Tics and Their Treatment
One could not desire a more typical example of reflex spasm, the area of irritation in this case being situated at a point on the centripetal arc close to the medullary centre.
We may be allowed to quote a last case from Cruchet:
A little phthisical girl, four and a half years old, began to complain of headache, and in the course of the next day became delirious. Three days later the delirium gave place to generalised convulsive seizures affecting chiefly the arms, and more pronounced on the left side. Simultaneously a tic of the right side of the face was observed, distinguished by raising of the upper lip and closure of the palpebral aperture. Sleep brought no modification in its train. Up to this stage a very feeble degree of contracture of the jaw muscles had been noted, but this speedily became accentuated to such an extent that nasal feeding had to be adopted. Some hours previous to the child's death the tic disappeared, only occasional slight convulsive twitches of the right arm remaining. Consciousness was maintained to the last minute.
At the autopsy the characteristic appearances of tuberculous meningitis were found: the base of the brain at the anterior perforated spot and origin of the sylvian artery was covered with gelatinous purulent patches, the colour of prune juice, which extended backwards to the pons; one in particular had enveloped the basilar trunk and sent out a prolongation on the right side, which surrounded the sixth, seventh, and eighth nerves at their point of emergence.
For our part, we cannot apply the word tic to the convulsive phenomena of tuberculous meningitis. If localised spasms occurring in the course of a grave illness, associated with fever, headache, and delirium, with contractures and generalised convulsions, and if the spasmodic manifestations of rapidly fatal pyrexias, are all to be denominated tics, then the term has no longer any significance, and it would be wiser to give it at once its quietus.
We are well enough aware that Cruchet believes there is a "convulsive tic symptom"; in other words, certain symptoms in such and such a disease appear in the guise of convulsive tic, "a movement or combination of movements representing in a clonic fashion a physiological act." Nevertheless, we are not convinced that the convulsive movements of Cruchet's patients exhibit the sequence of "regulated physiological acts."
He further draws an analogy between the foregoing case and the partial convulsions of toxæmias, cerebral tumours, etc., "transient convulsions supervening in the course of acute or chronic affections, and readily recognisable." In exceptional circumstances they may "assume the form of convulsive tic." In strict truth the form may be the same, but examination of the patient will soon demonstrate that the two are alike merely in appearance, and compel the reconsideration of an immature diagnosis.
Our position is that tic is more than a symptom – it is a symptom-complex. Cruchet's definition of convulsive tic just quoted is by itself insufficient; the additional and indispensable factor is the characteristic mental defect, of which so illuminating an exposition was given by Charcot.
Finally, the knowledge derived from the pathological investigation of myoclonus and polyclonus does not of necessity throw light on the morbid anatomy of tic.
In the case of an epileptic who suffered from myoclonus in his last years, ischæmic degenerations were found by Rossi and Gonzales disseminated throughout the brain, especially in the rolandic area, but any inference to hold good for the tics would be premature.
The term polyclonus has been employed by Murri to designate a succession of clonic contractions of the limbs, due to the existence of punctiform hæmorrhages or areas of softening scattered throughout the rolandic cortex. The character of the motor reaction in these cases, however, bears no resemblance either to tic or to chorea, although the fact of the relation between diffuse cortical lesions and convulsive movements is calculated to enhance the difficulties of diagnosis.
Vincenzo Patella48 has recently called attention to a case of polyclonus in which the disappearance of the symptoms during sleep suggested their purely functional origin, but histological examination of the rolandic grey matter at a subsequent period revealed the presence of numerous foci of degeneration. We are as yet, however, far from grasping the real meaning of such symptoms, which, moreover, from the clinical standpoint, cannot always be assimilated to those of the tics. Conclusive anatomical information is therefore still being awaited.
The functional nature of the movements we have had under discussion is unfortunately an obstacle in the way of our early knowledge of their pathology. As long as we remain ignorant of the actual cause of the neuroses and psychoses, so long will the pathological anatomy of tic continue a sealed book. All that has been written on this topic hitherto really concerns spasm and other convulsive affections secondary to irritation of nerve centres or conductors. If we may venture to express an opinion, it is that we should not be surprised if post-mortem examination rest constantly negative. As a matter of fact, we do not favour the view that the phenomena depend on an acquired lesion; rather are we inclined to believe that they represent some congenital anomaly, some arrest or defect in the development of cortical association paths or subcortical anastomoses, minute teratological malformations that our medical knowledge is still unhappily powerless to appreciate.
CHAPTER VII
STUDY OF THE MOTOR REACTION
THE general characters of the motor reaction constituting the objective manifestation of tic form the subject of previous analysis in the chapter on pathological physiology. It is our present intention to approach them from the semiological point of view.
To give a description of the motor disturbance of universal applicability is evidently to attempt the impossible. The modifications of functional acts are legion, and in the case of tic anomalies of muscular contraction vary not merely with the individual, but in the individual. Each tics after his own fashion; and no two tics are ever exactly interchangeable. As Trousseau was wont to say, "the disease in a sense forms part of the constitution of the person affected."
THE TYPE OF MOTOR REACTION – CLONIC TIC AND TONIC TIC
The motor reaction may be either clonic or tonic in type. Clonic tics are distinguished by more or less abrupt contractions, separated by longer or shorter intervals of relaxation or repose. The duration of a clonic tic convulsion may be exceedingly brief, though perhaps not so brief as the instantaneous "electric" twitches of a spasm, which have the extreme rapidity of pure reflex phenomena. Exception ought to be made for the face, no doubt, seeing that the suddenness of the movements in facial tic is precisely what complicates the diagnosis between it and facial spasm, as we shall see. In the limbs, the variations appear to stand in close relation to the nature of the primary factor, the mental condition of the patient, and the mode of reaction peculiar to him. The quickness with which the reaction occurs increases in proportion to the length of time the tic has existed, although once it has become habitual, any further change is rather in the direction of additional complexity.
Sometimes a relative deliberateness of execution raises suspicions as to the accuracy of the diagnosis. In the case of a child with several tics, one affecting the mouth in particular, Guinon was struck by the slowness of the muscular contractions.
To begin with (he says), the mouth was opened gradually, but as soon at the limit of separation of the maxillæ was reached, it was immediately closed, without remaining even for a moment in the extended position, as one would have expected had there been a tonic contraction of the infrahyoid muscles.
Cases of this kind, however, are not really instances of the tonic variety.
One of us has had the opportunity of observing a young woman afflicted with a curious combination of motor disorders, akin no less to the clonic form of tic than to the gesticulations of chorea and the undulatory movements of athetosis. Their resemblance to the clinical type described by Brissaud under the name of variable chorea is noteworthy, a distinguishing feature, however, being the sluggishness of the muscular contractions, which may well be a reflex of the patient's mental inertness.
Mademoiselle R., a young woman twenty-six years old, is a small and delicate creature with slender limbs and tapering fingers. She is extremely myopic, but her general health is excellent, and there is nothing to suggest that she is suffering from organic disease of the nervous system. Apart from the fact that her parents are rather "nervous," the family history is negative.
Since the age of twelve she has been subject to various tics of the face and head. She wrinkles her forehead and moves her scalp to and fro, and sometimes she turns her head slowly and steadily towards the left side, raising her eyes up and to the left at the same moment. Head and eyes forthwith resume their normal position. The deliberateness of the act is altogether exceptional. If, however, she happens to be wearing her hat – which is remarkable for its size, weight, and unwieldiness – the gesture is repeated in a quick and jerky manner. Any diversion, such as reading, knitting, listening to a conversation, especially if she feels she is not being noticed, will augment the intensity of the movements, which the thought of being observed, or the awakening of her interest, or rest in bed, or sleep, has the effect of abbreviating or checking.
Our earliest step was to confiscate the offending hat, and this had the instantaneous result of diminishing the violence and frequency of the tic, which the subsequent practice of appropriate exercises entirely dispelled.
If now we direct our attention to the psychical aspect of the case, we are struck with the goodness, devotion, and disinterestedness of our patient. Her one concern is for the welfare of others, and she is indifferent to the pleasures of literature, art, games, or even work. All that is required of her she performs with docility, but never with animation. The extent of her passiveness is seen in her childlike acceptance of her parents' wishes. Her temperament is neither gay nor sad, but merely dull. Indolence and maladroitness predominate in all her actions, and reveal themselves in the curious awkwardness and nonchalance that characterise the execution of even the simplest movement. She is essentially of a very unstable nature, but its torpidity is no less obvious than its instability. If there is no abruptness in her acts, it is equally true that she is never still. She cannot maintain any given attitude; she cannot fix her gaze on any particular object. Her restlessness is such that her position is changed from moment to moment, however slowly and imperceptibly. Her eyes are only half opened; as she speaks, her lips are scarcely seen to move.
It has been a laborious and protracted task to teach her to sit motionless with her hands in front of her, and no less unremitting effort has been required to make her open her mouth properly, or turn her head naturally from side to side.
In some ways the endless movements of her hands and fingers – she never ceases playing with her dress or her gloves or her handkerchief – are vaguely reminiscent of those of athetosis, and on the left side especially, if they become a little brisker, there is slight hyperextension of the phalanges. She reads aloud in a low, colourless, monotonous tone of voice, without punctuation or accent, articulating the syllables defectively and slurring the ends of the words. At the finish of each paragraph comes a halt, as if from fatigue, and on command a fresh start is made with the same careless indifference. As for the lower extremities, the tale is identical. Mademoiselle R. cannot stand upright. She rests on either one leg or the other. Her left foot is never flat on the ground, but sometimes on the inner border, sometimes on the outer. The faulty attitude is readily enough corrected, though she declares she is ignorant of it. It is a sort of half clonic, half tonic, tic of the foot, whose slowness is on a par with that of all her other acts.
It is because clonic tics are so easily recognised that they are the most familiar, but we must not ignore another variety – viz. the tonic tics, corresponding to the tonic form of convulsion.
Tonic tic is of common occurrence in cases of mental torticollis. In that disease rotation of the head may be sustained for a considerable length of time without interruption, showing the permanent nature of the muscular contraction. Strictly speaking, we are concerned not with a sudden gesture, but with an attitude. Abundant evidence is forthcoming to substantiate its mental origin, and it may therefore be described as an attitude tic. Among other instances of tonic tics may be specified the affection of the masseters known as mental trismus (Raymond and Janet), or that continuous contraction of the orbicularis which keeps the eye half closed, though it may momentarily disappear under the influence of the will – a tonic blinking tic. O. and young J. have already supplied examples of attitude tics, and reference may further be made to another of our patients49:
Sometimes the mouth is drawn directly and completely to the left, more usually to the right; at other times simultaneous contraction of the upper and lower lips takes place, constituting a sufficiently faithful reproduction of the grimace made by a child in the attempt to refrain from crying; at other times still, imperfect closure of the lids and upward deviation of the eyes bear a resemblance to children's imitation of a blind man. Displacement of the mouth to the right is the movement of longest duration, and while it persists the patient is capable of stuttering speech, without relaxing her lips. The other tics last but a few seconds, while all vanish if she laughs or opens her mouth wide to exhibit her tongue. They follow each other at irregular intervals, and during the moments of rest the face resumes its normal expression.
Cruchet, as has been already remarked, has criticised the use of the term attitude tic, on the ground that the adoption of an attitude, however vicious it be, need not be the outcome of a convulsion. Doubtless; but it is no less true that a tonic convulsion may "take shape" —e. g. the arc de cercle of hysteria, the phenomena of catatonia and catalepsy, etc. Of course if the word tic is to be synonymous with intermittent twitching, then it is inapplicable in this class of case; but if our connotation of the term be accepted, we must find an expression that will serve to differentiate between tonic and clonic varieties. We are not aware of any particular advantage in describing the condition as a permanent contraction, for the obvious result of a permanent contraction, whether it be clenching of the jaws, occlusion of the eyelids, or rotation of the head, is the production of an attitude, a "position in which the body is kept" (Littré). No other designation could therefore be more appropriate than attitude tic, or could indeed be imagined, seeing that Cruchet himself ranges mental torticollis among the tics, and describes it as "an attitude of defence and of repose."
It may sometimes happen that the manifestations of stereotyped acts consist in the assumption of attitudes, but in spite of their affinity to the tics we deem it preferable to reserve the term "stereotyped attitude" or "akinetic stereotyped act" for cases where the motor reaction is clothed in the form of a normal movement. As it is inaccurate to describe as a tic a repeated gesture whose execution is normal in degree and in rapidity, so the mere immobility of a limb, or the prolonged contraction of a muscle, ought not to be called an attitude tic if the muscular effort does not differ from that which a healthy person would make to preserve the same position. In such circumstances we say that it is a stereotyped gesture or attitude. For the diagnosis of tic it is insufficient to establish the existence of a transient or permanent muscular contraction, and to note the inopportuneness of the movement; the contraction must be abnormal in itself, its abruptness unwonted and its intensity excessive – in short, it must be a convulsion; and finally, its repetition must be continued and exaggerated.
We have felt that some such explanation as the foregoing is required to justify our use of the term tonic or attitude tic, to whose close intimacy and association with the better-known type pathogeny and clinical observation alike bear witness. In any case such terms as myotonus or myoclonus are too comprehensive, in view of our present-day knowledge, to specify the particular motor affection with which we are concerned.
As a general rule it is only one part or segment of the body that is immobilised by a tonic tic, but in regard to the possibility of a general involvement, the following instance50 may be cited, although we do not think it can be considered decisive:
A man thirty-two years old, who had recovered from a first attack of mental torticollis, underwent a relapse in quite a different form. If when walking with his head perfectly straight he were asked to go round to the right, he instantly appeared to become rooted to the spot and could not turn even his head in the required direction; at the same time he felt a compression of his throat as if he were being strangled, and for a few seconds he experienced acute anguish. A moment later he was all right again, and his action unimpeded.
Without going so far as to classify this incident as a tic, and without venturing to assert the existence of a tic of immobility, one cannot but be struck with its analogy to the attitude tics of which we have been speaking, and to catatonic conditions met with in the insane, of which too the pathogeny presents more than one point of similarity with that of this species of tic.
[In this connection reference may be made to certain conditions occasionally noted among those who tic – viz. a curious tendency to maintain abnormal positions of the limbs or trunks, and difficulty in or impossibility of relaxing various muscles (catatonic aptitudes). Patients are sometimes given to the exaggerated repetition of the ordinary movements of their members (echokinesis), as well as to imitation of the actions of others (echomimia). Such catatonic and echopraxic phenomena51 are not confined to sufferers from tic, for they are encountered among psychopathic subjects generally, and indicate defect of cortical control – what is called by Brissaud "passive activity." These catatonic aptitudes may be discovered by resort to clinical tests, such as letting the arm fall from the horizontal position.52]
INTENSITY OF THE MOTOR REACTION
The muscular contraction varies considerably in intensity, in most cases exceeding that of the corresponding normal movement, and, especially in tonic tics, being often so powerful as to necessitate the exertion of great force to overcome it. Even though one's effort prove unavailing, however, it is only needful to distract the patient's attention to perform any and every passive movement with consummate ease.
In the case of S., any attempt to budge the head from its torticollic position on the left evokes strong muscular resistance; but engage him in conversation or otherwise divert his mind, and the difficulty soon vanishes. By similar means, the resistance awakened by sudden change of the direction of passive rotation will rapidly die down.
Occasionally the muscles brought into play surpass their fellows of the opposite side in size and power, this secondary hypertrophy being the natural sequel of repeated exercise. It was noted by Charcot that in rotatory tics the disused muscles atrophied, whereas the affected muscles hypertrophied, but they may do so only in appearance. The tonus of the muscles at the moment of examination may create differences inappreciable during relaxation. Sometimes one comes across such expressions as "paresis" or even "paralysis" of antagonistic muscles, and "contracture" of those in which the tic is localised. To draw a distinction between slight contracture of the latter and mild paresis of the former is a problem practically always insoluble. Opinion has been ever divided on this point; yet some, in their desire to harmonise the two, take up an eclectic position and do not hesitate to speak53 of "paralytic contracture," or "mixed contracture, at once active and passive," a terminology by no means calculated to simplify the question, and one the discussion of which we do not care to pursue.
We should like, however, to allude to a matter of clinical observation that we frequently have had occasion to remark. What simulates muscular enfeeblement in the subject of tic is often nothing else than a want of accuracy and adresse in the performance of a given movement. For instance:
S. enjoys robust health; his only trouble is a lack of accurate control over his limbs. His execution of the most elementary movements is incorrect. There is no tremor, no jerkiness, simply a loss of the sense of position. He never knows whether he is holding himself straight, whether his arms are exactly horizontal or his shoulders symmetrical. Often he confuses right and left, and when requested to perform some act on one side, he declares he is tempted to perform it simultaneously on both. The order to fold his arms and rotate the upper part of his body to the right evokes an inconceivable display of contortions. In the attempt to bend his head and body backward, fear of losing his balance causes him to twist and turn about most strangely, and the remark that all this he might avoid by merely putting one foot further back seems to cause him infinite surprise.
Or again:
The absence of precision in Mademoiselle R.'s movements, her habit of arresting the action before attaining the desired end, are not to be ascribed to any feeling of discomfort, but to her ignorance of the amplitude of her efforts, and of the position of her limbs. Her acts are always feeble, hesitating, and curtailed, a curious mixture of muscular languor and vigilance, "as if she were afraid of breaking herself." She appears to be constantly seeking some new position for herself, and to be as constantly oblivious of her actual attitude. With eyes closed, however, she indicates the relation of her limbs exactly.
Another example is furnished by the case of L., to which reference is made on p. 135.
There is no call to multiply instances. Enough has been said to demonstrate the frequent occurrence, if not of motor inco-ordination, at least of faulty orientation in space and of defective estimation in regard to the range and intensity of voluntary movements, among the subjects of tic. The topic is a very interesting and fruitful one, on which considerable light may be thrown by the application to it of the results of Pierre Bonnier's54 remarkable studies on the sense of attitudes, a subject that we intend to develop on another occasion.
FREQUENCY AND RHYTHM – RHYTHMIC TIC
The frequency of the muscular contractions in tic is so very variable that it cannot be regarded as a distinctive feature, nor is there any evidence to show that it is rhythmical, as some would have us believe. Contrary to what obtains in tremor, there is no periodicity in the motor phenomena, even when the tic is based on derangement of a function whose manifestations are rhythmical, such as the function of respiration. Conditions described as rhythmic tics, or less well as rhythmic spasms, seem to form a group by themselves; probably they do not belong to the same family as the tics, indeed in some cases they are symptomatic of encephalic lesions, as in the spasmus nutans of infants, or the rhythmic tics of idiots and imbeciles. In this connection the remarks of Noir are very pertinent:
We shall be well advised to refrain from drawing too absolute conclusions in questions so difficult, where even the framing of an hypothesis demands prolonged observation, but we cannot withstand the temptation to note the co-existence of certain of these tics with certain definite lesions recognisable post-mortem. This has been done before us by our master Bourneville, who has on several occasions made the diagnosis of chronic meningo-encephalitis, cerebral sclerosis, etc., from this association of rocking, rotation, and krouomanic movements with a special symptom-complex, and verified it at the autopsy. Nevertheless, there is not always an absolute correspondence between them, wherefore Bourneville, with an altogether praiseworthy scientific reserve, has hesitated to consider these tics as actual symptoms of the affections alluded to, and we shall follow his prudent example.