seat slowly and with difficulty; hesitates a moment before starting to
{436} 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
{437} 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]
[Footnote 2: Quoted by Hamilton, _Diseases of the Nervous System_, p.
500.]
{438} 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]
[Footnote 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. Ross[4] 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.
[Footnote 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,
effort, or is not increased by | and ceases during repose.
it. |
|
Tremor regular and fine. | Coarse tremor, becoming more so
| during voluntary effort.
|
Face expressionless; tremor of | Facial muscles affected; nystagmus
face rare. | 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.
{439}
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]
[Footnote 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]
[Footnote 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 reports[3] 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.
[Footnote 3: _Lectures on Nervous Diseases_, p. 69.]
{440} 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.
[Footnote 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 {441} 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
age[5]—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.
[Footnote 5: _Journal of Nervous and Mental Diseases_, July, 1881.]
[Footnote 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 2 | At 7 years 23 | At 16 years 6
At 2 years 6 | 8 " 24 | 17 " 10
3 " 4 | 9 " 31 | 18 " 5
4 " 7 | 10 " 23 | 19 " 5
5 " 9 | 11 " 18 | 20 " 1
6 " 24 | 12 " 25 | 21 " 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.
{442} A. McLane Hamilton[7] 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.
[Footnote 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 Brown[8] 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.
[Footnote 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. Hammond[10]
believes “that the influence of rheumatism upon chorea is not greater
than that of a depressing agent to the organism.”
[Footnote 9: _Ziemssen's Cyclopædia, loc. cit._, p. 427.]
[Footnote 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]
[Footnote 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.
{443} 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, 100-2/5°. Pulse,
140. He was ordered sodii salicylat., gr. v. q. q. h., and inunctions
of cod-liver oil.