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.
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, 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.
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.
Condition on examination Aug. 14, 1884: General health good, well grown for age. The nutrition of the affected side is good, but the left side of the face is markedly smaller than the right, although there is no paralysis. The position of the arm attracts immediate attention. The arm, hand, and fingers are in extreme extension, as shown in the cut, which is made from a photograph. The arm-muscles are tense and rigid. The fingers are continually in motion, sometimes flexed, sometimes extremely extended; then in a few moments they will be widely separated or distorted in some other way. The favorite position, however, seems to be with the fingers extended until bent backward, separated from each other, and the thumb adducted slightly. The patient is unable to bring the arm down by a voluntary effort, but when asked to put it by his side he pulls it down with the right hand, and keeps it down by sitting upon the hand. The muscles of the arm are hypertrophied, especially those of the upper arm and shoulder. The circumference of the left arm around the biceps is almost an inch greater than that of the right. The leg is stiff and the foot is usually inverted. Here also the position changes at different times. When he walks the stiffness increases and the foot is dragged. The speech is clear and distinct, and the intellect perfect. He goes to school, and is fully equal to or more advanced than boys of his age. There is no evidence of cardiac disease.
FIG. 26.
Case of Athetosis.
The patient's condition in Oct., 1885, had changed somewhat. The arm is in a different attitude. It is still extended, but is held down by the side or away from the body. At times the forearm is strongly supinated. There are still constant but slowly-changing movements of the fingers. One plan which the patient has of keeping the arm flexed is to put the forearm behind the back with the right hand, and it becomes locked there by the action of the extensors. By a strong effort of will he can slowly and with great difficulty open and shut the fingers, and can flex the forearm. The leg is in the same condition as before.
The resemblance between athetosis and post-paralytic chorea is very close. Most of the cases of the former disorder which have been reported have been of the hemiplegic type. In Case I. the disease came on after an attack of delirium tremens, which was followed by six weeks of unconsciousness. The first symptom the patient was capable of noticing was numbness of the right arm and leg. The involuntary movements came on later. This would look as if there had been some lesion involving the left hemisphere of the brain. It is stated that no paralysis existed when the case was examined by Hammond, but there may have been a slight hemiplegia which had passed away. In another case reported by the same author the peculiar movements were preceded by hemiplegia and aphasia.
Oulmont has written a complete essay on athetosis, and has collected therein all the literature of the subject.3 He sustains the view of the close connection between athetosis and post-paralytic chorea, but he considers that, although nearly allied, a difference does exist between the two affections. He believes this is most marked in the bilateral form of athetosis, for here the disorder is usually not preceded by paralysis, and it is not accompanied by disturbances of sensation.
3 Étude clinique sur l'Athétose, Paris, 1878.
Gowers4 has also collected a number of cases, some of which came under his own observation, and has written a valuable treatise on the subject.
4 Medico-Chirurgical Transactions, 2d Series, vol. xli.
PATHOLOGY.—The symptoms point to a brain lesion, probably in the gray matter, and a perverted condition of the nerve-cells which leads to over-action, either spontaneously or under the influence of a motor impulse. In most of the cases reported by Claye Shaw there was imbecility. Charcot found in three post-mortem examinations lesions in each instance in the posterior portion of the optic thalamus, the most posterior part of the caudate nucleus, and the most posterior part of the corona radiata. Gowers has made an autopsy in one case in which there was post-hemiplegic inco-ordination affecting the arm only, without the spasmodic fixation of the limb. In the brain was found but one lesion, and that was a cicatricial induration of the optic thalamus, extending across its centre beneath its upper surface, and approaching at its outer part, but not involving, the ascending white fibres of the crus. No secondary degeneration was found in the cord.
PROGNOSIS.—This is almost always unfavorable. In some cases the inco-ordination decreases and the spasm becomes less violent, but it seldom disappears altogether. One of Gowers's cases was benefited by treatment, and two of those related by Hammond were relieved.
TREATMENT.—Many remedies have been given without marked benefit. In Gowers's case there is no doubt that the decided improvement which took place was due to galvanism. The treatment lasted three months, and a descending galvanic current was used. The positive pole was put upon the nucha, and the negative on the over-acting muscles and on the hand and foot. One of Hammond's cases, as related above, was apparently cured by stretching the median nerve. A prolonged course of galvanism and some alterative, like mercury or chloride of gold and sodium, would seem to offer the best prospect of benefit.