fatal. Post-mortem examinations were made in all of these. Cardiac
lesions were found in 13. In 10 of these deposits were found upon the
valves, and in 3 there was some change in the pericardium. He speaks
of having noted congestion of the nervous centres six times, and
softening of the cord once.
[Footnote 28: _Brit. and For. Med.-Chir. Review_, Jan., 1868.]
In all of 11 autopsies reported by Pye-Smith[29] there were cardiac
lesions found. In every case old or recent deposits were observed upon
the valves. In two instances the heart was hypertrophied, and in one
there was pericarditis. Changes in the nervous system were less often
found by this writer. In 1 case there was hyperæmia of the cord, and
in 3 cerebral hyperæmia.
[Footnote 29: _Guy's Hospital Reports_, 1874.]
Dickinson found in 22 fatal cases of chorea 17 in which the heart was
diseased. “In every instance making up the large tale of cardiac
disease there were recent vegetations on the mitral valve, and often
also elsewhere.”
In the fatal case of Hutchinson referred to above the heart was found
diseased, the aortic valves were incompetent, the leaflets being
swollen {451} and softened, and the aorta was atheromatous above the
sinus of Valsalva.
Of late years a number of careful autopsies have been made in cases of
chorea. The brain and spinal cord have been closely examined, and in
almost every instance some lesion has been found in both of these
organs.
Steiner reported in 1868 the results of post-mortem examinations in 3
fatal cases of chorea. In 1 case he found cerebro-spinal anæmia,
serous effusion into the spinal canal, and proliferation of the
connective tissue in the upper part of the cord; and in another
hyperæmia of the brain and cord.
Elischer,[30] who reports a fatal case in a parturient woman who had
an attack of chorea in her eighth year, two in her sixteenth year, and
another in a previous pregnancy, found at the autopsy hyperæmia and
œdema of the brain and gray substance of the cord. Microscopically,
the brain showed fatty, amyloid, and pigmentary changes in the
nerve-elements and vessels of the large central ganglia, small
secondary extravasations of blood in the connective tissue, and
numerous emboli in the smallest vessels, especially in the cortex. In
the spinal cord there was seen abundant proliferation of nuclei in the
adventitia of the vessels. In the central canal serum was found, and
the surrounding connective tissue was harder than usual.
[Footnote 30: _Cyclopædia of the Practice of Medicine_, Von Ziemssen,
vol. xiv. p. 450.]
Dickinson has contributed an excellent paper on the pathology of
chorea.[31] He relates the particulars of the autopsies in 7 fatal
cases in which he personally made microscopical examination of the
brain and spinal cord. He also adds the results of post-mortem
examinations in 17 other cases at St. George's Hospital and at the
Hospital for Sick Children. In all of the 7 cases in which microscopic
examinations of the brain and cord were made there were found
hyperæmia of both of these structures, in many instances hemorrhages
into the substance of the nervous tissues, dilatation of the smaller
vessels, and in chronic cases sclerotic changes in the course of the
vessels. “The first visible change,” he remarks, “would seem to be the
injection or distension of the arteries, succeeded by extrusion of
their contents, to the irritation and injury of the surrounding
tissue.” The changes seemed to affect both brain and cord in all
cases. The parts of the brain most constantly affected lay between the
base and the floor of the lateral ventricles in the track of the
middle cerebral arteries, the substantia perforata, the corpora
striata, and the beginning of the Sylvian fissures. “Of the cord no
region was exempt, but perhaps the cervical and dorsal regions were
usually more affected than the lumbar. With regard to the vertical or
physiological divisions of the cord, these all, whether white or gray,
shared in the vascular destruction; this condition, however, was
usually most marked in the vessels belonging to or in connection with
the lateral part of the gray matter about the root of each posterior
horn. And it is to be observed that this was also the chosen situation
of the more definite and special changes, whether hemorrhagic (as in
two instances), sclerose, or exudatory. Speaking generally, the chosen
seats of the choreic changes are the parts of the brain which lie
between the beginning of the middle {452} cerebral arteries and the
corpora striata—the parta perforata; and in the cord the central
portion of each lateral mass of gray matter comprising the root of
each posterior horn.”
[Footnote 31: _Medico-Chirurgical Transactions_, vol. xli. p. 1,
1876.]
The embolic theory of chorea has been held by several investigators,
among them Hughlings-Jackson. It is undoubtedly an attractive and
reasonable view, especially when we consider the large proportion of
cases in which there is valvular disease of the heart. Dickinson,
however, does not consider this hypothesis tenable. In none of the
cases in which he made post-mortem examinations did he find evidences
of embolism. “In none of the instances described were decolorized
fibrin, detached clots, or signs of impaction detected, and the
erraticism of embolic accident was wanting: the constancy indeed with
which the changes repeated themselves in certain positions, and the
equality with which they affected both sides of the body, are
conclusive objections to this hypothesis. The corpora striata, for
example, were affected with almost absolute symmetry, notwithstanding
that these bodies receive their blood respectively from the right and
left carotids and different parts of the aortic arch.”
Rheumatism is associated with or precedes chorea in a large proportion
of cases, and this was pointed out by Kirkes in 1850 and again in
1863. This connection between rheumatism and chorea, and the frequent
occurrence of endocarditis in chorea, has led some authors to believe
that the endocarditis is always rheumatic, and that the chorea is the
result of the endocarditis. Dickinson, however, points out that in
cases in which there is a distinct history of the chorea beginning
suddenly from fright there are often well-marked cardiac murmurs
heard. He believes that in all cases of chorea in which there are
cardiac murmurs they are due to endocarditis, and suggests that in
these cases from fright the endocarditis is due to irregularity of
cardiac action. This, of course, is mere hypothesis, and we must bear
in mind that in all cases of chorea there is anæmia, and that the
murmur may be purely functional.
H. C. Wood, in a communication read before the College of Physicians
of Philadelphia,[32] gives his views of the pathology of chorea, based
upon the results of post-mortem examinations made in a number of dogs
who had the disease. He believes the history of chorea to be this:
“Owing to emotional disturbance, sometimes stopping of various vessels
of the brain, or sometimes the presence of organic disease, there is
an altered condition of the ganglionic cells throughout the
nerve-centres. If the cause is removed and the altered condition of
the nerve-cells goes only so far, it remains what we call a functional
disease. If it goes so far that the cells show alteration, we have an
organic disease of the nervous system.”
[Footnote 32: _Philada. Med. News_, May 30, 1885.]
In two dogs which were choreic the movements continued after section
of the cord. This shows that in dogs, at any rate, the movements
originate in the cord. In four instances of canine chorea in which
Wool made autopsies there were found in the cords of three mild grades
of infiltration of leucocytes in the gray matter. In the fourth, in
which the dog had died of the disease, the ganglion-cells were
degenerated, and in some places had disappeared. He concludes,
therefore, that choreic movements may depend upon a diseased condition
of the motor cells of the cord.
{453} Although there are several recorded cases of human chorea in
which lesions of the spinal ganglionic cells have been found, we
cannot believe that this can be a constant lesion in chorea. The
disease is too transient in many cases, and presents too many
variations and anomalies, for the cord to be always the seat of the
diseased condition.
In an interesting paper read by Angel Money before the London Medical
and Chirurgical Society in 1885 he detailed some experiments in which,
by injecting a fluid containing arrowroot, starch-granules, or carmine
into the carotids of animals, he produced movements closely resembling
chorea; and this was found to be associated always with embolism of
the capillaries of the cord. In the discussion which followed
Broadbent and Sturges expressed their disbelief in the embolic origin
of chorea in man. Hughlings-Jackson said that he held the view of the
cerebral origin of chorea, one of his reasons being the frequency with
which the face-muscles are affected in this disease.
The probabilities are that in chorea there is a disordered condition
of the brain and cord more or less general. The lesions are no doubt
slight in mild cases of short duration, but in severe cases of long
standing there occur well-marked changes in portions of both brain and
cord. We cannot do better than to sum up the pathology of chorea in
the words of Dickinson: “A widely-distributed hyperæmia of the nervous
centres, not due to any mechanical mischance, but produced by causes
mainly of two kinds—one a morbid, probably a humoral, influence which
may affect the nervous centres as it affects other organs and tissues;
the other, irritation in some mode, usually mental, but sometimes what
is called reflex, which especially belongs to and disturbs the nervous
system, and affects persons differently according to the inherent
mobility of their nature.”
DIAGNOSIS.—The only diseases for which chorea may be mistaken are
paralysis agitans and disseminated sclerosis. The former occurs only
in adult life, and the tremor is of a regular rhythmical character. In
the latter the tremor occurs only on voluntary effort, and is also
more regular than the movements of chorea. There are forms of
congenital sclerosis seen in children which closely resemble chorea.
Here the duration of the disease and the association of contractures
with it distinguish it from chorea.
Hysterical subjects have a form of chorea which can only be
differentiated from the true disorder by noting the general hysterical
character of the case and the result of treatment, which strongly
influences the will-power of the patient.
PROGNOSIS.—In the great majority of cases this is favorable. If the
disease occurs in childhood and is without complications, recovery
generally takes place spontaneously after a few weeks. Should the
movements be violent and continuous, so as to interfere with sleep and
the taking of food, or should there be any complication, such as acute
rheumatism or cardiac disorder, then the prospect of recovery is not
so good.
The prognosis as to relapses should be given with caution. If in a
child, it is possible that there will be a return of the disease after
a longer or shorter interval. It is not likely to recur until after
several months, usually at about the same season the following year.
As the child grows older the intervals become longer, and it may
safely be {454} asserted that after puberty is passed and bodily
development completed there will be no more returns of the affection.
The cure is usually complete. It must be remembered, however, that for
some time after an apparently complete cure there may be slight
inco-ordination of movements, particularly in the arms and the face.
These are shown in the unnecessary haste in making uncertain motions
or in slight grimaces, or if excited an awkwardness in the use of the
fingers.
Death is a rare termination in uncomplicated cases, especially in
children. The fatal cases are generally when acute rheumatism has been
associated with the chorea or when there has been a fracture or an
injury as a complication.
In Sée's statistics there is a mortality of 5.7 per cent. in 158 cases
in the Children's Hospital. In adults, and more particularly in
pregnant women, death is more common. Wenzel's cases referred to above
gave a mortality of 27.3 per cent.
The cause of death in chorea may be from the intensity of the disease,
and in this case the symptoms are generally violent from the outset,
increase to an extreme extent, and then collapse and coma come on. The
movements may cease when the collapse occurs, but they may continue to
the last, growing gradually less until death.
TREATMENT.—A vast number of remedies have been popular in this disease
from all ages. The medicine which is most generally depended upon at
the present day is arsenic. It is advised by most writers, and in my
own experience is decidedly the most reliable remedy for chorea which
we know. The best way to administer it is in the form of Fowler's
solution, and it should be given in large doses. I have given the
bromide of arsenic, but did not find it superior to Fowler's solution.
The amount of arsenic which can be safely borne by children with
chorea is surprising to those who have not had experience in its
administration. The medicine should be given in gradually increasing
doses until the toxic effects are well marked or until the patient is
convalescing. In a child of six years three drops may be given to
begin with, three times a day. One drop additional should be added to
the dose each day, and the child soon acquires a remarkable tolerance
of the drug. As much as twelve or fifteen drops at a dose is borne by
a child of eight years. If vomiting or much œdema of the face occurs,
the medicine should be stopped for a day or two, and then the original
dose should be taken, to be again increased as before.
Seguin recommends that the patient should begin again with the dose at
which tolerance ceased. For instance, if vomiting occurred after a
dose of nine drops, he stops the medicine for a day, and begins again
with eight drops. I have found that sometimes this causes vomiting
again, and I think it preferable to resume the medicine with a small
dose.
It is often seen that a patient becomes worse during the first few
days that the arsenic is taken, but improvement generally begins after
a week of the arsenical treatment, and is well marked after two weeks.
In obstinate cases it is of marked advantage to give the arsenic
hypodermically. Cases which do not yield to the drug when given by the
mouth often improve at once when it is given hypodermically. Chronic
cases which have resisted all forms of medication sometimes are cured
by {455} hypodermic injections of arsenic. For giving arsenic in this
way it is best to use Fowler's solution, made without the compound
spirit of lavender. It is less likely to cause abscess to form at the
point of puncture.
Other remedies enjoy a reputation in the treatment of chorea. Sulphate
of zinc is relied upon by many, and it is the means which Ross
recommends. It should be given in increasing doses like arsenic, and
very large doses may often be taken without disturbing the stomach.
Trousseau, Hammond, and Hamilton favor strychnia, but I have had no
experience in its use.
Cimicifuga and conium are both often beneficial in their effects. I
have seen the former do good when arsenic had failed. Conium to be
efficacious must be given in large doses. Eserine and hyoscyamine have
both been successfully employed, the former by Bouchut, and the latter
by Oulmont and Laurent. Recently, DaCosta[33] has reported, in a
clinical lecture at the Pennsylvania Hospital, a case of very severe
chorea successfully treated with hyoscyamine. The patient was a boy of
eleven years, and the disorder had followed an attack of acute
rheumatism. He was given 1/100 gr. of hyoscyamine three times a day.
[Footnote 33: _Philada. Med. Times_, Jan. 23, 1886.]
Ziegler[34] has recorded several cases which recovered under the use
of nitrite of amyl. The bromides and chloral are useful adjuncts to
treatment in case of sleeplessness or mental irritability. Cases of
cure by the use of chloral alone have been reported. Bouchut gave a
girl of fourteen and a half years, with chorea and dementia, 45 grains
of chloral a day for twenty-seven days. She slept most of the time,
but improvement was seen on the fifth day, and cure was completed on
the twenty-eighth day of the use of the chloral. Electricity has been
efficient in the hands of many writers. I have found galvanization of
the spine to produce a quieting effect in some cases.
[Footnote 34: _Ibid._, vol. vi. p. 486.]
Iron is always of use in chorea; it may be given during the course of
the disease, and is generally necessary in convalescence. Cod-liver
oil or malt extract should be given in feeble persons.
It is scarcely necessary to mention the other remedies which have been
recommended. DaCosta has used the bromide of iron. H. C. Wood has used
a preparation of skunk cabbage, and there are a great number of other
remedies which have been found of value.
Next to the internal means come external applications. Baths and
frictions are useful in their effect on the general health. The ether
spray to the spine or the application of an ice-bag for ten minutes
once or twice daily is sometimes found to assist the other means. Cold
douches have been advised by some, but they may do harm. The care of
the general health of the patient is of first importance, and his
surroundings should be as quiet as possible.
It is of the greatest value in bad cases to place the patient in bed
and keep him there until the symptoms improve. John Van Bibber of
Baltimore has treated a number of cases of chorea successfully by
keeping them secluded in a darkened room. Such an extreme degree of
isolation is not often necessary, and it might make a child more
nervous.
In children the patient should always be taken from school and kept
from exciting play. Plenty of fresh air and wholesome food should be
{456} insisted upon. Change of air to the mountains or to the seashore
often effects a cure in a short time.
Some cases do not appear to be benefited by any treatment. These are
the hereditary form of chorea and some of the localized choreas. The
latter are often helped or cured by the hypodermic use of arsenic even
in long-standing cases.
{457}
ATHETOSIS.
BY WHARTON SINKLER, M.D.
This disease was first described by Hammond in his work on _Diseases
of the Nervous System_ in 1871, and cases have since been reported by
many observers, among them Clifford Allbutt, Claye Shaw, Eulenburg,
Oulmont, and Gowers. The disease is named by Hammond from the word
_ἀθετος_, without fixed position.[1] The principal features are an
inability to retain the fingers and toes in any position in which they
may be placed, and the continual movements which persist in the
parts—a condition called by Gowers mobile spasm.
[Footnote 1: _Diseases of the Nervous System_, p. 722.]
Athetosis is often connected with impaired mental powers; many of
Shaw's cases were in imbecile children.
The movements of athetosis are not confined to the hand in all cases,
but they are sometimes met with in the foot, and even in the muscles
of the face and back.
The following is Hammond's original case:[2] “J. P. R——, aged
thirty-three, a native of Holland, consulted Hammond Sept. 13, 1869.
His occupation was bookbinding, and he had the reputation, previous to
his present illness, of being a first-class workman. He was of
intemperate habits. In 1860 he had an epileptic paroxysm, and since
that time, to the date of his first visit to me, had a fit about once
in six weeks. In 1865 he had an attack of delirium tremens, and for
six weeks thereafter was unconscious, being more or less delirious
during the whole period. Soon after recovering his intelligence he
noticed a slight sensation of numbness in the whole of the right upper
extremity and in the toes of the same side. At the same time severe
pain appeared in these parts, and complex involuntary movements ensued
in the fingers and toes of the same side.
“At first the movements of the fingers were to some extent under the
control of his will, especially when this was strongly exerted and
assisted by his eyesight, and he could, by placing his hand behind
him, restrain them to a still greater degree. He soon, however, found
that his labor was very much impeded, and he had gradually been
reduced from time to time to work requiring less care than the
finishing, at which he had been very expert.
“The right forearm, from the continual action of the muscles, was much
larger than the other, and the muscles were hard and developed like
those of a gymnast. When told to close his hand he held it out at
arm's length, clasped the wrist with the other hand, and then,
exerting all his power, {458} succeeded, after at least half a minute,
in flexing the fingers, but instantaneously they opened again and
resumed their movements.
“In this patient there was impairment of intellect, his memory was
enfeebled, and his ideas were dull. There was no paralysis of any part
of the body, but there was slight tremor of both upper extremities.
The involuntary movements were of the right arm, and continued during
sleep. Sensation was normal. The spasm of the muscles causes severe
pain in the arm, and keeps him from sleeping at night.” Hammond used
various remedies without relief, and had the patient under his charge
for many years. Finally, he showed the patient to the American
Neurological Society at the annual meeting in 1883, with almost
complete relief to the movements as a result of nerve-stretching.
[Footnote 2: _Ibid._]
Athetosis is found in two forms—the hemiplegic and the bilateral
varieties. In the former there has usually been an attack of
hemiplegia more or less marked, or there has been an epileptic fit or
unconsciousness from alcohol, as in Case I. There is often
hemianæsthesia or some disorder of sensation. In the bilateral type
the movements exist in all of the limbs, and are unaccompanied by
weakness or disorder of sensation. The degree of movement varies in
different cases. In some it is very slight, and can be controlled by
extreme effort on the part of the patient. In other cases the
movements are violent and uncontrollable. The muscles of the affected
limbs become hypertrophied from the constant exercise.
The following case of athetosis has come under my care:
Case II.—W. A——, aged thirteen years, male. He had good health until
1877, when at the age of six years he had diphtheria. The attack was
not severe, and he was up most of the time. About ten days after
apparent convalescence he was suddenly seized with left hemiplegia.
The paralysis was complete, involving the left arm and leg, the left
side of the face, and the muscles of deglutition. There was also
aphonia. In two weeks he began to talk, but indistinctly. In a month
he could move the arm, but the movements were inco-ordinate. At about
the same time he began to walk, but dragged the leg. The arm never
regained power of voluntary motion, but instead there came on a
spasmodic condition of the muscles which fixed the arm in various
positions, and at the same time there were kept up constant but
irregular movements of the hand and fingers. No spasmodic action of
the leg-muscles occurred until a year later. He has never walked well
since the attack of hemiplegia. The positions which the arm has
assumed have varied at different times, but it usually retains one
attitude for several months at a time. Sometimes the arm has been held
in extension; at another time it has been flexed; indeed, the
positions have been numerous. His general health improved and the
speech became perfect.