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.
Barnes[12] has collected 58 cases, and Bodo-Wenzel[13] 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 {444} had had chorea in previous
pregnancies. Sometimes the chorea appears only at the time of labor.
[Footnote 12: _Obstet. Transactions_, vol. x. p. 147.]
[Footnote 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 Mitchell[14] states that it does not
appear to exist to any less extent in the southern than in the
northern portion of this country.
[Footnote 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, Hammond[17] found 54 attacks in the six
months from October to March, and but 28 in the remainder of the year.
Gerhard[18] 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.
[Footnote 15: _Loc. cit._, p. 128.]
[Footnote 16: _Ziemssen, loc. cit._, p. 443.]
[Footnote 17: _American Journ. Med. Sciences_, July, 1876.]
[Footnote 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.
Hamilton[19] 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.
[Footnote 19: _Nervous Diseases_, p. 490.]
{445} SYMPTOMS.—An attack of chorea is usually preceded by more or
less failure of the general health and evidences of some mental
disturbance. It is quite common to be told by the parents of a child
suffering from chorea that the little patient had seemed unwell for
some time previous to the attack; that the appetite had failed, and
that the child had looked pale; that he had been irritable or
excitable, and at school the teacher had complained of restlessness or
inattention in the pupil. In a little girl who was brought to me
recently with her second attack of chorea her mother stated that for
several days before the outbreak the child had been in excessive
spirits, and that she had been singing loudly and in a peculiar
manner. The same symptoms had preceded the first attack. Sometimes
nothing is observed until it is found by the parents or teacher that
there are abnormal twitchings and movements of the limbs.
At first there is a general restlessness and fidgetiness. The child
may be punished at school for not keeping still or for dropping
things. Soon irregular movements of groups of muscles are seen. The
shoulder is shrugged or the fingers move spasmodically. At first the
patient is aware of the movements and tries to control them, but
before long the twitching and jerking are constant, and extend to most
of the voluntary muscles of the body. He is then unable to control
them for any length of time.
The sudden jerk of a limb followed by an odd grimace, the quick
protrusion of the tongue, and the rolling of the eyes or snapping of
the lids give a characteristic picture which can hardly be mistaken.
The extent of the movements varies in different cases. In some they
are slight and affect only certain muscles. Often the disorder is
confined to one lateral half of the body. In other cases the movements
involve all the limbs and the trunk, and are so violent and constant
that the patient does not seem to have a moment's rest. The trunk may
be suddenly drawn backward, then the arms are extended or thrown up,
and the legs flexed and tossed about with great quickness. Sometimes
the patient is thrown off the bed or from the chair on which he may be
to the floor.
The speech is often affected. The patient speaks in a thick or jerky
manner, as if the tongue were too large for the mouth, and saliva
usually flows in great quantities. Sometimes in bad cases there are
involuntary utterances made at frequent intervals.
The features undergo contortions continually, and when at rest relapse
into a condition of vacancy which makes the patient look almost
idiotic. The expression of a child with chorea is so peculiar that the
disease may almost be diagnosticated by this.
During sleep the movements usually cease, but generally the patient is
restless while asleep, and in some instances the irregular movements
continue even at this time.
The mental condition commonly shows some change. The child is
irritable and peevish, cries and laughs readily, or is sullen and
morose. Sometimes he is violent to those about him, but this is rare.
Intellectually the patient suffers somewhat. He is not able to study
as before, and the memory may be impaired. Sometimes there is a mild
form of dementia.
{446} During the course of the disease there may be exacerbations, and
sometimes after convalescence has seemed established there are
relapses.
Recovery is gradual, and as the abnormal movements cease the mental
condition improves, and the patient regains his health without any
traces of the disease remaining.
We will now consider some of the symptoms separately. First, as to
disturbances of motion. As before remarked, the disorderly movements
occur soon after the general restlessness is seen. They most commonly
begin in one upper extremity. The hand is thrown into various
positions, the fingers are flexed and extended or separated, and all
of the movements occur with great rapidity. In a day or two the whole
arm is affected, and then the leg of the same side is involved in the
jerkings and twitchings. In many cases the facial muscles are
contorted, the mouth is pursed up or opened wide, and then quickly
twisted into some other shape. If the patient is told to put out his
tongue, it is protruded after a moment's hesitation, and then suddenly
retracted, the jaws coming together with a snap. A smacking sound is
made with the lips quite often, and words are uttered involuntarily.
The movements may remain confined to one side of the body,
constituting what is called hemichorea. This is quite common, and the
right side is rather more frequently involved than the left. There is
so great difference of opinion among authors on this point that it is
probable that one side is affected about as often as the other. Of 252
cases which I have examined, 69 were right and 43 left hemichoreas.
Gerhard[20] found in 80 cases of chorea that 32 were unilateral; of
these 20 were right and 12 were left. Sée, however, found that in 97
of 154 cases the movements were either confined to the left side or
were more marked on that side. He states that in his experience the
proportion between left and right hemichorea is as 37 to 27. Pye-Smith
in 33 cases of unilateral chorea found 15 on the right and 18 on the
left side. Many cases which begin as hemichorea soon become general.
[Footnote 20: _American Journal of Med. Sci._]
The disease reaches its greatest severity in about two weeks, and if
the case is a bad one we find by this time all of the voluntary
muscles are in constant movement. At this time the French name for
chorea, folie musculaire, is most appropriate. Patients are often
unable to walk or to sit up, and sometimes they may be thrown from the
bed by violent spasmodic movements of the trunk. Strange as it seems,
patients rarely complain of fatigue, notwithstanding the violent
muscular exercise. This is probably because each set of movements is
of short duration and is constantly changing its seat.
As a rule, the movements cease completely during sleep or under an
anæsthetic. Sometimes occasional twitchings of muscles are seen in
sleep, and in rare instances we are told by the parents of a child
with chorea that the movements are as active in sleep as in the waking
hours.
The movements of chorea occur either while the limbs are at rest or
under the influence of voluntary effort. This fact has been pointed
out by Mitchell and by Gowers. In some cases the movements are most
marked when the patient is at rest. If a directed effort is made to
use the member for a time, the choreic movements are suspended. For
example, a patient may be able to carry a glass of water to the mouth
without {447} spilling a drop, while a moment before the hand may have
been performing a continual dance. I have often observed that while
the limb to which the whole attention has been directed in performing
some movement has been steadied, the other limbs become violently
agitated.
In another class of cases the movements are comparatively slight when
the part is at rest, but when a motion is attempted the disorder of
the muscles is so much increased that it is almost impossible for the
act to be completed. The patient is told to pick up some small object:
he throws the hand out toward it, and it is jerked away before he can
grasp it. He again puts the hand forward, reaches the object, and the
fingers open and shut and sprawl over the article before it is taken
up. Sometimes it cannot be grasped at all. This has been called
choreic ataxia, but it is only one type of the cases commonly seen.
This brings us to the influence of the will on the movements in
chorea. There are some cases, as mentioned above, in which the
movements may be controlled by the will for a brief period, but they
will sooner or later return. In other cases it is quite impossible for
the patient to check the movements at all, and one frequently sees in
a case of hemichorea the sound hand used to grasp the other, so as to
control the movements. We have referred to this because of Niemeyer's
opinion that corporeal punishment would shorten an attack of chorea.
Chorea is sometimes confined to a single muscle or group of muscles.
When limited in this way it is generally in the head, face, or perhaps
in the shoulder. These cases of localized chorea have been spoken of
by Mitchell as habit chorea.[21] They are often very obstinate in
resisting treatment, and sometimes last during life.
[Footnote 21: _Lectures on Nervous Diseases_, p. 146.]
PARALYSIS.—Not infrequently in chorea there is paralysis to a greater
or less extent. It is generally one-sided, and most often involves the
upper extremity. The limb affected is the one in which the movements
were most violent. The arm may hang entirely powerless or it may be
only enfeebled, and feeling to the patient like a dead weight. The
paralysis always recovers with the chorea or soon after.
POST-PARALYTIC CHOREA.—Under this term Mitchell and Charcot have
described a variety of chorea which is seen in patients after an
attack of hemiplegia. The movements are chiefly on voluntary effort,
and are those of inco-ordination. They come on from one to several
months after an attack of unilateral paralysis, and are sometimes seen
in cases in which almost complete recovery has taken place. Mitchell
has reported[22] a case which was under my care for several years, and
which he saw in consultation with me. This patient had two attacks of
left hemiplegia, the last being fatal. After the first attack there
was great gain of power to use the arm and leg, but the movements were
performed awkwardly and with an irregular jerking movement. A
post-mortem examination revealed a spot of softening the size of a
filbert in the left corpus striatum, which was apparently recent, and
a point of red degeneration in the right crus cerebri. The vessels at
the base of the brain were extensively atheromatous.
[Footnote 22: _American Journal of the Med. Sci._]
Of the electrical condition of the muscles in chorea but little is
known. Rosenthal[23] found increase of faradic contractility in three
cases of hemichorea, and the galvanic test showed a high degree of
excitability, {448} demonstrated by the fact that weak currents gave
contractions at cathodic closure, or even tetanic contractions, and
also contractions were produced at cathodic opening.
[Footnote 23: _Ziemssen's Cyclopædia, loc. cit._, p. 434.]
The affection of speech which is so common in chorea is due to
disordered action of the laryngeal muscles, or it may be from choreic
action of the abdominal muscles. Sometimes it is chiefly from the
awkwardness of the tongue. The usual form of trouble is that the
patient speaks in a staccato manner and the syllables seem as if they
were driven out. When the chorea is in the laryngeal muscles, the tone
and pitch of the voice are altered.
Chorea of the heart is sometimes spoken of, but it has never been
satisfactorily demonstrated that there is any real disorder of cardiac
rhythm in chorea. It is not unusual in chorea to meet with over-action
or palpitation of the heart, but these conditions do not necessarily
depend on the disease.
Valvular murmurs are often met with from the beginning of an attack.
In some instances they are the result of an endocarditis, but
frequently they are functional or anæmic. They are usually heard at
the apex. Sometimes there is a reduplication of the first sound,
giving the idea of a want of synchronism in action of the two sides of
the heart; but this is probably not the result of chorea of the heart.
I recall one patient, a child of seven or eight years, in whom the
reduplication of the first sound was very distinct during an attack of
St. Vitus's dance. She was brought to me at the beginning of a second
attack a year later, and the reduplication of the cardiac sounds was
heard again, so it is likely that it had continued during the
interval, and was probably a congenital condition.
The pupils are commonly dilated in chorea and respond sluggishly to
light.
REFLEXES.—I have examined the condition of the patellar reflex in 50
cases. In 26 of these it was present in normal degree, in 15 it was
diminished, and in 9 it could not be excited. In one patient it was
absent during the height of the choreic movements, but could be
readily produced after the patient had recovered. The condition of the
reflexes has also been examined by Joffroy and Saric,[24] and they
found that of 16 cases of chorea the reflexes were abolished or
diminished in 12.
[Footnote 24: _L'Union médicale_, Sept. 22, 1885.]
SENSIBILITY.—Authors state that disorders of sensation are met with in
chorea, such as localized anæsthesia or a general hyperæsthesia: I
have never met with any such instances. Patients often complain of
pain in the joints or in the limbs, and this may be unaccompanied with
swelling or tenderness on pressure. Tenderness on pressure over the
vertebræ is rare in my experience, although others speak of its being
of frequent occurrence. Mental disorders are generally present, but
only to a slight extent. There is almost always irritability of temper
and peevishness. The most sweet-tempered children become cross and
perverse, laugh immoderately at trifling things, or cry as readily if
they are annoyed. There is generally failure of memory and incapacity
for study or thought. In most cases, however, this exists to so slight
an extent as not to be noticed except on very close observation.
Sometimes there is marked mental disorder amounting almost to
imbecility, and occasionally the mental weakness remains for some time
after the motor disorders have recovered.
{449} The condition of the pulse is generally unchanged, but sometimes
it is abnormally frequent. The temperature, according to Von Ziemssen,
is unchanged.
The nutrition generally suffers. The patient rapidly loses flesh, and
becomes anæmic; the skin grows dry, and the hair gets harsh. The
digestion is apt to be disordered. The tongue is large, pallid, and
coated thickly, and there is sometimes nausea or vomiting. The
appetite is not good. The bowels are often constipated. The urine has
been examined by several observers. Bence Jones found an excess of
urea at the height of the disease. Albumen is not present except
accidentally, but there is usually an excess of phosphates. In several
cases in which we have examined the urine at the Infirmary for Nervous
Diseases we found that the specific gravity was high while the chorea
was at its height, but fell to normal as the patient recovered.
Chorea is spoken of as acute and chronic, but all cases are more or
less chronic. Those cases which last eight or ten weeks may be
considered acute, while those running on for months or years are
properly called chronic.
DURATION.—Considerable difference of opinion exists as to the duration
of chorea. Some writers speak of three or four weeks as an average
attack. Gray and Tuckwell, in a series of cases treated by the
expectant plan,[25] found an average duration of ten weeks.
Occasionally a patient is seen with an attack of chorea which lasts
only a few days. The parents of a little patient whom I saw a few days
ago assured me that her second attack lasted only a week. They are
educated and intelligent persons whose statement can be relied upon.
[Footnote 25: _Lancet_, Nov. 28, 1876.]
The course is not always regular. In some cases the disease gradually
reaches a crisis, remains stationary for a few days, and then by
degrees declines; in others there are exacerbations. The patient will
seem to be almost well, and then become very much worse for a time.
Relapses are not infrequent, and are generally caused by fright or
excitement.
The recurrence of attacks of chorea is well known. A child who has had
the disease one year may have it a second or third year. It is most
likely to recur in the spring. Some cases have as many as five
attacks, but as puberty approaches the attacks are lighter, and
finally cease. Of 282 cases to which I have referred, 198 were first
attacks, 47 had had chorea twice; 23 were in their third attack, 8 in
the fourth, and 3 in the fifth attack.
TERMINATION.—The disease in most instances terminates in complete
cure, but sometimes there is nervousness or want of co-ordination
remaining for a time. Rarely the inco-ordination or a certain
quickness in movement becomes permanent.
Death is a rare termination of chorea except in pregnancy. If it does
occur, it is usually from some complication. In pregnancy the
mortality is great. Of 64 cases collected by Wenzel, 18 died. In
Philadelphia, in seventy-four years from 1807 to 1881, there have been
but 64 deaths from chorea; of these, 38 were under twenty years, and
26 over that age.
Hutchinson reports a fatal case in a boy of twelve years.[26] After
complaining of headache and rheumatic pains for several days, choreic
{450} movements began. They soon became general and very violent. At
the end of two weeks he was admitted to the Pennsylvania Hospital. At
this time the patient was so extremely convulsed that it was
impossible to keep him in bed without tying him. The movements
continued but little abated, and the child died in two days.
[Footnote 26: _Philadelphia Med. Times_, vol. vi. p. 535.]
Another case of unusual interest is reported by Hunt.[27] The patient,
a man of twenty-nine years, had suffered from chorea of the face and
arms for years. In consequence of a fall on the pavement he fractured
the left humerus. The movements were immediately exaggerated, and in
spite of a carefully adjusted splint it was impossible to keep the arm
at rest. The fragments were in a state of constant movement, and the
points of bone threatened to penetrate the skin. The skin was so much
excoriated that it was determined to dispense with the splint and
attempt to keep the limb at rest by the administration of morphia
hypodermically in half-grain doses three times daily. This failed to
keep the arm quiet, and the seat of fracture became greatly inflamed.
No form of appliance or medication succeeded in keeping the arm at
rest, and the patient finally sank and died from exhaustion on the
tenth day after admission to the hospital. The post-mortem examination
revealed no gross lesion of the brain or cord. No microscopic
examination was made of the brain.
[Footnote 27: _Pennsylvania Hospital Reports_, vol. ii.]
MORBID ANATOMY AND PATHOLOGY.—In a disease so seldom fatal as chorea
it is not surprising that there have been but few post-mortem
examinations made. In the earlier autopsies, before the microscope was
extensively used, but little of value was recorded. Sée, who collected
84 cases in which post-mortem examinations were made, reported that in
16 no changes were found in the nervous system. In 32 there were
lesions in the brain and nervous centres, usually softening and
tuberculosis, and in the remainder inflammatory changes in the serous
membranes. In 29 there were evidences of heart disease. Sée considered
that but few cases of death in chorea were caused by inflammatory
diseases of the heart, but that the majority should be referred to
nervous excitement and anæmia.