At first the left side of the face was flaccid and deviated in the other direction, but at the time of examination it presented no unusual feature beyond a continual twitching, a real convulsive tic, of the upper lip.
Now, whatever a facial convulsion of apoplectic origin, secondary to facial palsy and accompanied with spastic hemiplegia and athetosis, may be, it is at all events no tic.
Take one more case, given by Buss[45] as "convulsive tic of the left side of the face."
The patient was an atheromatous subject, with cardiac hypertrophy, bronchitis, and emphysema. When he first came under observation at the hospital, his eyelids, cheek, and buccal commissure were the seat of painless clonic contractions, which a month later were complicated by giddiness, vomiting, inability to stand or walk, lancinating pain over the right side of the face, weakness of the right limbs, and left facial paresis, and had become fugitive and insignificant. Loss of consciousness was followed by flaccidity of all four extremities, hyperpyrexia, and death. The section showed a hæmorrhage of the dimensions of a pigeon's egg which had destroyed the left half of the pons, and an atheromatous dilatation of the left posterior cerebellar artery, impinging at one spot on the seventh and eighth nerves of the same side. Microscopical examination of their trunks and of the facial area in the pons disclosed no abnormality.
The pathological anatomy of this case indicates its nature unmistakably, and its symptomatology and evolution, moreover, do not bear the remotest resemblance to those of tic.
In the opinion of Debrou,[46] convulsive tic is a functional derangement of a motor nerve, analogous to the neuralgia of a sensory one. To strengthen his argument he relied on such cases as those of Romberg, Schultz, Rosenthal, Oppolzer, where disease of neighbouring structures (enlarged glands, otitis media, caries of the temporal bone, etc.) was the agent in the production of muscular twitches in the domain of the facial. In our view, however, they are simply spasms provoked by irritation on the centrifugal path of a reflex bulbar arc.
The slight contractions occasionally seen both on the paralysed and on the non-paralysed side in the secondary contracture stage of facial palsy—a condition noted by Duchenne of Boulogne, Hitzig, and others, and distinct from fibrillary twitching—are nothing more than spasms, and the same obtains where the palsy is consecutive to affections of the ear.
Chipault and le Fur recently[47] communicated to the Academy of Medicine a case of intermittent attacks of acute pain in the right hypochondriac region, associated with violent contractions of the muscles of the right abdominal wall, which they described as a tic comparable to tic douloureux of the face. It was seen at the subsequent operation that the eighth, ninth, and tenth posterior spinal roots on the right side were surrounded in their passage through the meninges by a patch of matted and cicatricial arachnoiditis, dissection of which was instrumental in effecting immediate relief.
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 Patella[48] 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 patients[49]:
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 instance[50] 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 phenomena[51] 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 speak[53] 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's[54] 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.
To the combination of various rhythmical acts with hysteria we shall revert at a later stage. Under the title "rhythmic spasm" an interesting case has been reported at length by de Buck,[55] concerning a young woman, free of hysterical stigmata, in whom convulsive movements first appeared at the age of seven years.
When she had attained her nineteenth year she commenced to suffer from attacks of anguish of some hours' duration, but disappearing under the influence of sleep, in which she felt as though her breathing were going to stop and she herself were about to die. On the termination of these sensations some eighteen months later, their place was taken by convulsive movements of the tongue, lips, neck, trunk, left arm, diaphragm, pharynx, and muscles of respiration. These consisted of clonic rhythmical twitches, each preceded by an inspiration and succeeded by an expiratory ejaculation, repeated fifty or sixty times a minute. During the seizure the tongue was protruded and deviated to the left, the left arm was raised, the head and trunk bent down and forward. All day long the movements were continued with unflagging regularity. Rest in bed was without effect, but they were dispelled by sleep. Distraction and occupation exercised an inhibitory influence on them, whereas voluntary control was both feeble and fleeting. In the condition of the patient there was nothing else abnormal with the exception of slow, monotonous, and syllabic speech. Her mental development was perhaps a little immature, but signs of hysteria were lacking, and all attempts at treatment by suggestion and hypnotism failed of their object. Death ensued from pulmonary tuberculosis.
De Buck observes that while the action of some of the muscular groups involved in the rhythmic spasm was, so to speak, purposive, the whole did not constitute any known, conscious, and logical movement. It may have been a species of tic, but the rhythmical sequence of the convulsions imparts to it a quite peculiar character.
ATTACKS
A further mark of the motor reaction is the circumstance that it ceases for a longer or shorter interval, independently of the tic's localisation, intensity, or form, the result being an alternating series of "attacks" and periods of respite. In different patients, and in the same patient, the number and the length of these attacks are as variable as are the spaces of rest that separate them. We remember a girl with a tic consisting in a toss of the head repeated perhaps fifteen times a minute, three or four occurring together at intervals of one or two seconds, and being succeeded by a relatively long pause. The effect of treatment was to modify the sequence entirely, and to reduce the tic to an isolated jerk reappearing not oftener than once in a quarter of an hour, and in itself constituting the attack. In another case the patient's head used to turn to the left, remain so for a moment, then resume its ordinary place. After a time of repose the tic began again, and even when the movements followed each other more rapidly, the intervening period was always appreciable. On the other hand, we have seen a youth afflicted with multiple tics which continued without intermission the whole day long; the attack lasted, strictly speaking, from morning to night, and any break in its continuity was altogether exceptional. It might then be more exact, perhaps, to use the epithet paroxysmal in reference to the external manifestations of tics, but it signifies little what word we employ provided we are familiar with the clinical facts.
The attacks vary with circumstances and environment. One of our patients remained quite free from them during a visit to the theatre. Tissié had a young patient who did not tic at all while on holiday, but the reopening of his classes was the signal for a fresh outbreak. Similarly, no rule whatever seems to govern the duration of the times of relief; they may never be longer than a few seconds, or they may run into months. In the face of these data we cannot supply further generalisations; it will be sufficient if we impress on ourselves the importance of one fundamental element in the constitution of tic—viz. its repetition.
LOCALISATION OF THE MOTOR REACTION—VARIABLE TICS—FIXED TICS
The localisation of the motor reaction in cases of tic is essentially physiological. In rare instances its sphere may be limited to a single muscle, if one muscle only be requisitioned for the performance of a functional act; but it is very much more usual to find several muscles contributing, whose synergic contractions fashion the movement of which the tic is a caricature. If the same effect is yielded by the action of either of two different muscles or groups of muscles, as in rotation of the head, and if one be hindered from fulfilling its function, the incidence of a tic originally located in it will promptly be transferred to the other. This is the explanation of the persistence of rotatory tics after exclusion of the sternomastoids by surgical means.
Two symmetrical muscles may be affected, as in tics of blinking and of affirmation, or a median muscle, such as the orbicularis oris. Much more frequently the tic is unilateral in its distribution, as, for instance, when it involves the face; in this respect its figuration as a functional disturbance is well exemplified, for expressional movements of the face are normally bilateral. A tic may settle itself on two mutually antagonistic muscles, and manifest its presence in the immobilisation of a limb or segment of a limb; or only a portion of a muscle may contract, as in the case of the deltoid or trapezius, which are composed of bundles anatomically associated but physiologically independent, and so capable of being functionally differentiated by voluntary education. Fibrillary contraction and tic have nothing in common.
Inasmuch as the muscles concerned are under voluntary control, and their contractions such as the will can effect, it follows that with adequate practice the movement of a tic can always be imitated, and in predisposed soil imitation tics may thus take root; it is not always feasible, on the other hand, to counterfeit a spasm.
Several functional muscular territories may be simultaneously affected, and several tics may follow one another in quick succession, the duration of any one tic on any one site being a more or less varying quantity.
We have already noted the occurrence of variable tics. They appear one day to disappear a few days later, and reappear again after another space. Weeks or months may elapse without any vestige of them, until they suddenly break forth again unheralded. As a general though not absolute rule, the younger the patient, the less stable his tics. Occasionally they are isolated, limited, and stationary, one of the most frequent of this kind being a tic of blinking, but the intimate alliance between the motor troubles and the mental level of the subject helps to explain why these tics of children are so changeable.
In the case of young J., for instance, it was shortly after attaining his tenth year and entering school that first he began to tic, and thenceforward, at unequal intervals, trunk, arms, shoulders, legs, became in turn the seat of "movements of the nerves," while other more definite tics were not slow in developing.
When only six years old B. exhibited a respiratory tic, which changed a year later to one of the tongue, and after another year to one of the leg; at the age of twelve he used to nod his head in affirmation, and this was eventually succeeded by movements of negation, etc. He has since started a salaam tic, and finally a torticollis with deviation of the eyes.
We may cite an analogous case from Grasset:
A young girl, who had had eye and mouth tics in childhood, commenced at the age of fifteen to advance her right leg involuntarily—a sort of tic which lasted several months and gave place to paralysis of the same limb; for this affection was next substituted a whistling tic, and then for a whole year she used from time to time to give vent to a loud "Ah!" When she came under observation she was suffering from a tic of salutation, with retrocollic jerking of the head and shrugging of the right shoulder.
One of our own patients furnished us with the following story:
The disease made its debut by a blinking tic of both eyes, whose origin in the absence of any visual defect remained undetermined; grimacing and distortion of the mouth were the next to appear, as well as wrinkling of the nose and forehead, twitching of the eyebrows and contraction of one platysma, sometimes even of the ear muscles and the entire scalp. Then ensued up-and-down tossing of the head, or rotation of it from right to left, and, later, elevation and advancement of the shoulders, with restless agitation of hands and arms. A former trick of his of biting his nails is quite in abeyance at present; instead, he catches hold of his under lip every moment and abrades its mucous membrane with his nails, so much so that the lip is swollen and cracked like those of children with nibbling tics. Some months ago he acquired the habit of giving utterance to a soft little cry not unlike the sound made by a guinea-pig.
One tic has succeeded another in an unbroken series. The facial tics were more of the nature of grimaces, which the child amused itself in repeating; no doubt the scratching of the lip was a sequel to the desire of experiencing a new sensation, while the movements of hands, arms, and shoulders were very variable and different enough from the accompanying phenomena. No one of the tics was at all of protracted duration; on the contrary, each was fugitive and changeable, and therein presented a resemblance to the child's mental status. In sleep they completely disappeared; in the presence of strangers or if his interest was in any way aroused, they quieted down, while they increased on holidays, during games, or with physical fatigue.
It is clear that determination of the tic's localisation and mode can come only with the mental evolution of the patient, and that the transformation from the psychical inconsistency of childhood to the stability of the adult is paralleled by the change in the tic's manifestations as the scale of age is ascended. Any individual, whatever his years, who is in the stage of mental infantilism, will tic after the manner of a child, for the characters of a tic are dependent on the state of mind of its subject.
CHAPTER VIII
ACCESSORY SYMPTOMS
REFLEXES
THE question whether in cases of tic there is any alteration in superficial or deep reflexes can be decisively answered only by an appeal to statistics, the information afforded by which has hitherto been too scanty and too incomplete. Judging from our own observations in about thirty cases, we feel compelled to admit that disorders of this kind are altogether exceptional. Careful and repeated examination has convinced us that in patients suffering from tic the knee, ankle, wrist, elbow, and other jerks, the plantar and fascia lata reflexes, as well as those of the pharynx, eyes, etc., are all but universally normal, and any trifling exaggeration or diminution not only varies from day to day, but also in no wise exceeds the differences met with in health, and is therefore symptomatologically negligible. In the manifold varieties of tic represented by R., S., P., N., M., B., etc., whose cases are quoted here in part, our inquiries have always been negative. Noir's research on the state of the reflexes in idiocy complicated with tics failed to expose any abnormality, and even where the knee jerks were increased no departure from the usual manifestations of the tic was discoverable. It is of course permissible to suppose that a combination of the latter with organic disease of the nervous system might explain the modification of the reflexes. In this connection it may be remembered that on one occasion we found the customary diminution of O.'s knee jerks had passed into actual loss, and although on the next day they were present again, the occurrence was suspicious enough to justify one in entertaining the idea of incipient tabes. Even if the existence of other signs had corroborated this diagnosis, the incontestable genuineness of O.'s tics would have remained, so that the attempt to correlate the derangement of the reflexes with the existence of tics is somewhat questionable.
We have enjoyed the co-operation of M. Babinski in the examination of one of our patients, L., in whom we were able to determine a definite and symmetrical exaggeration of the patellar reflexes, a slight increase in the right triceps jerk, and, in making the subject rise from a prone to a sitting position with the arms folded, a very minor degree of flexion of the thigh on the trunk.
The first of these symptoms is of no pathognomonic value, and while the others no doubt are characteristic of organic disease, their development in this instance is too imperfect to warrant the deduction of pyramidal involvement. For the last ten years L.'s motor control has been very defective. The muscular activity of the right half of his body takes the form of irregular contractions, badly timed and inaccurate in range; in spite of the absence of pain, the timidity with which they are executed hinders their ever attaining a normal amplitude; and at the same time his inability to appreciate the direction and intensity of the motor reaction, the outcome of excessive muscular vigilance, illustrates a certain loss of the sense of position of his limbs.
The existence of an actual irritative lesion is therefore problematical, and it is scarcely conceivable that organic mischief of ten years' duration could have produced these clinical symptoms without creating graver disturbance of the reflexes, or effecting changes of a trophic nature in muscular and other tissues.
From the pathogenic and diagnostic point of view, the detection of conspicuous and persistent alterations in the reflexes is of deep significance. It is an important factor in the differentiation between tic and spasm.
Sometimes the task is unusually arduous, as when the unilateral distribution of the motor troubles recalls the clinical picture of lesions of the pyramidal paths. In L., for instance, the hemichorea and the torticollis were on the right side, and in a case published by Desterac a similar condition obtained, the writers' cramp, hip spasm, and head rotation being all confined to the right. Notwithstanding the fact that this patient had exaggerated knee jerks, ankle clonus, and a double extensor response, an opportunity for examination given to one of us made it clear that the untimely movements and bizarre attitudes were similar to what has been noted in certain cases of tic.
At the Neurological Society of Paris a case was shown by Babinski[56] of left spasmodic torticollis, with marked spasms of the left arm and left leg, and a homolateral extensor response, and it was contended that if one and the same cause underlay these phenomena—nor did there appear any adequate reason to doubt it—and if the reversal of the plantar reflex was, conformably to received opinion, to be interpreted as indicating a derangement in the function of the pyramidal system, then it was allowable to attribute the muscular spasms to the same derangement, in which circumstances the natural conclusion was that spasmodic torticollis itself might sometimes at least be dependent on pyramidal irritation of an as yet undetermined kind.
More recently still, another patient was exhibited by the same observer,[57] in whom the association of head rotation and convulsions of the arm on the left, with increase of the triceps reflex, was conceivably the outcome of pathological stimulation of the pyramidal tract. Yet the symptoms in each of these cases were curiously analogous to what we find in mental torticollis, in which abnormalities of the reflexes are conspicuous by their absence. We ought not on that account to reject the hypothesis of concurrent organic disease, inasmuch as a structural modification may be no longer the cause but the consequence of inordinate repetition of a motor reaction. Muscular hypertrophy or atrophy may be the sequel to tics born of ideas that find motor expression, and circulatory and even cellular changes may ensue on gesticulatory excess. The objective signs that reveal the existence of a point of irritation, on the presence of which the diagnosis of spasm depends, are commonly so trivial as to be wellnigh valueless, and should they be awanting, the motor disturbance appears to be purely functional, and may be considered a tic. At the same time we must admit the possibility of mixed forms, where the functional element is linked with primary or secondary organic disease, and perhaps their occurrence is more general than is ordinarily imagined. We repeat, however, that rigorous and lengthy investigation alike of the psychical and the somatic phases of the condition, embracing the state of the reflexes, will usually furnish sufficient information to facilitate the question of diagnosis and justify a positive statement.
ELECTRICAL REACTIONS
The examination of the electrical reactions of the muscles concerned in a tic is a clinical method seldom, if ever, resorted to, and we can scarcely expect it to yield decisive results from the symptomatological aspect. As with the reflexes, it may happen that we cannot afford to neglect its diagnostic significance in certain cases. For example, we have had occasion to test its worth in studying the case of young J., whose trouble consisted in a clonic tic of elevation of the left shoulder, and a tonic attitude tic of the left arm whereby it was firmly applied against the body. No important alteration in electrical contractility was discovered, although the response in the upper part of the left trapezius—which, by the way, was more voluminous than on the right—was brisker than in its fellow. On the other hand, the right deltoid, sternomastoid, and pectoral, were more excitable than on the left.
Here, of course, the evidence supplied by electrical examination only served to confirm the knowledge gathered from other clinical sources.
VASOMOTOR AND SECRETORY AFFECTIONS
Disorders of the vasomotor system rarely fail to assert themselves in the subjects of tic, but they do not in any wise differ from such as are met with in the majority of "nervous" individuals. The average sufferer from tic is emotional, and apt to betray his emotion by blushing for the most childish reason. This symptom may be in itself trifling enough, yet it may afford the earliest indication of mental instability the nature and extent of which subsequent research will determine. It is even conceivable that fear of blushing—the ereutophobia of Regis—may be at the bottom of certain gestures intended to conceal the heightened colour the apparition of which is so humiliating. The form they assume is generally a movement of the arm or hand over the face, to mask the momentary discomfort, and while in most instances they are no more than stereotyped acts, they may develop into full-blown tics.
In regard to secretory affections, we have frequently observed the concurrence of hyperidrosis and emotional phenomena in those who tic. Young J., S., P., are cases in point. The slightest exertion, the least effort of attention, are followed by an extraordinary secretion of sweat, entailing constant carrying of a handkerchief in the hand, and ceaseless mopping of the forehead or temples. This performance becomes stereotyped, and is gone through even when there is no perspiration at all. Suppression of the handkerchief sometimes causes actual malaise, but this injunction must never be forgotten if a cure is to be effected.
[Persons afflicted with tic often develop a sort of visceral instability which betrays itself in indigestion, dyspepsia, constipation, diarrhœa, and in every variety of dietetic and alimentary caprice.
It is rare to meet with troubles of micturition, nocturnal enuresis scarcely deserving mention owing to its frequency among all young degenerates and to its being so commonly the outcome of neglect. Oppenheim,[58] however, considers diurnal enuresis worth including in the symptomatology, and Brissaud[59] has described polyuria and pollakiuria in association with obsessional preoccupation. These are really functional disturbances in which increased desire is followed by increased vesical action, and may be regarded, if one likes, as micturition or sphincter tics.[60]]
AFFECTIONS OF SENSATION
Generally speaking, objective disturbances of sensibility do not occur, and while subjective changes are more frequent, they may be entirely lacking even in long-standing and aggravated cases. What the patients usually complain of is a more or less persistent, disagreeable, uncomfortable sensation, rarely described as painful, and often compared with a feeling of stiffness or fatigue. Or, again, they may be conscious of a sense of constriction or of dragging in the affected muscles, either at their insertions or in the muscle belly, or sometimes in the joints concerned. These subjective sensations are characterised by extreme variability in time and in degree. Moreover, the accounts given by patients of their own sufferings ought to be accepted with reserve. Not merely are they ready to exaggerate and incapable of accurately depicting and localising their sensations, but they also exhibit a curious tendency to false interpretation: they attribute an erroneous pathological significance to their feelings, and proceed to elaborate a thousand ridiculous variations, thereby inviting in a sense the eruption of fresh tics. In all this behaviour their mental imperfections are abundantly manifest.
We may remind ourselves in this connection how O.'s various inventions had no other effect than that of provoking new tics, and another illustration is to hand in the case of S., an account of whose mental torticollis will be found in a previous chapter.