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SYNONYMS.—Parkinson's disease; Shaking palsy; Trembling palsy;
Senile chorea; Chorea festinans. The first name is due to the fact
that the disease was first fully described by Parkinson in a book
published in England in 1817.

Paralysis agitans is a neurosis, chronic in its forms and

characterized by a tremor which gradually increases in extent and
severity. The tremor is not increased by voluntary muscular
movements. A peculiar manner of walking, known as festination,
comes on later in the disease, and there are also alterations in the
attitude of the head and trunk.

It is a disease which belongs to middle age, being rarely seen before

forty years, although cases are quoted by Charcot as early as twelve
and sixteen years. Constant and prolonged exposure to dampness
and cold seems to bring on the disease, and it is sometimes caused
by sudden emotion, like fear or distress. The following case is an
instance of the latter:

Case I.—Mr. A. M——, æt. fifty-two years, consulted me Oct. 1,

1883. He is a bookbinder by occupation. His habits have been good.
He had a chancre in 1861, but had no secondary troubles. He was in
the army from 1861 until 1866. In 1866 he went into business for
himself, and, although his business was large, he had no great
anxiety or worry. His general health has been good, and he has had
no illness except an attack of malarial fever about six years ago. In
May, 1883, he was standing by an elevator door on the fourth floor of
his place of business, and, seeing that the elevator was caught by
something, released it. It immediately fell with a crash to the second
floor, and as there were two or three persons on it, Mr. M——
thought they must have been killed or severely injured. He was
greatly excited and alarmed, and soon after he had assured himself
that none of the occupants of the elevator had been hurt, discovered
a trembling of the right hand. The tremor has continued ever since,
and has extended to the arm and leg.
On examination there is seen a coarse tremor of the right arm and
leg while the limbs are at rest. A voluntary muscular movement stops
the tremor, and it also ceases during sleep. When he makes an effort
with the right hand, as, for instance, in squeezing the dynamometer,
the tremor ceases in the arm, but becomes greatly exaggerated in
the right leg. While occupied in doing anything he does not notice the
tremor, and it stops when he is lying down.

The dynamometer shows, right hand 150°, left hand 120°. After two
years have elapsed the disease has gradually progressed in
severity.

SYMPTOMS.—The course of the disease has been divided into three

stages—the period of invasion, the stationary period, and the
terminal period.1
1 Lectures on Diseases of the Nervous System, by J. M. Charcot.

Period of Invasion.—There are several modes of invasion, but the

most frequent by far is slow in its onset. The disease comes on
gradually, first showing itself as a slight tremor in the hand or fingers
while the part is at rest. It is not constant, and ceases as soon as the
patient's attention is called to it. There is sometimes preceding the
tremor rheumatic or muscular pain in the affected arm. The tremor
may first occur in the foot. Should the disease begin in the hand—
and this is most common—the movements are peculiar. They may
consist of a fine rhythmical tremor, or the fingers move in a
methodical way over each other. Charcot speaks of the thumb and
forefinger being rubbed together as if the patient were spinning wool.
While this movement of the thumb and finger is going on the wrist is
being flexed by jerks. During the early stages of the disease the
tremor is observed only at intervals. It comes on intermittently when
the patient is not thinking of it and while the limb is at rest, and
ceases as soon as any voluntary muscular effort is attempted. The
act of grasping the hand or taking up an object is enough to check it
for the time. In a patient now under my care I have often noticed
during the early stages a well-marked tremor of the right hand while
it was lying in her lap, but it would at once cease when I called
attention to it. As soon as the mind of the patient was diverted to
some other subject the tremor would begin again.

As the disease progresses voluntary effort no longer controls the

tremor, or if it does at all it is only for a few seconds, when it begins
again. As the tremor increases in violence it extends to other parts of
the body. At first it may have been confined to the hand; now it
extends to the arm, a little later to the foot and leg on the same side.
Then the other arm will be affected, and finally all of the limbs will
succumb to the tremor.

Charcot speaks of decussated invasion—that is, the disease begins

in the right upper extremity, for example, and next passes to the left
lower extremity. This is a rare form; it is much more frequent to see
the hemiplegic type, which may persist for some time, or the
paraplegic type, when both legs are affected.

There is a progressive form of invasion when the tremor is not the

first symptom. The patient has neuralgic or rheumatic pains in the
limbs, which are afterward affected with tremor. Sometimes there is
some mechanical injury of the limb, which subsequently is the seat
of pain and tremor. The general health of the patient is at the same
time more or less impaired. There is a sense of general weakness
and lassitude; the temper is irritable, and there may be some vertigo.
The features and countenance are characteristic even at the earliest
periods of the disease. There is an absolute absence of expression,
and the features are fixed. The face looks like a mask, and although
the patient may smile or laugh, immediately after the features return
to the original blank expression. Amidon showed two cases of
paralysis agitans to the American Neurological Society in 1883, in
which there was no tremor whatever, but all the other features of the
disease were present.

After a great mental or moral shock the trembling begins suddenly,

abrupt invasion, as in Case I., or the case described by Charcot,
where the wife of a gendarme, seeing her husband's horse return
riderless to the barracks, received a shock of great severity, which
was followed on the same day by tremor. The tremor is at first
confined to one limb. It may even disappear for a time, but gradually
and slowly extends to the other limbs, and takes the same
progressive course.

Period of Stationary Intensity.—After the disease has become fully

developed the tremor is incessant. The intensity is not the same all
the time. It may be augmented by cold, over-excitement, or voluntary
effort, and is lessened by repose and sleep. The trembling ceases
during anæsthesia.

During this time all the characteristic movements are at their height.
The moving of the thumb and finger, already referred to, is present,
and seems like a partly co-ordinated movement. Charcot describes
these movements as being in some cases like the rolling of a pencil
or a paper ball between the thumb and finger, and in others the
movements, he says, are more complicated, and are like what occur
in crumbling a piece of bread.

The handwriting is almost illegible, and every letter shows the

excessive trembling of the hand, most marked in the up strokes of
the pen. All this time the head and neck are unaffected. There is no
nodding or shaking of the head to be observed on the closest
inspection. This is an important fact to bear in mind, for it is a
distinguishing feature between the disease under consideration and
disseminated sclerosis. There is no nystagmus, and the muscles of
the jaw are unaffected by tremor. The tongue, however, while lying
on the floor of the mouth undergoes tremor, and this is increased
when the organ is protruded.

The speech is slow and jerky, and the patient usually speaks in a low
tone of voice. He eats his food without difficulty, but in advanced
cases the saliva sometimes escapes from the mouth during
deglutition. A characteristic symptom of the disease is a rigidity of
the muscles of the extremities, trunk, and of the neck. When the
muscular stiffness first begins the patient complains of cramps
followed by a sense of rigidity. The flexor muscles are first affected.
This stiffness causes peculiarities in the patient's attitudes. The head
and neck are usually bent forward, and seem fixed in that position.
The body is inclined slightly forward in standing. The elbows are held
somewhat away from the chest, the forearms are flexed on the arms,
and the hands are flexed on the forearms. The thumb and forefinger
are extended and brought together as if holding a pencil. The other
fingers are also flexed. The attitude of the hand and the prominence
of the knuckles make it closely resemble the hand of rheumatoid
arthritis. In paralysis agitans, however, there are no bony deposits in
the joints, and no cracking is heard on bending the knuckles.

In the lower extremities there is often intense rigidity. Contractions

occur, and the legs are strongly flexed. The feet often are extended
in the position of equinus. It is this rigidity which causes the difficulty
in walking (Charcot).

The gait of the patient now becomes very striking. He gets up from
his seat slowly and with difficulty; hesitates a moment before starting
to walk; then, once having made a few steps, goes at a rapid pace.
The tendency is to fall forward; in order to preserve his equilibrium
the patient hurries forward as if to catch up with his centre of gravity.
This gait has been called paralysis festinans—festination or
propulsion.

Although propulsion is the usual form the gait assumes, sometimes

there is a tendency to fall or run backward. This tendency is not
always apparent even when it exists. Charcot has a method of
showing its existence which is very successful: when a patient is
standing he pulls her slightly backward by the skirt, and this is
sufficient to start a movement of retropulsion.

Propulsion and retropulsion are not necessarily always present.

Many cases progress to the end without these symptoms.

As the disease advances the muscles become more rigid and the
patient is confined to bed. He is, however, restless from a sense of
prostration and fatigue. He is unable to turn himself, and often calls
to his attendant to change his position. The sufferers from this
disease, although not having actual pain, complain of disagreeable
sensations. There is a constant sense of excessive heat whether the
temperature of the room be high or low. In winter they cannot bear
much bed-clothes at night, and prefer to wear very light clothing.
Associated with this sensation of heat is often profuse perspiration
(Charcot). Notwithstanding all these troublesome sensations the
sensibility of the skin is not changed. Heat and cold are readily felt,
and there is no anæsthesia or analgesia.

Terminal Period.—The duration of paralysis agitans is generally

great. The disease may extend over many years—even as long as
thirty years in some cases. As the tremors and rigidity increase in
intensity the patient becomes obliged to sit all day in a chair or is
confined to bed. Occasionally the tremor becomes less while the
rigidity increases. The nutrition suffers, and the muscles especially
become greatly wasted. Up to a certain point the intellect remains
unaffected, but late in the disease the mind fails. General prostration
of the whole system sets in, bed-sores occur, the urine and feces are
passed unconsciously, and the patient dies of exhaustion. It is not
often that the end comes in this way. It is much more frequent that
some intercurrent disease, like pneumonia, ends the life of the
patient. The disease is undoubtedly a most painful and trying one to
both patient and physician. It lasts for years, and there is no prospect
of relief. Charcot says that he has seen the terminal period last for
three or four years. The following case is a fair example of the
disease:

Case II.—Margaret Hays, aged fifty-four, single, applied for treatment

at the Infirmary for Nervous Diseases, Oct. 9, 1882. She is
housekeeper for her brother, who keeps a restaurant. She has had
to be up late at night, and has had very much washing to do for
many years. The kitchen in which she is most of the time is damp,
and opens into a yard into which she has often to go. About two
years ago she thought that she hurt herself in lifting something, and
soon after this, on putting her hands from hot into cold water,
suddenly felt a numbness in both forearms. One year ago she
noticed tremor in both hands and both legs. The tremor interfered
with her work, and has increased. She also felt as if there were loss
of power.
Present Condition.—The face is expressionless and looks as if it
were a mask. She articulates without using her lips to any extent,
and speaks in a low tone, scanning the syllables. The head is held
stiffly, and the attitude of the whole body is peculiar.

The tremor is fine, and is constant while the hands are at rest. On
voluntary effort the tremor ceases. On examination with the
dynamometer, right hand 95°, left hand 80°. She feels weak
generally, and says she cannot use her hands even to button her
clothes or to dress or undress without aid. She performs all
movements slowly and with great deliberation. The patellar reflex is
not excessive.

Sensation.—She has lost the sense of numbness she used to have,

but says she cannot feel a needle between the fingers when she
attempts to sew. There is loss of sensation in the finger-ends; the
compass points are not felt at less than three lines.

Her walk is slow and deliberate like all her movements, and there is
no festination. Her eyes were examined, and there was no decided
change in the fundus and vision was about normal. Pupillary reflexes
good.

This patient was under observation for several months, and steadily
grew worse. One peculiar feature was observed, however: it was
that at one time the tremor almost ceased, although all of the other
symptoms were worse.

ETIOLOGY.—As already mentioned, the causes which have been

observed are fright or sudden grief and prolonged exposure to cold
and dampness. A number of cases of the former are mentioned by
Charcot, and a case which I have related above is a good illustration
of paralysis agitans produced by fright. When caused in this way the
disease does not present any peculiar features in its progress or
termination. I have seen many cases in which the disease had been
preceded by more or less exposure to dampness. One of my
patients had worked in a basement room which was damp; another
(Case II.) was a great deal of the time in a kitchen which opened on
a wet yard, and she was constantly going in and out of doors, getting
her feet wet frequently. Sometimes irritation of a peripheral nerve
seems to have been the origin of the trouble. Charcot quotes several
cases of this kind.

Sex does not appear to exert any special influence in the production
of the disease. Some writers assert that it is more common in males
than in females, but Charcot in his large experience at La Salpêtrière
has not found this to be the case.

MORBID ANATOMY.—A number of autopsies have been made in cases

of paralysis agitans without any constant lesion of the nervous
system having been discovered. Charcot refers to three cases in
which he made careful post-mortem examinations in which the
results were negative. Parkinson and Oppolzer each report one case
in which was found induration of the pons, medulla, and cervical
portion of the cord. More recently, however, Charcot and Joffroy
have examined cases in which microscopic examination revealed
blocking up of the central canal of the cord by increase of the
epithelium of the ependyma and pigmentations of the ganglion-cells.

Leyden has reported a case in which the disease was confined to

the right arm, and on post-mortem examination a tumor of the left
optic thalamus was found.2
2 Quoted by Hamilton, Diseases of the Nervous System, p. 500.

Dowse and Kesteven found degeneration of the nerve-cells of the

anterior pyramids, changes in the olivary body, nucleus of the ninth
nerve, laminæ and corpus dentatum of the cerebellum and of the
anterior cornua of the spinal cord. Also cortical sclerosis of the right
lateral column of the cord and miliary changes in the white matter of
the corpus striatum and hemispheres.3
3 Ross, Diseases of the Nervous System, vol. ii. p. 797.

In this disease, as in chorea, there must be two classes of cases—

those in which there is no lesion to be discovered after death, and
others in which there are changes throughout the brain and spinal
cord more or less widespread. The cases in which the disease
comes on suddenly from some moral shock probably belong to the
former class; while in cases which have come on gradually during
senility one would expect to find organic changes in the nervous
system. Ross4 suggests that the cause of the tremor is probably a
diminution in the conductivity of the fibres of the pyramidal tract,
which prevents impulses from the cortex reaching the muscles in
sufficiently close proximity to produce a continuous contraction. This,
however, does not explain the cause of the tremor in the cases
where it began suddenly from fright.
4 Op. cit., p. 798.

DIAGNOSIS.—The only diseases with which paralysis agitans may be

confounded are disseminated sclerosis, senile tremor, and chorea in
the aged. From senile tremor it may be distinguished from the fact
that it begins before old age—that the tremor is more excessive and
the gait and facial expression are distinctive. Chorea in old persons
resembles paralysis agitans, but is not progressive, the tremor is not
lessened as a rule by voluntary effort, and the peculiar gait and
expression of the face are wanting.

There are many points of difference between paralysis agitans and

disseminated sclerosis, as can be seen below:

PARALYSIS AGITANS. DISSEMINATED SCLEROSIS.

Tremor ceases on voluntary Tremor induced by muscular
effort, or is not increased by it. effort, and ceases during repose.
Tremor regular and fine. Coarse tremor, becoming more so
during voluntary effort.
Face expressionless; tremor of Facial muscles affected;
face rare. nystagmus frequent.
No tremor of head. Tremor of head generally present.
Belongs to advanced age. Usually comes on before middle
age.
Propulsion (festination) and Staggering walk.
retrogression.

TREATMENT.—The results of treatment are not encouraging. Cases

have been reported in which cures were effected, but it is doubtful if
they were true instances of paralysis agitans. Hyoscyamus and
conium have been given with temporary relief to the tremor.
Trousseau recommends strychnia, but Charcot declares that it
aggravated the cases in which he gave it. Hammond advises the use
of galvanism, at the same time giving strychnia or phosphorus. I
have seen one case in which decided relief was obtained from
arsenic hypodermically, and another in which the patient was
benefited for a long time while taking small doses of strychnia
combined with iron and quinine, and at the same time static
electricity was applied.

CHOREA.

BY WHARTON SINKLER, M.D.

Chorea, or St. Vitus's dance, has been known for years, and the
literature of the subject, especially among the older writers, is as
extensive as that of any other disease.
It has been recognized by a variety of names, some of the most
common being derived from some saint who enjoyed a popular
reputation of power to cure the disease. For example, it has been
called St. John's dance, St. Guy's dance, St. Modestus's dance, and
St. Anthony's dance. Besides these names it has been termed
ballismus, paralysis vacillans, epilepsia saltatoria, and
orchestromania.

It will be observed that almost all of the names which have been
applied to the disease relate to a dancing movement. This arises
from the fact that the first notice of the affection dates back to the
fourteenth century, when a kind of religious mania appeared in
Southern Europe in the form of an epidemic. It was characterized by
excessive dancing and gesticulatory movements, and affected large
numbers of people at a time. In 1375 an epidemic which arose was
spoken of as St. John's dance, and in 1418, in another outbreak of
the disorder which occurred at Strasburg, by the order of the
authorities those suffering were conducted in troops to the chapel of
St. Vitus in Zabern, and there masses were said and other religious
ceremonies performed for its cure.

We are informed that St. Vitus removed from Sicily when a boy, at
the time of Diocletian's persecution of the Christians in the year 303,
and suffered martyrdom in Florence in company with Crescentia and
his tutor, Modestus.1
1 “Hecker's Epidemics of the Middle Ages,” Sydenham Society's Transactions,
contains full details of these outbreaks.

Von Ziemssen states that as a pandemic disease the dancing mania

died out in the fifteenth century, but that traces have remained on the
Rhine up to the present time.2
2 Cyclopædia of the Practice of Medicine, vol. xiv. p. 416.

In our own country there have been many epidemics of the same
disorder on a small scale, and we may regard the Shakers as
representing a type of the dancing mania. Weir Mitchell reports3 an
outbreak which occurred quite recently In a children's asylum in
Philadelphia. Prompt measures and separation of those affected
from the well children checked the disorder, which at one time
threatened to spread through the entire institution.
3 Lectures on Nervous Diseases, p. 69.

These epidemics were quite different from what we now call chorea,
and the individuals suffering were clearly affected by a psychical
disorder of an hysterical form. In time, however, the name has come
to be applied to a systematic disease characterized by irregular
spasmodic movements of the limbs and other parts of the body.

The disease has been divided by some writers into chorea magna
and chorea minor. The former, however, as described, is only an
aggravated variety of hysteria, and need not be considered in
connection with this subject.

Although the name chorea does not correctly describe the disease
under consideration, it has been used for so many years that it is
more convenient to retain it.

DEFINITION.—Chorea, as we now understand it, is a spasmodic

neurosis, characterized by constant involuntary and irregular jerkings
and twitchings of muscles or groups of muscles, which, in the
majority of cases, cease during sleep, and are accompanied by more
or less psychical disturbance in most instances.

ETIOLOGY.—Hereditary influence in the production of chorea is more

or less marked. In some cases the connection seems to be remote,
but in many instances it will be found that one of the parents has
suffered from some form of nervous disease or has inherited a
nervous diathesis.

George Huntington of Pomeroy, Ohio,4 has recorded some

remarkable instances of hereditary chorea. The affection, as he
describes it, differs in many features from chorea as ordinarily met
with, but it is apparently the same disease. It is found in the eastern
end of Long Island, and has been studied in several generations by
Huntington, his father, and grandfather. This part of Long Island is
remarkably free from the usual type of chorea, none of these
physicians having ever met with an example of it. The hereditary
chorea is confined to a few families. It occurs more frequently in
males than in females, and never attacks the patient until after
middle life. It comes on gradually and takes years to develop, but
when once established it yields to no form of treatment. In most
cases there is a marked tendency to insanity and suicide in the later
stages of the disease.
4 Medical and Surgical Reporter, April 13, 1872.

Some additional cases of hereditary chorea have been lately

recorded by Peretti in No. 52 of the Berliner klin. Wochenschrift,
1885, and others by Clarence King in the New York Medical Journal,
vol. i., 1885. The history of all these cases is strikingly like those of
Huntington, and establishes without question a distinct form of
chorea.

Mrs. N., one of Peretti's cases, had a mental affection with choreic
movements, and there was a history of a similar condition in her
parents and grandparents. Two of Mrs. N.'s four children, Mrs. A.
and Anton N., had chorea in adult life; some of these became
insane. Mrs. A. had five children; three of these became choreic; one
had tremors and one became insane. Anton N. had ten children; of
these six had chorea. In all of Peretti's cases the disease came on
after the age of forty years, and persisted. In several members of the
family insanity was associated with the chorea.

In the families where it occurs the nervous temperament

predominates. It sometimes will be found that neither of the parents
of the patient has had chorea or any other nervous disease, but that
an uncle or an aunt has had St. Vitus's dance in childhood.

Chorea may occur at any period of life, from infancy to extreme old
age. I have reported two cases in patients over eighty years of age5
—one at eighty-two and the other at eighty-six—who had
characteristic attacks of St. Vitus's dance. Robert Saundby has
collected twelve cases of chorea in the aged. The two cases just
referred to are included in the number. He considers the affection
very rare in old persons.6 The following case is an example of
congenital chorea, and I believe this to be very unusual. The
movements of all infants are choreic, so that it is difficult to say when
the chorea begins; still, it seems fair to infer that when a child has
never had any but choreic movements it is a case of congenital
chorea:

Case. I.—Jennie W——, aged nineteen years. Family history is good

as regards nervous diseases. Her mother was frightened by seeing a
case of chorea some time before the child was born. The
movements were observed at birth, and have continued always. The
patient was brought to my clinic at the Infirmary for Nervous
Diseases, and her condition noted as follows: The movements are
general and continuous; the arms and legs are in constant motion,
and the mouth is perpetually grimacing; there is tremor of the tongue
when it is protruded; volitional efforts increase the movements;
during sleep they cease entirely; tendon reflexes are normal; there is
no paralysis; heart-sounds are normal and the general health is
good; menstruation is regular, and the choreic movements were not
influenced in any way at the time of its first appearance.
5 Journal of Nervous and Mental Diseases, July, 1881.

6 Lancet, Nov. 24, 1884.

Chorea occurs most frequently during the period of approaching

puberty. Sée in an examination of 531 cases found 453 between the
ages of six and fifteen years.

I have examined the notes of 282 cases of chorea, most of which are
in the case-books of the Infirmary for Nervous Diseases; the rest are
from my own note-books. Of the 282 cases, 217 were between six
and fifteen years. They were distributed as follows:

Under 1 year 2At 7 years 23At 16 years 6

 At 2 years 6At 8 years 24At 17 years 10
At 3 years 4At 9 years 31At 18 years 5
At 4 years 7At 10 years 23At 19 years 5
At 5 years 9At 11 years 18At 20 years 1
At 6 years 24At 12 years 25At 21 years 3
At 22, 25, 27, 28, 35, 38, 82, and 86 years, each 1 case.

Sex exerts considerable influence on the disease. Sée states that

three-fourths of all the cases observed in the Children's Hospital in
Paris occurred in girls. Of 328 cases which I have examined in
reference to this point, 232 were females and 96 males.

Social condition has little or no effect on the production of chorea,

but it is more common in cities than in the country. Indeed,
everything which increases the excitability of the nervous system
during development adds to the tendency to the disease.

West and Hamilton point to over-study as a cause, and I have

frequently verified their observations. The annual examinations at
our public schools give a number of cases of chorea.

A. McLane Hamilton7 has recently investigated the frequency of St.

Vitus's dance among school-children in New York, and found that 20
per cent. of the children in the schools were affected with some
variety of the disorder.
7 American Psychological Journal, Feb., 1876.

Rheumatism is certainly a predisposing cause, in my experience, but

I have not found it associated with chorea as frequently as some
authors have. In 279 cases which I examined with regard to this
question, there was a clear history of rheumatism in but 37. Cardiac
complications were much more frequent. In 82 cases there was a
cardiac murmur heard. In some of these the murmur was no doubt
anæmic, but in the majority there had probably been a rheumatic
endocarditis. Many cases in which there is said by the friends of the
patient to have been a previous attack of acute rheumatism, on
investigation are found not to have had articular rheumatism, but
merely some muscular or joint pains which were not inflammatory.
Quite recently Joffroy and Saric have expressed the opinion that the
pains in the joints during an attack of chorea are to be regarded as
choreal arthropathies of nervous origin.

English and French writers have observed the relation between

rheumatism and chorea in a large proportion of cases. Hughes and
Burton Brown8 found that in 104 cases which they examined as to
rheumatism and affections of the heart, there were but 15 in which
some rheumatic condition had not preceded the attack or a cardiac
murmur did not exist.
8 Guy's Hospital Reports, 1856.

Sée and Roget consider the causal relation between the two
diseases so close as to make their connection almost a pathological
law.

On the other hand, Steiner made quite opposite observations in

Prague. He saw among 252 cases of chorea only four which
originated during the course of acute articular rheumatism.9 This
statement does not prove the absence of a relation between chorea
and rheumatism, for Steiner does not say what proportion of cases
occurred after an attack of rheumatism which had taken place some
time previous. Hammond10 believes “that the influence of
rheumatism upon chorea is not greater than that of a depressing
agent to the organism.”
9 Ziemssen's Cyclopædia, loc. cit., p. 427.

10 Dis. of the Nervous System, p. 715.

Octavius Sturges, physician to the Hospital for Sick Children,

Ormond St., London, says that in two years 219 cases of chorea
have been treated at that institution: 20 per cent. of them were
connected with rheumatism, but he does not believe in the rheumatic
origin of the disease, because 15 per cent. of all children have
rheumatism.11
11 Lancet, Sept. 20, 1884.

The following cases illustrate the direct sequence of chorea upon

acute rheumatism:

Case II.—Kate S——, æt. 17 years, came under my care in May,

1877, with an attack of acute rheumatism which lasted for two
weeks. She regained strength slowly, and about June 11th, not more
than two weeks after the subsidence of the rheumatic symptoms,
choreic movements were noticed in the right arm. The movements
were constant, and were worse when she was conscious of being
observed. She often dropped things. The right leg jerked often and
gave away under her in walking. Under the use of liq. arsenici
bromidi the patient was entirely well in one month. A sister had had
chorea.

Case III.—Rudolph C——, æt. 5 years, was placed under my care

May 12, 1883. He has had scarlatina, and in Dec., 1882, he had an
attack of inflammatory rheumatism, accompanied with slight choreic
movements, from which he recovered in a short time. Three or four
weeks before coming under my charge he had a return of
rheumatism from getting his feet wet, and at the same time choreic
trouble began again. He rapidly became worse, and in a short time
was utterly helpless. On examination he is found unable to sit up or
make any voluntary movements. He is unable to move the legs on
account of the intense pain in the knees and ankles from the
rheumatism. These joints are swollen and red. The right arm is in
constant and violent motion, swinging about or thrashing up and
down. The left arm lies motionless, and, although painless, he
seems unable to move it. The fingers are clenched, and they are
continually and rhythmically being pressed against the palm. He
cries if an attempt is made to open the fingers. The facial muscles
are contorted all the time, and there is a peculiar vermicular
movement of the upper lip. He cannot speak a word nor can he
protrude the tongue. He sleeps but little, and during sleep the
movements are occasionally seen. He is very fretful and irritable, but
is perfectly intelligent. The appetite is poor. Bowels regular; urine
phosphatic and contains no albumen. There is a loud but not harsh
cardiac murmur heard at the apex, replacing the first sound of the
heart. Temperature, May 12, evening, 100°; May 13, morning,
1002/5°. Pulse, 140. He was ordered sodii salicylat., gr. v. q. q. h., and
inunctions of cod-liver oil.

On May 18 the rheumatism was so much better that the salicylate

was stopped and Fowler's solution of arsenic was given instead. The
chorea had also become better. The arsenic was continued in
increasing doses, and the case convalesced steadily. On June 26 he
was able to return to his home in Maryland entirely well.

He remained well until July, 1884, when he had a slight attack of

chorea, which was preceded for about a week by rheumatism. The
cardiac murmur, which was almost absent after recovery from the
first attack, was now heard again, but faintly.

Scarlatina is sometimes the forerunner of an attack of chorea. Other

diseases, whooping cough or measles, may also be the immediate
cause of an attack of chorea, but it is generally in cases where there
is a predisposition to the disease.

The connection between pregnancy and chorea is of great interest.

Barnes12 has collected 58 cases, and Bodo-Wenzel13 has added 8
more, making 66 in all. Of 51 of these patients, 31 were primiparæ,
and in the majority of cases the ages were between twenty and
twenty-three years. Four cases which came under my own personal
observation were all young primiparæ, and were between the
second and fourth months of gestation when the disorder began.
The affection usually appears in the first half of pregnancy. It may
cease before the eighth month or may continue to the end of
gestation. Sometimes it persists after delivery. It does not
necessarily occur in patients who have had the disease in childhood,
but from the above cases it would seem that an attack in one
pregnancy would tend to its recurrence in another. Of the 66 cases,
14 had had chorea in previous pregnancies. Sometimes the chorea
appears only at the time of labor.
12 Obstet. Transactions, vol. x. p. 147.

13 Ziemssen's Cyclopædia, loc. cit., p. 428.

The immediate cause of chorea in pregnancy, when a cause can be

found, is most often fright or rheumatism. In two of my own cases the
patients were unmarried. Of the 66 cases of Barnes and Wenzel,
fright is named as a cause in 7, and in 7 more rheumatism and
endocarditis are stated to have preceded the attack.

Climate has been supposed to have a marked influence upon

chorea. It was thought to be less frequent in warm than in cold
regions; but Hirsch denies this, and Weir Mitchell14 states that it does
not appear to exist to any less extent in the southern than in the
northern portion of this country.
14 Loc. cit.

Season, according to Mitchell,15 largely affects the production of

chorea. He has gone to great pains to collect statistics on the
subject. He shows that the majority of attacks in his cases occurred
in spring. This agrees with Wicke,16 who found that among 35
relapses, 13 were in spring, 12 in winter, 9 in autumn, and 1 in
winter. On the contrary, Hammond17 found 54 attacks in the six
months from October to March, and but 28 in the remainder of the
year. Gerhard18 in a study of 80 cases found that of 68 attacks, 39
occurred in spring, 10 in summer, 7 in autumn, and 12 in winter. In
Mitchell's cases a study was made of 170 attacks. In the spring and
summer months there were 115 attacks against 55 in the winter and
autumn months. This observer does not find that rain or dampness
has any notable effect in causing the disease, but that the influence
of storms is probably great.
15 Loc. cit., p. 128.
 16 Ziemssen, loc. cit., p. 443.

17 American Journ. Med. Sciences, July, 1876.

18 Loc. cit., p. 715.

Mitchell has written upon the relation between race and chorea. He
states that in answer to a circular bearing on this question, sent out
by the Smithsonian Institution, he has received a large number of
letters from physicians in our Southern States and the West Indies.
The general testimony was that chorea is rare among negroes.

Among the exciting causes of chorea are fright or mental

apprehension of some kind. Of my 279 cases, 44 were ascribed to
fright.

Malaria has been pointed out as influencing the production and

course of chorea. Kinnecut has reported some cases in which the
movements were aggravated with a certain periodicity.

Chorea may also be brought on by reflex irritation from nerve-injury.

In a case which I saw in the practice of John H. Packard several
years ago there was an injury to one of the digital nerves of the
thumb from a splinter, which was the apparent cause of an attack of
chorea; a portion of the nerve was excised, and the chorea ceased
in a short time.

I have lately seen a case in which an attack of chorea came on

apparently in consequence of a severe mash of the finger. The finger
had been crushed by the runner of a sled, and the choreic
movements began before the wound was healed.

Hamilton19 has found chorea associated with eczema. He saw a

case in which eczema of the calves of the legs and of the scalp
developed at the same time with an attack of chorea. Both got well
about the same time.
19 Nervous Diseases, p. 490.