Kitabı oku: «Tics and Their Treatment», sayfa 11

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 Brissaud⁵⁹ 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.

Any trifling item of passing interest used to make S. forget altogether the more or less acute pain he experienced in his neck and shoulders, and reacted no less successfully on his torticollis. When systematic and methodical exercise of the muscles was ordered, nothing was more natural than that their long period of inactivity should have the result of causing a vague feeling of stiffness in them with the unwonted action. Yet S. never dreamed of such an ordinary explanation, but pessimistically exaggerated the sensation, and deemed it an infallible sign of the spread of the disease. It proved to be a simple enough matter, however, to convince him of its harmlessness, for it was sufficient to remind him of the corresponding stiffness he had felt after his first attempts at riding and fencing, and from that moment he ceased to pay any attention to it and therefore to complain.

With spasm, on the other hand, pain is more frequently, though not always, associated. It may be said, of course, that since a tic may be evolved from a spasm, the pain of the latter is really the exciting cause of the former, but in the tic as it is constituted all these initial disturbances have disappeared, and what the patient does feel is the consequence of excess of muscular action or of articular displacement. His dolorous sensations form the sequel, not the prelude; they are not symptoms, but, so to speak, complications.

CHAPTER IX
THE DIFFERENT TICS

THIS chapter we shall devote to a review, necessarily incomplete, of the principal sites in which tics are to be met with. We do not pretend to have collated every known case observed up to the present, and we foresee the likelihood, moreover, of new tics coming into being. Their numbers are as unlimited as is the diversity of functional acts of which they form the pathological expression. We must content ourselves, then, with the consideration of the most familiar and most recent examples.

A rational classification would entail discussion of the various modes of derangement to which functional acts are liable, and this would demand in its turn a preliminary tabulation of function. How onerous such a task is, is patent from the uniform imperfection of the attempts already made, and the equivocal nature of their conclusions.

We have studiously avoided the designation of a tic by the muscle or muscles that determine it. To specify the precise muscle involved is sometimes attended with no little difficulty, while if several, as is customary, are concerned, their association is rarely anatomical; indeed, this is one of the chief aids to diagnosis between tics and spasms. Should the convulsion chance to follow an anatomical distribution, neighbouring muscles are apt to participate as well. Hence it is advisable to name a tic after its morphological situation, or, better still, from the functional act of which it is, in Charcot's phrase, the caricature.

This is the plan we shall pursue in our successive examination of the different parts of the body disposed to be the seat of tics.

FACIAL TICS – TICS OF MIMICRY

Of all tics, those of the face are the most frequent, and the most easy to see. No other part is as rich in muscles whose functions are so diversified – nictitation, mastication, suction, respiration, articulation, etc. Moreover, the face is the abode of the mimic expressions, each one of which is the revelation, by muscular play, of some sentiment, or passion, or emotion. Hence the idea has been entertained of adopting a physiological classification. In the smiling tic of Bechterew, for an instance, the muscular contractions are framed into a smile in the absence of any provocative to mirth; in a similar fashion, the sniffing tic brings to mind the inhaling performances of snuff-takers.

Facial tic is frequently unilateral. It is rare to find the whole muscular distribution of one facial nerve involved, however, this being a property rather of spasm, as is also the restriction to a particular branch. A common event is the simultaneous abstention of some facial muscles and implication of others belonging to a different nerve supply.

If the condition is bilateral, as a general rule only those muscles on each side co-operate that are wont to act in concert for the accomplishment of some function. In a case reported as bilateral facial spasm by Claus and Sano,⁶¹ in which both sides of the face and neck were affected, the exaggeration of the convulsions by emotion, their curtailment daring rest and disappearance in sleep, coupled with the fact of their temporary arrest by recourse to subterfuge, suggest that the condition is really one of tic.

The contractions of the facial muscles are usually associated to produce a more or less complex grimace. Movements of forehead, eye, nose, or mouth, may succeed each other or be superimposed one on the other without any preconceived order, or the tic may consist in the synchronous activity of two or more muscles.

Of course any and every facial tic may occur by itself, but careful investigation will often reveal concomitant reactions of other muscular groups. The sniff that accompanies puckering of the nose indicates the engagement of the muscles of inspiration.

Facial tic, moreover, may be tonic as well as clonic, instances in point being closure of the eyelids, wrinkling of the forehead, twisting of the nose, distortion of the mouth, etc., of longer or shorter duration.

Any of the facial muscles may be attacked by tics. These commonly furnish an illustration of functional disturbance of mimicry, as in Oppenheim's cases of tic limited to the frontales, whereby astonishment or dismay was expressed, or in contraction of the superciliary muscles, which conveys a look of pain or of mournfulness. Spread to the scalp muscles may take place, causing a perpetual to-and-fro movement of the hair, of which O. and Miss R. supply examples. The platysma is sometimes the seat of a tic. One of Oppenheim's patients was a child with alternating twitches of his two platysmas; it is of interest to note he was able to contract either voluntarily. This condition is generally associated with similar contractions in other facial muscles, as in a case of facial and palpebral tic with platysma involvement recorded by Meirowitz,⁶² or as in young M.

A not infrequent accompaniment is a shrug of one or both shoulders, due to synergic contraction of the trapezius. The resulting complex may be considered an act of mimicry in so far as it is an expression of disdain.

TICS OF THE EAR – AUDITORY TICS

The muscles of the external ear come often into play. One of our patients had a tic of the left ear, consisting in visible elevation of the pinna. A case of tic of the ear muscles has been described by Romberg, and another by Bernhardt, in the distribution of the occipital and posterior auricular nerves. Reference is made by Seeligmüller⁶³ to a ten-year-old girl suffering from unceasing involuntary contractions of the eyelids and of various head and neck muscles, with wrinkling of the forehead and movements of the ears. His original diagnosis of chorea was discredited by his subsequently learning that the child, in common with a younger sister and a brother, had for several years been exercising herself by making faces, and in particular by attempting to move her ears.

It is quite conceivable that certain middle-ear phenomena are comparable to the tics. O. used often to complain of hearing noises in his right ear, which came and went with his tics of face and neck. Now, it is well known that the probable explanation of the humming sound attending forcible closure of the orbiculares palpebrarum is the variation in labyrinthine tension due to the synergic contraction of the stapedius. This absolutely normal effect may be exaggerated by predisposed and preoccupied individuals into a sort of auditory tic.

TICS OF THE EYES – NICTITATION AND VISION TICS

For the sake of precision, tics of the eyes may be subdivided into eyelid tics and eyeball tics.

A. Eyelid Tics.– These, perhaps the commonest of all tics, may be either unilateral or bilateral. They consist simply in a palpitation of the upper lid, repeated at irregular intervals, and differing from ordinary blinking only in augmented frequency and abruptness. The form they usually assume is that of a wink, attributable in the first instance to contraction of the orbicularis, but supplemented by the zygomatics and muscles of the nose.

The tonic variety of the same tic is constituted by a contraction of inordinate length, the outcome of which is the all but permanent maintenance of the eye in a half-closed position. The suspension of this tonic tic by volitional effort accentuates its distinction from contracture. In one of our patients a tic of this nature, which gave a singularly sleepy cast to the features, was easily relieved by suitable gymnastic treatment. The converse condition obtained in another case, where excessive gaping of the palpebral fissure contributed an unwonted fixity to the expression, which simultaneous contraction of the corrugator supercilii served to heighten into one of wild anger. These two tics corresponded to two diametrically opposed traits in their subject's character – viz. nonchalance and impatience respectively, and it is interesting to recall in this connection how the varying moods depend for their physiognomical delineation chiefly on the degree of curvature of the palpebral arc.

Valleix,⁶⁴ who employed the term "idiopathic facial convulsion" to designate tic, cites a case where even in moments of tranquillity the left eye seemed slightly smaller than its fellow, by reason of a feeble contraction of the orbicularis. Persistent grimaces of this kind resemble tics of attitude and stereotyped acts, and the possibility of their occurrence must not be overlooked, once the diagnosis of facial paralysis or spasm has been rigorously excluded.

The terms blepharospasm and blepharoclonus, sometimes applied to tonic and to clonic involuntary palpebral contractions respectively, ought to be strictly reserved for spasms and contractures properly so called. For example, von Graefe's case of blindness consequent on permanent closure of the eyelids in a child is undoubtedly one of blepharospasm. No tic could have been attended with such a result, whereas compression of branches of the trigeminal at their points of exit might determine reflex tonic contraction of the orbicularis, and so, for that matter, might a central lesion. Hence in these circumstances it is correct to use the word spasm.

Palpebral tics are among those that ordinarily begin by a spasmodic reaction to an extraneous source of irritation, such as that yielded by a foreign body, a speck of dust, an eyelash, or by any form of conjunctival inflammation.

Eyelid tics (says Parinaud⁶⁵) are known to ophthalmologists as clonic blepharospasms. Their starting-point is always some peripheral stimulus, in particular an everyday variety of conjunctivitis characterised by the presence of granulations in the lower part of the sac. To discover these granulations it may be necessary to explore the internal aspect of the lid. In my opinion, they are a prolific cause of tic, especially in young children, and their removal effects a cure in the vast majority of cases.

It is only when the blinking abides in spite of the suppression of the exciting cause that it can be comprised in the category of tics, otherwise the fact of its being contingent on the continuance of the irritation shows it is a spasm.

A bright light sometimes suffices to initiate these conditions. During a course of sittings for her portrait, G., a little girl eleven years of age, acquired the habit of drooping one eyelid slightly to shield the eye from the somewhat glaring light of the studio, but the persistence of this movement in other surroundings was evidence of its degeneration into a tonic tic.

Noir quotes the case of one of his colleagues who was for a long time inconvenienced by a most disagreeable blinking, which he held to be a tic; but a simple explanation was forthcoming in the unusual length of some of the eyelashes on the outer part of the upper lid having caused their entanglement with others in the under one, and when they were cut off the spasm disappeared.

In the following instance, reported by Toby Cohn,⁶⁶ the diagnosis remains undetermined:

The protracted use of a magnifying glass in the left eye was the means, in a watchmaker, of inducing occasional localised twitches of the orbicularis, which were not slow, however, in spreading to the whole of the left half of the face. They may at first have been an involuntary motor response to nipping of palpebral twigs of the trigeminal, but at a later period their independence was constant and pronounced. With certain associated movements such as articulation or deglutition, or during the act of wiping the nose or shutting the eyes, the form they assumed was tonic. There were neither subjective nor objective sensory phenomena to note.

We have recently had the opportunity of observing a genuine case of eyelid tic, of obscure origin perhaps, but one whose clinical features eliminate the hypothesis of spasm.

Brif., a metal polisher, forty-seven years old, came on March 10, 1902, to Professor Brissaud's clinic at the Hotel Dieu, complaining of involuntary closure of the eyes, especially when out walking. In his family and in his personal antecedents there was little or no neuropathic or psychopathic tendency. The sole trouble for which he sought advice was this spasmodic shutting of his eyes, rare enough under most circumstances, but aggravated instantly by a walk of even a few paces.

The onset had been quite insidious eighteen months previously, and at the first the average frequency was scarcely more than thrice or four times daily. Whenever Brif. passed into direct sunlight the movement was particularly liable to occur. As long as he remained seated at his work he was free from it, while he had but to rise and take a step or two for it to reappear and forthwith commence to repeat itself. At home any effort engaging his attention inhibited the tic, nor was there any sign of it in the course of our interrogation and examination of him.

Even when he was on his feet, the incidence of the act was not always uniform; if promenading with his wife and children, or fishing along a river side, or running to catch a tram, he was not hampered by his affliction. When he rose in the morning, it made its appearance ere he could reach the window to look out. During his journeys to and from his place of business, he was generally unable to moderate the spasmodic movements, particularly towards evening, whereas his professional pursuits in the daytime, and any occupation – such as reading the newspaper – when at home again, wholly counteracted the inclination to tic.

The production of this untimely gesture of his Brif. was disposed to attribute to the action of sun or wind, though he acknowledged the regularity of its occurrence irrespective of either. In its actual nature the contraction was tonic in type and of several seconds' duration, so that he used to cover some yards with eyes shut. From the outset the will had always exercised a marked influence on it, so much so that on certain days and for a certain space he could check the convulsion, and even when it was prolonged he contrived by volitional effort to open his eyes sufficiently to pilot himself in avoiding obstacles.

Careful search by the ordinary tests at the Quinze-Vingts hospital failed to reveal any abnormality whatever in his eyes. On our part, we satisfied ourselves that there was no restriction of the visual fields.

As far as his mental state was concerned, its chief peculiarity was a somewhat childish turn of mind, a soupçon of that psychic infantilism so common in the subjects of tic; in addition, he was of an emotional temperament, and prone to perspire or blush for no valid reason. He was further a victim to a premature baldness which was hereditary in the family, and which may be cited as a physical stigma of degeneration.

B. Eyeball Tics.– The extrinsic muscles of the eye occasionally participate in the tics we have just discussed. Assiduous observation of patients suffering from blinking tics will enable the physician now and then to detect movements of the eyeball behind the lowered upper lid.

In the case of F., for instance, with each tic of the lids the eyeballs deviated briskly upwards and to the left. Similarly Miss R. turned her head from right to left at the same time as the eye moved obliquely to the left and in an upward direction. A patient mentioned by Otto Lerch⁶⁷ used to open and shut his eyes while rotating the eyeballs and throwing the head back. Occasionally he inclined his trunk to one or other side, accompanying the act with disagreeable little grunts.

The eruption of these tics may equally be attributed to some foreign body or minute conjunctival granulation, as was the case with a small child of ten years under our care, who, in spite of the withdrawal of the irritating particle, acquired the trick of tickling the inner surface of his upper lid by rolling his eye about whenever he happened to blink. The delight he took in this trivial manœuvre led to its mechanical reiteration, and was the means eventually of its developing into a tic which required a sufficiently delicate muscle exercise and drill for its repression.

Defects in the visual apparatus sometimes induce abnormal movements and attitudes which may become tics if careful examination does not elicit their explanation.

Tic of the eyeball is generally associated with other tics, ocular or facial, but it may occur alone and bear a resemblance to nystagmus, a peculiarity we have noticed in a patient perfectly free from any cerebro-spinal disease. It is almost always bilateral, but in some cases of unilateral palpebral tic it is more pronounced on the side of the latter.

Fixity of the eyes is characteristic of tonic tics of the extrinsic ocular muscles, and gives a somewhat haggard or maybe merely attentive expression to the countenance. Very frequently it escapes observation, and indeed cannot be considered a tic unless there be an incongruity between it and the ideas at that moment uppermost in the patient's mind.

Reference has already been made to the historic example of an ocular tic in the person of Peter the Great. A series of interesting discussions has taken place recently at the Neurological Society of Paris in regard to the question of a tic of elevation of the eyes.

The patient, who had come to consult Professor Marie at Bicêtre in December, 1899, was presented to the Society in the first instance by M. Crouzon.⁶⁸ He entered the room with his eyes fixed on the floor, but in a few seconds they had resumed their normal position in the horizontal plane. At frequent intervals he raised them upwards, or inclined his head so as to bring the pupils into contact with the upper lids, the natural position of rest of the globes being regained by a voluntary effort after each displacement. When interrogated, he complained of not being able to distinguish objects in an area of his visual fields limited by an imaginary line drawn from his eyes to strike the ground at a point six feet in front of him; otherwise his sight was excellent. The history he gave was to the effect that five months previously, in the enjoyment of perfect mental and physical health, he had had a sudden stroke, and been unconscious for seventeen hours. No sinister results ensued till four days later, when he lost his vision, began to articulate very indistinctly, and failed to recognise his wife, continuing in that state for the next two months. Gradual recovery of speech and sight then commenced, but the habit of looking upwards persisted. The absence of injury to the visual apparatus, coupled with the presence of admitted psychical disorders, decided Crouzon in his consideration of the condition as a functional disturbance of the ocular muscles analogous to tic.

In this connection the significant observation was made by Joffroy that in the recumbent position the patient's eyes assumed their ordinary place, suggesting a comparison with those dolls whose eyes open or close according as they are held vertically or horizontally. In his opinion, the eye mobility negatived any idea of contracture consequent on central lesions.

A few months later the same patient was submitted a second time to the Society, on this occasion by M. Babinski,⁶⁹ who declared himself in disagreement with the hypothesis of M. Crouzon. In all cases of mental torticollis, so called, the contrary movement to that the execution of which is impelled by the spasm can from time to time be accomplished, whereas in the case under discussion downward as opposed to upward deviation was never obtained. Further, the acute onset, with loss of consciousness, militates strongly against the tic theory, and indicates rather a variety of paralysis of the inferior recti, or paralysis of conjugate downward movement, secondary to organic disease of the nervous system. The difficulty experienced by the patient in inducing his eyes to resume the horizontal position after once elevating them is explicable on the assumption that the action of the superior recti is no longer controlled by their antagonists the inferior recti, the former passing into a state of temporary spasm, which is, however, strictly consecutive to the paralysis of the latter.

M. Parinaud expressed himself as being in accord with M. Babinski, and recalled certain rare forms of associated ocular palsies occurring with paralysis of convergence, a combination manifest in the subject in question. Curiously enough, in these cases the disturbance of function is always ushered in by a stroke, which justifies the belief in the focal nature of the lesion.

On the other hand, it was noticed by M. Ballet that the range and facility of downward deviation varied inversely with the attention devoted to the patient by the examiner.

On yet a third occasion this identical case provided a subject of discussion at the Society, after being under the observation of Professor Pierre Marie in Bicêtre.

Professor Marie⁷⁰ had failed to satisfy himself of the paralytic nature of the phenomenon, and demonstrated the ease with which the eyeballs moved downwards if the patient was made to hold his head in the position of maximum extension, while in the attempt to look at his feet – the head being held normally – they were forthwith inclined violently upwards, and were so maintained for thirty or forty seconds. The only view tenable was that he was suffering from a sort of neurosis whose outward expression was this spasmodic elevation of the eyes. Additional confirmation of the accuracy of this hypothesis was supplied by a consideration of the circumstances attending the commencement of the illness. The sudden and unexpected apoplexy, of seventeen hours' duration, had been accompanied neither by stertor nor by relaxation of sphincters, and had been followed by an equally sudden return to consciousness, the faculty of speech and the desire for food reasserting themselves unexpectedly. The ensuing three or four weeks the patient had spent in a curious delirious state, not unlike the post-seizure stage of hysteria, a trace of which remained in the guise of certain eccentricities of mind. The difficulty in his speech bore a resemblance to hysterical stammering; and, finally, his visual fields were concentrically and bilaterally restricted.

Of the subsequent history of the case some information was forthcoming at a later date,⁷¹ corroborating the opinion originally propounded by Professor Marie. Simultaneously with the diminution in intensity of the ocular spasm there had been grave deterioration of the patient's mental level, as evidenced by the development of ideas of persecution.

In the subjects of tic, and especially in cases of mental torticollis, we have noted an analogous symptom, consisting in inability to look down at the feet, except perhaps by the aid of innumerable contortions, in contrast to the consummate ease of upward glances. By making the person write at a blackboard, and observing his action according as his hand is above or below a horizontal plane through his eyes, one can soon convince oneself of the reality of the occurrence, yet search will fail to discover any sign of ophthalmoplegia.

Patients of this class evince a remarkable aptitude for elevation movements, and the trouble they experience in depressing the eyeballs is not of necessity to be construed as denoting paralysis of the depressors, but rather indicates the presence of a tic of the elevators, as Professor Marie says – a tic born of a habit, and nourished perhaps by the dread such persons feel of witnessing an exaggeration of their convulsive movements whenever they cast their eyes down.

Our object in summarising this discussion has been twofold: at once to note the existence of tics of extrinsic eye muscles, and to illustrate the intricacies of their diagnosis.