of the right hemisphere.

[Footnote 172: _Op. cit._, p. 253.]

Other cases reported by the same author, numerous instances of nervous
troubles in inherited syphilis reported by Hughlings Jackson,[173]
Fournier,[174] Henoch,[175] and Hutchinson,[176] warrant the statement,
then, that the nervous diseases of inherited syphilis fall into the
same general category as those of the acquired disease; that they may
appear at any age, from three or four months to that of puberty; that
they depend for their production upon a cell-proliferation which,
according to its locality, results in the development of new growths,
the production of localized meningeal inflammations, or the obstruction
and inflammation of arteries; and that the symptoms are those usually
associated with such pathological changes, the diagnosis of syphilis
depending chiefly on the history and the presence of other specific
phenomena.

[Footnote 173: _Journal of Mental Science_, Jan. 8, 1875; _Transactions
of St. Andrew's Med. Graduates' Ass._, vol. i., 1868.]

[Footnote 174: _Annales des Derm. et Syph._]

[Footnote 175: _Nouveau Dict. de Méd. et Chirurgie_, p. 885.]

[Footnote 176: _Op. cit._]

The prognosis is more unfavorable than in similar cases in acquired
syphilis, the meningeal and vascular lesions being the most frequent
and showing themselves very obstinate even under careful treatment.
Spinal troubles, chorea, and idiocy have been attributed to inherited
syphilis, but must occur with great rarity.

Hill[177] reports a case of a child aged five years who had been
hemiplegic since he was two and a half years old, and who developed
paralysis of the flexors of the ankle in each leg.

[Footnote 177: _Op. cit._, p. 254.]

Keyes reports a case of a boy five years of age, with nodes on tibiæ
and other signs of inherited syphilis, who had two attacks of
paraplegia.

Hill reports a case of imbecility associated with inherited syphilis,
but there is no evidence that it was other than a coincidence.

Fletcher Beach found not more than 1 per cent. of syphilitic children
in the Dareult Asylum, and Mr. Mercier could only trace syphilis in 5
out of 220 female idiots, probably imbecile from birth.[178]

[Footnote 178: _Ibid._, p. 255.]

Hughlings Jackson only found 1 case of inherited syphilis among 80
{305} cases of chorea. The most carefully recorded cases of the latter
affection associated with hereditary syphilis are two reported by
Alison.[179]

[Footnote 179: _American Journal of the Medical Sciences_, July, 1877.]

Syphilis of the Spleen.--Disease of this organ in inherited syphilis is
especially important from two points of view. It is a valuable aid to
diagnosis, and by its size and the degree of persistence of the
swelling gives an approximate indication of the severity of the case.

Attention was first called to the frequency and importance of
enlargement of the spleen in early hereditary syphilis by Gee in a
paper read before the Royal Medical and Chirurgical Society in
1867.[180] He gave the histories of thirteen children in support of the
statement that such enlargement occurred in almost one-fourth of all
cases of hereditary syphilis, sometimes with, sometimes without,
enlargement of the liver and lymphatic glands. According to him, the
degree of splenic enlargement may be taken as a sort of index of the
severity of the cachexia; the majority of cases with great enlargement
die, but sometimes such children survive, the spleen gradually
diminishing in size as the health improves--not diminishing, however,
pari passu with such improvement, but remaining for a long time "a
monument of past cachexia."

[Footnote 180: _British Medical Journal_, 1867, vol. i. p. 435.]

Barlow,[181] ten years later, thought Gee had rather understated the
proportion of cases in which splenic enlargement occurs, he having
found it in 22 out of 28 children with definite hereditary syphilis.
Birch-Hirschfeld, Eisenschitz, and Tepel[182] corroborate these
observations, finding that the enlargement is almost invariable and
that the spleen is often double its normal size.

[Footnote 181: _Trans. of Path. Soc. of London_, Jan. 20, 1877.]

[Footnote 182: Quoted by Hill and Cooper, _op. cit._, pp. 164, 165.]

Mr. W. J. Tyson has reported[183] a cure of a child born of syphilitic
parents, in whom, at two years of age, the spleen extended downward
three and a half inches, reaching the crest of the ilium and
approaching closely to the umbilicus. The liver was not enlarged; the
urine was not albuminous. He ordered mercury with chalk, one grain
every morning and evening, and one grain of iodide of potassium, with
ten minims of syrup of iodide of iron to an ounce of water, three times
a day. Sixteen months later the spleen had become imperceptible, and
three years afterward the child was in excellent health.

[Footnote 183: _The Lancet_, Oct. 23, 1880.]

The diminution of the liver under treatment appears to take place
before there is any diminution in the size of the spleen.[184] This
persistence of the latter renders it, as has been stated, a valuable
diagnostic sign. In the paper already quoted from,[185] Macnamara and
Barlow allude to this as follows: Enlargement of the liver, although it
ought to be noted because it is often present in hereditary syphilis,
has but little value as a confirmatory symptom--first, because the
liver is proportionally large in infancy, and it is difficult to state
the limit of what is actually normal; and, secondly, because other
causes besides congenital syphilis lead to its enlargement.

[Footnote 184: Barlow, _British Medical Journal_, Jan. 20, 1877.]

[Footnote 185: _British Medical Journal_, Dec. 16, 1882.]

With regard to enlargement of the spleen the case is different. Gee's
observation, that in the early stage of infantile syphilis some
enlargement of the spleen occurs in a large number of cases, has been
abundantly {306} confirmed. Although with the subsidence of the other
symptoms this enlargement often disappears, so that on post-mortem
examination two or three m