Athetosis
62 Art. V.? :Author: A. C. HUNRO, M.B. Southern Counties Asylum, Dumfries.
It was in 1871 that Dr Hammond, of New York, first drew attention to an affection of the nervous system having analogies with chorea and with cerebro-spinal sclerosis, of which he had seen one case in his own practice, and another had been minutely reported to him by Dr Hubbard of Ashtabula, Ohio. This affection he called Athetosis (from aflrroj?without fixed position), and having regard too exclusively to its particular manifestations in his own case?for Dr Hubbard’s exhibited a different phase of the disease?he considered it to he mainly characterised by ” an inability to retain the fingers and toes in any position in which they may be placed, and by their continual motion.” Since he first drew attention to the matter, a number of cases of Athetosis have been placed on record, but I do not think the clinical history of the disease is yet so clearly made out as to render the report of such a case as the following a work of supererogation.
J D , aetat 23, was admitted to the Southern Counties Asylum, Dumfries, on the 2nd May 1876, and reported to have been “insane from birth.” A maternal history of intemperance is to be noted. His condition is congenital: lias altered little since admission, and is as follows:? His general health is fair, muscularity and fatness average, there is no heart murmur, and no trace of chorea in the family history. His memory is good, intelligence fair, attention acute; the pupils are normal as regards size and sensibility, sight and hearing are normal, but the gustatory sense is under par. Sensibility as to heat and pain is normal, as is also reflex sensibility, but tactile sensibility is slightly dulled. There is no malformation cf the palate. The tongue is large and thick, and bound down to the floor of the mouth to within a quarter of an inch of the edge all round by thick fleshy adhesions which render it almost incapable of lateral motion, and limit antero- posterior motion to nearly the same extent. Articulation is almost entirely unintelligible except to those accustomed to his mode of speech. It is impossible to determine how much this is due to the tongue adhesion, how much it has in common with the conditions to be presently detailed.
There is a general affection of motivity, hut this is much less marked in the lower extremities than in the head and arms ; in fact it will soon he seen that it is most clearly exhibited the more delicate the movement attempted : that is, the more necessary it is to have combined action of the muscles, the disease being, as I believe, one of incoordination. At will the patient can by clenching his fists maintain an attitude of entire repose, with the exception that he is unable generally to control the action of the occipito-frontalis and corrugator supercilii muscles (the continued contraction of which gives his counten- ance an expression of chronic surprise), and that there are faint fibrillary contractions of the extensors of the arms. When, however, volition is slightly relaxed, and under the influence of emotions, there is a gliding “protrusion of the head forwards and upwards ” and to one side or another, mostly over the left shoulder; the muscles of the neck are perceptibly hypertrophied. When inhibitory force is not brought into action there is fioin time to time a flickering play of all the muscles of expression, and this developes into more violent action under the influence of any volition which would normally bring any of them into Play. So, in the effort at articulation, in which only the oral muscles are concerned normally, the eyebiows are twitched up, the eyelids wink violently, and the risoiius, zygomatic, buccinator, and the muscles of the lower part of the face are thrown into violent and irregular action : at the same time the thyroid cartilage is raised and depressed spasmodically. In laughing the muscles of the face twitch convulsively, and each independently of the other, and the head is thrown fiom side to side. The muscles of expression are hypertrophied. As further illustrating the incoordination of the face muscles, it is observed that while the patient can maintain the lowei part of the face in a state of almost entire quietude by the tonic apposition of the lips, when desired to keep his mouth open, t le circum-oral muscles twitch in all directions, and he cannot hold an object such as a pencil between his lips?it is at once dropped, from the inability to coordinate the labial muscles m such an action. So in eating, he cannot retain food within the mouth unless it is secured by the teeth. The coarser action of mastication, requiring less coordinating power, is performed tolerably well, but the jaws are closed with rather a snap.
In the arms there is in repose foint tonic contraction of the flexors not at all amounting to rigidity, with slight twitching of the extensors. The movements of the hands and arms are very characteristic. Almost entire immobility can be maintained so long as the patient keeps his hand and fingers in a state of flexion. When, however, this state of flexion is given np, as in initiating any movement, the fingers assume most grotesque and characteristic positions?the thumb is arched backwards into almost a complete semi-circle (for the joints have acquired an altogether abnormal mobility backwards), the index finger is frequently in a state of hyper-extension at the middle joint, while at the terminal it is flexed, the other fingers move about capriciously, the little finger being mostly widely abducted. All this time the hand is generally flexed at the wrist; and here again the over-balance of the flexors is shown, for while patient can maintain a tolerably uniform flexion of all the joints, he is unable to extend the wrist and fingers at the same time : if the fingers are fully extended, the wrist is partly flexed, and vice versa. The grasp is pretty nearly of normal strength, and patient can lift heavy weights with ease. Movements when car- ried out are not jerky but rather slow, rhythmic, and wavelike? corresponding to those of the head and face. This is well seen when patient is told to make a prehensile effort?as to lift a small object, such as a pencil, from the table. There is a moment’s pause, as if patient were concentrating his coordinating energies, and then a series of awkward, sinuous movements begin, the muscles of the trunk being engaged in rotating patient’s body so as to diminish the effort to be made by the muscles of the arm, and also, it would seem, to give a fixed point, or ‘point d’ appui, in the trunk. The movement ends by patient’s applying often not the tips of the fingers?the more delicate control required for their action putting a great strain on patient’s power of coordination?but some more proximal and lateral part to the object aimed at, and closing the fingers over it, joint by joint, in a manner somewhat suggestive of a camel kneeling. Coarser movements of the arm and forearm are carried out more easily. It is observed that the blow delivered by the extensile action of the arm is much feebler than that given by flexile, or combined flexile and supinative action. The more complex movements, such as fastening a shirt button, are impossible from inability to coordinate the action of the fingers sufficiently. And in executing any difficult movement the head and arms are thrown into varying and grotesque movement. The affection is similar in both arms, but patient from constant practice is able to use his right arm to more advantage than the left. The affection of the legs is much less marked, as was to be expected from the movements of the lower limbs being of a much coarser nature, and requiring much less power of coordina- tion in their execution. Here again the flexors seem to over- balance the extensors, the legs in walking are considerably flexed?the body is thus thrown hack, arid the action is a shuffling- trot; so that the patient looks as if he were always on the point of falling-, which, however, lie seldom or never does. When at rest, too, it is observed that the feet are slightly flexed and inverted. Entire immobility can be maintained in tbe lower extremities, with the exception of occasional faint twitch- mgs of the extensors of the toes. The toes, also, mostly retain their normal position, with the exception of the great toe of tbe left foot, which is kept in an analogous position (of hyper- extension) to the thumbs. All movements cease in sleep.
Dr Hammond’s first case was that of a man aged 33, of in- temperate habits, who had suffered from epilepsy for five years ; the disease first appeared on regaining consciousness after a prolonged attack of delirium tremens ; its onset being cha- racterised by numbness on the right side, with pain and rhyth- mical involuntary movements of the fingers and toes on that side?the movements soon passing almost entirely beyond the control of volition. The second was also an adult, of tubercular diathesis, and with a family history of intemperance: the attacks here, after several premonitory signs, succeeded an epileptiform seizure; the patient was aphasic, and had ” lost the normal antagonising force of the flexors and extensors” on the right side of the body, the flexors being weakest in the arm, strongest in the foot. In both cases there was impairment of the mental powers. Professor Gairdner ot Glasgow has since then put on record two cases of the disease in children, in one of which, at least, it was not congenital: in one there were almost incessant irregular involuntary movements in the left arm and leg; in the other, similar movements in the right arm and leg, most distinctly seen in the upper extremities, and in this ease it is noted that no individual muscle was entirely with- drawn from the will. Dr Shapter, who has apparently written in ignorance of Dr Hammond’s observations, records in the Medical Times and Gazette of June 7th, 1873, what is evidently a case of Athetosis, coming on in a girl of 12, and affecting the left arm and temporarily the left leg, as a case of ” Motor Incoordination (Locomotor Ataxy) without paralysis.” In the appendix to Vol. II- of his ” Maladies du Systeme Nerveux,” Professor Charcot furnishes notes of three rather doubtful cases. The only other cases yet recorded, so far as I know, are those of Dr Claye Shaw, who has given a most graphic delineation of the disease in Vol. IX. of ” St. Bar- tholomew’s Hospital Reports.” His cases are seven in number, all of them congenital; in one, the disease affected merely the head, in the others the arms and legs were affected in varying degrees.
The establishment of a new disease with a new name has been objected to by many, who think that the differentiation of Athetosis is an altogether unnecessary and unwarranted refine- ment. One gentleman at the last annual meeting of the British Medical Association is reported as having objected to its tabulation as a disease on the ground that it is ” but a symptom which may be due to very different lesions.” But, on the same grounds, the recognition of epilepsy and many other clinical entities as diseases must be discarded. And I further foil to see on what authority this decided statement rests, since, to the best of my knowledge, no post-mortem examination has yet been made in a case of Athetosis, and what the lesion or lesions may be is still a matter of conjecture.
The contention of those who regard Athetosis as an un- important modification of chorea, into which, in fact, they say it merges, is somewhat more worthy of notice. This objection has probably in part its origin in Dr Hammond’s rather faulty definition of the disease, which would apply equally well, or better, to chorea itself, and was based too exclusively on the one case he had himself examined. No case of Athetosis pass- ing into chorea, or vice versa, has yet been placed on record, and careful clinical observers of the disease easily recognise a group of symptoms 8ui generis. The movements in chorea are clonic and jerky?sudden, rapid, unexpected: in Athetosis they are slower?wavy, gliding, and with some method. The peculiar position of hyper-extension of the thumb and index finger, with sprawling abduction of the other digits, which the hand in the more marked cases constantly tends to assume, is pathognomic of the disease, and once seen, can never be mis- taken. The gliding protrusion of the head, when it occurs, is equally characteristic of the disease. I consider the case above reported a particularly representative one. It is well differen- tiated from chorea, in that all muscular action, with the slight exception of that of the forehead muscles, can be quieted at will, and almost entire immobility maintained. And it seems to form a bond of unity between two sub-classes, having on the one hand such cases as Dr Shapter’s in which there was ” no chorea,” but where, when the patient was ” told to pick up a small object, the fingers were seen to separate from one another instead of acting in concert, then futile and awkward attempts would be made to grasp it; ” and Dr Hubbard’s, in which there was ” incoordination effecting the right forearm and leg” but no involuntary movements. On the other side, there are the cases of Drs. Hammond, Claye Shaw, and Gairdner, where the most prominent symptoms were the incessant complex involun- tary movements. And it illustrates my position that the essential feature of the disease is incoordination (consisting largely in loss of balance betweeen the flexors and extensors, as is well seen in the above recorded case)?that in this unique group of cases is always present, while the ” incessant voluntary movements ” may be absent. In chorea there is a discharging lesion in Athetosis there is rather an interruption and diffusion of a ‘normal nerve current. The further differentiation of Athetosis is very clearly made out by Dr Claye Shaw: “In imbecility with ataxia (for so he designates the group of cases in which the affection is congenital), no history of chorea can be traced, even in other members of the family; there is no sign of heart disease. In chorea there is not the wavy, sliding movement resembling the peristaltic action of involuntary muscle, and the jerkings are more sudden, rapid and un- expected, so that it is quite impossible to say which muscle will he next affected. In ataxic imbecility one part of a muscle may be quite at rest, the other being in motion, but in chorea the whole of any muscle that is affected acts at once and sud- denly. The mode of speech is another element of diagnosis, for in choreic insanity the words are jerked out in a quick- decisive manner; in imbecility with ataxia they are drawled out in a manner due obviously to the slow and gradual manner in which the angles of the mouth are retracted. Chorea is rare , before the sixth year of life, Athetosis is most frequently con- genital. In chorea, the class books say, “the recovery of the patient, with very few exceptions, may be always prognosticated: in the disease which is the subject of this paper, recovery has not yet been known to take place, and therapeutics are mostly P?WCha?cot is one of the chicf opponents of the differentiation of Athetosis, which he regards as a mere variety of posthemiplegic Hemichorea. I object to this tabulation for, in the first place, Dr Hammond’s and Professor Gairdner s eases at least were not poet-heniiplegic; and in the second place, the case I have above recorded and some of Dr Claye Shaw s were not in any sense hemi-choreie, as the disease affected the body gene- rally, and not one side only. Again, while Professor Charcot’s first case may have been one of Athetosis, it will be seen that his conception of the disease is not that above evolved or generally adopted, for he speaks of the movements 111 his third case as ” mccades, brusque,” and says that when the patient tries to put her hand to her head she slaps herself; while in the second, the choreic movements are associated with anaesthesia. Then, in each of the three cases there was distinct loss of power, while it has been repeatedly noted that in true Athetosis there is no paralysis. Athetosis has as well, it will he seen, points of comparison with other diseases besides chorea, as cerebro-spinal sclerosis, paralysis agitans, writers’ cramp, and post-hemiplegic rigidity, though from these it is more easily differentiated. Finally, as to the pathology of Athetosis. Charcot considers that in the cases he records the lesion is unilateral cerebral atrophy. Such is very probably the pathology of these cases. But in Athetosis proper the seat of the lesion can in the mean- time merely be conjectured. From the analogies of Dr Ham- mond’s cases the disease may be supposed to have its origin in some disruptive lesion of the corpus striatum, of intra-uterine occurrence in the congenital cases, as the result of which an obstacle of some sort is interposed to the transit and coordina- tion of nerve force in that ” centre of automatic or sub-volun- tary integration of the various voluntary-motor centres dif- ferentiated in the hemispheres.” Dr Shaw, in addition, would anticipate a lesion of the cervical portion of the cord as well as of the nucleus of the seventh pair of nerves, but the sudden onset of the symptoms in immediate sequence to headache, vertigo, and loss of consciousness in Dr Hammond’s cases seems to point to a purely cerebral origin in the first instance, whatever the consecutive deterioration may be.
Disclaimer
The historical material in this project falls into one of three categories for clearances and permissions:
Material currently under copyright, made available with a Creative Commons license chosen by the publisher.
Material that is in the public domain
Material identified by the Welcome Trust as an Orphan Work, made available with a Creative Commons Attribution-NonCommercial 4.0 International License.
While we are in the process of adding metadata to the articles, please check the article at its original source for specific copyrights.