Tics and Their Treatment

From our rapid survey of the vicissitudes through which the tic has passed, we may profitably gather one or two lessons.

In so far as is compatible with its nature, the schematisation of tic is indispensable. The inevitable variability of the personal factor and the absence of a real breach of continuity between any two essentially differing morbid affections ought not to deter us from the attempt to project a line of demarcation between them. Natural science is pledged to the labour of differentiation. It is the glory of Charcot's alternately synthetic and analytic work to have demonstrated the value of this method in the sphere of neuropathology. At the same time, the wisdom of attaching only a provisional importance to any scheme and of welcoming possible modification is of course self-evident. Inexact and undiscriminating inference may be a stumbling-block in the path of progress and inimical to the cultivation of the faculty of observation. Further, inaccuracy of definition not only exaggerates the liability to misunderstanding, but has sometimes also the disadvantage of promoting an illusory belief in the possession of the truth.

CHAPTER III

THE PATHOGENY OF TIC

TIC AND SPASM

Our study of tic can be approached only after a preliminary understanding as to the meaning of two words too frequently confounded even in scientific literature —tic and spasm. Let us explain, then, once for all, exactly what we intend by the latter.

Etymologically (σπασμὁς, σπἁω, I draw) the word signifies a twitch, but as it is unfortunately considered a synonym for convulsion, the two expressions are used indifferently in medical parlance, though the desirability of restricting the application of the former has more than once been indicated. Littré's definition – "an involuntary contraction of muscles, more particularly of those not under voluntary control" – may appear somewhat idle, as the contraction of muscles not under the influence of the will can scarcely be other than involuntary. His intention was, no doubt, to reserve spasm for convulsive phenomena in non-striped muscle fibres; but in this limited sense the term has not met with acceptance, and it remains equivalent to "involuntary muscular contraction," whatever that may mean. Thus interpreted, it is applicable to any and every involuntary muscular movement, physiological and pathological, to the inco-ordination of tabes, to chorea, athetosis, tremor, etc.

Rather than imagine a new substantive to characterise certain of these muscle contractions, we may retain the word in a somewhat wider though equally precise sense, and follow the distinction drawn by Brissaud9 in 1893: "a spasm is the result of sudden transitory irritation of any point in a reflex arc; … it is a reflex act of purely spinal or bulbo-spinal origin."

By definition, then, a spasm is the motor reaction consequent on stimulation of some point in a reflex spinal or bulbo-spinal arc. To differentiate between the reflex, which is physiological, and the spasm, which is pathological, we may add as a corollary: the irritation provocative of the spasm is itself of pathological origin, and no spasm can occur without it. The anatomo-pathological substratum of a spasm is, then, some focus of irritation on a spinal or bulbo-spinal reflex arc, which may be situated in peripheral end organ, in centripetal path, in medullary centre, or in centrifugal fibre. Whatever be its localisation, it will determine a spasm in our sense of the word.

Cortical or subcortical excitation, however, as well as peripheral stimuli, may provoke these bulbar and spinal centres to activity. Irritation of a point on the rolandic cortex, or on the cortico-spinal centripetal paths, is followed by a motor reaction exactly as with afferent impulses; the sole change is in the route taken by the centripetal stimulus; the reflex centre remains bulbo-spinal, and the efferent limb of the arc is as before.

The application of the word spasm to these motor responses to cortical or subcortical stimulation is quite justifiable. Developmentally the grey matter of the cerebral convolutions is ectodermic, as is the skin, and capable of functioning as a sensory surface; it may be considered the end organ of an afferent path that conducts to medullary reflex centres. According to our definition, then, provided the centre of the reflex arc be bulbo-spinal and the irritation pathological, the consequent motor phenomenon is a spasm.

A distinction most nevertheless be drawn between the two cases, inasmuch as in the one the afferent path is peripheral, in the other it is cortico-spinal, and there is a corresponding difference in the clinical picture. Jacksonian convulsions, consecutive to cortical stimulation, do not seem to bear much resemblance to spasmodic movements indicative of peripheral —i. e. sensory nerve – irritation. As a matter of fact, it is not always easy to differentiate the two, except by the aid of concomitant phenomena. The characteristic evolution of the Jacksonian convulsion is of course readily recognisable. We can similarly diagnose an irritative lesion of the internal capsule not so much from the objective features of the convulsive movements as from accompanying indications. In short, there need never be any occasion for confusion. Convulsive conditions attributable to irritation of cortico-spinal centripetal paths have long been described and analysed: they constitute well-recognised morbid entities, among which may be enumerated Jacksonian epilepsy, hemichorea, hemiathetosis, pre-and post-hemiplegic hemitremor, etc.

These clinical denominations for the affections under consideration it is at present desirable to retain. We shall not call them spasms; above all, we must not call them tics, else we shall end by confounding conditions absolutely distinct. The case recorded by Lewin,10 under the title of "convulsive tic," of a three-year-old infant still unable to walk, who has daily attacks in which "all the muscles" twitch for about a minute at a time, is indeed a most singular tic. We were under the impression that such an attack is usually known as an epileptiform convulsion. Is the term "convulsive tic" quite a happy synonym?

Again, in the recent thesis of Cruchet the attempt has been made to base the pathological physiology of tic on researches of von Monakow and Muratow apropos of the occurrence of choreic, epileptoid, or athetotic movements after certain lesions of the cerebro-spinal axis, and to find an analogy in the action of various convulsion-producing substances (Richet and Langlois). Cruchet's conclusion is that convulsive tic is as often cortical or subcortical as spinal in origin; that it is, in short, a mere symptom, common to many cerebro-spinal conditions.

The same regrettable confusion is discernible in various treatises on neuropathology the work of German and other foreign authors.

As far as we are concerned, the outcome of the whole matter is simply this: if tic is doomed to be used indifferently for convulsion, its retention in scientific terminology is unjustifiable. Rather, then, than widen its application, we prefer to restrict it; we shall employ the term convulsion in its most general sense of "any anomaly due to excess of muscular contraction," of whatever variety or origin; and we shall limit the use of the word spasm to phenomena the result of irritation at any point on afferent or efferent reflex paths, or in reflex bulbo-spinal centres.

In thus indicating our position, we find ourselves once more in accord with generally received opinion since the days of Charcot. These views have been excellently expressed by Guinon:

Convulsive movements differ widely in kind. Some consist of localised spasms in the domain of a motor or mixed nerve, most frequently one of the cranial series – in especial the seventh – consecutive to some anatomical lesion, central or peripheral. The great majority of observers, French and foreign alike, are in the habit of designating such movements "tics." … But they are only partial convulsions limited to the area of some one nerve, not true convulsive tics, differing alike in essential features and concomitant symptoms. From the anatomo-pathological standpoint, moreover, lesions are as constantly present in the one as absent in the other.

The opinion of Brissaud on the subject coincides with our own.

If we suppose now that the cortex ceases to act as a surface of peripheral excitation, and becomes itself a reflex centre, we note at once a complete change. The modification effected by the cortex on afferent impressions is obvious in altered motor reactions, which appear with the stamp of cortical intervention, herein differing from bulbo-spinal phenomena. To this category belong the tics; we shall soon see why and how.

Conformably, then, to convention sanctioned by usage, and especially by the teaching of Charcot and Brissaud, we have given a precise definition to the word spasm, and we can only solicit its general adoption.

To resume briefly the argument we have advanced in the foregoing paragraphs, we maintain:

If in a given motor phenomenon there is no evidence of actual or previous cortical intervention, it is not a tic.

If the motor reaction is consecutive to pathological irritation at any point on a bulbo-spinal reflex arc, it is a spasm.

If the cortex is or has been involved in its production, it is not a spasm.

Should it present, in addition to the fact of cortical participation, certain distinctive pathological features, it is a tic.

It is precisely these distinguishing characteristics that we shall now proceed to examine, preluding our study of them with one or two physiological considerations.

TIC AND MOTOR REACTIONS; REFLEX, CO-ORDINATED, FUNCTIONAL, AUTOMATIC, AND VOLUNTARY ACTS

The instantaneous muscular contraction that follows the application of a drop of sulphuric acid to the limb of a decerebrate frog is an example of a pure spinal reflex. With the persistence of the irritation contraction of the other limb and of the whole body ensues; the simple spinal reflex has become generalised. Observe the frog a little longer. Soon the sound foot approaches the affected limb and attempts by rubbing to remove the point of irritation. A movement of attack has succeeded the simple movement of defence, and indicates a complete change in the nature of the motor reaction. In the first case the limb is withdrawn briskly from the painful stimulus; in the second the animal performs a series of co-ordinated purposive movements. The first reflex is automatic, and so no doubt is the second, since the frog is decerebrate. But a co-ordinated movement is not of necessity automatic from the outset; its automatism may be the sequel to voluntary education. Co-ordination is often a manifestation of cortical activity.

Take, next, the case of the infant. His earliest muscular movements are pure spinal reflexes. Pinch his leg, and he withdraws it; continue the stimulus, and he moves the other leg, his arms, his whole body; he starts to cry. The original reflex is becoming generalised, yet he makes no attempt to remove the source of irritation. Should a particle get into his eye, his lids will blink so long as the pain persists, but he never rubs them to expel the foreign body. In Virchow's phrase, the newborn infant is a spinal animal, endowed with spinal reflexes only; his responses to stimuli are beyond voluntary control.

More complex motor phenomena, however, equally independent of cortical influence, characterise the early days of the infant's life. The contact of his lips with the breast at once elicits a reflex in the shape of sucking movements. These are obviously co-ordinated and adapted for a particular end; suction is a functional act. Yet the cortex plays no part therein; the act is automatic from the beginning. Peripheral excitation from tactile impression of nipple, teat, or finger is sufficient to provoke this reflex response.

Similarly with the functions of respiration and nictitation – their establishment follows the stimulation by air of the respiratory or conjunctival mucosa. The appropriate movements constitute the spontaneous reaction to afferent impulses; they are simple bulbar reflexes. Co-ordinated and purposive though they be, they do not come within the sphere of the will. The newborn child cannot voluntarily accelerate or retard his respiratory rhythm.

But a day comes when the formation of cortico-bulbar or cortico-spinal anastomoses renders possible the interaction of higher and lower centres; respiration may be made quicker or slower; the eyelid may be closed less rapidly, more often. In a word, cortical modification of function becomes a reality.

A further step in advance is soon taken.

Under the "law of least effort" the inhibitory power of the will reduces motor reaction for the attainment of a given object to a minimum. The infant begins to make more complicated movements, attempting the removal of a source of annoyance by direct attack, learning to scratch itself, to spit instead of swallow, etc.

The essential difference between these acts – a thousand other examples might be chosen – and the reflexes of the first group, is that the precise and regular execution of the former demands more or less prolonged education, repetition, and voluntary co-ordination.

It is true these co-ordinated acts are eventually performed with all the spontaneity of the simplest reflexes; voluntary co-operation is no longer indispensable; scratching, spitting, walking, can be effected without any actual intervention of the will. But we must not forget such muscular automatism entails a preliminary training in the shape of frequent repetition of purposive movements – a training which varies in duration with the individual and the nature of the particular movement. It is only after several years of volitional effort that such acts as locomotion or the expulsion from the throat of an irritant particle become really automatic.

The fact that the newly hatched chick is capable of walking has been advanced as an argument for the existence of congenital automatism. It is true that the chick's movements are very imperfect – it stumbles and falls, as does the infant, on the slightest provocation, and even without any apparent cause; but the rapidity with which certain animals acquire the faculty is so surprising that the latter almost appears to have been innate.

In all phenomena characterised as instinctive we cannot deny the existence of a certain congenital aptitude, the result possibly of ancestral education, owing to which some individuals learn infinitely more quickly than others, and in their case a period of preliminary education may seemingly be awanting. Probably the truth is, however, that this stage has been a very brief one. In man there is a gradual transformation of voluntary into automatic acts. Though no teacher be necessary, teaching is requisite. The infant learning to walk is really independent of his parents, and might, for that matter, be entirely self-taught; but the point remains, however automatic his walking subsequently become, that he begins by voluntarily co-ordinating the movements of his lower limbs and trunk towards a definite end.

Another advance is still to be made.

With increasing cortical development the individual is able, on stimulation no longer peripheral but central in origin, spontaneously to execute movements which frequent repetition has endowed with all the features of functional acts. Of these ideomotor phenomena physical exercises, games, manual trades, readily furnish instances. Swimming, for an instance, requires the rhythmical co-ordination of arm and leg, to attain which perseverance, retentiveness, and above all repetition are essential. At length the time arrives when the swimmer is surprised at the absence of any necessity for voluntary co-ordinating effort on his part. In fact, to reintroduce volition into this acquired automatism would be to court disaster. "What I do naturally," said Montaigne, "I can no longer perform if I attempt it expressly."

From these physiological considerations we are led to make the following classification of motor reactions:

1. Simple spinal reflexes, innocent of co-ordination or functional systematisation, on whose production or inhibition the will has no influence. To this division belong the movements known as spasms.

2. Functional motor acts. Among these we may distinguish:

a. Essential movements, e. g. respiration, suction, etc., appearing at birth, and co-ordinated in view of some definite function.

b. Acts such as locomotion, mastication, etc., whose acquisition is subsequent to a more or less prolonged period of education.

c. Non-essential ideomotor acts, acquired later in life, which soon assume all the characters of functional acts.

The movements belonging to the first group in this latter category may manifest themselves without any exertion on the part of the will, but its activity is essential to the perfecting of the second, and the originating of the third.

In this last division are placed the motor phenomena known as tics.

TIC AND CO-ORDINATION

We have thus come to see that a tic is a co-ordinated, systematised, purposive act. The majority of observers are satisfied on this point, although there exist various differences of opinion, more apparent than real, the inevitable result of disagreement as to the interpretation of certain expressions. It is imperative to obviate misunderstanding once and for all.

In his first contribution to the study of the disease which bears his name, Gilles de la Tourette gave the general description of motor inco-ordination to the convulsive movements of his patients. It has been argued by Guinon, on the contrary, that they are really systematised, and that they reproduce, in an involuntary manner, the co-ordinated movements of everyday life. That this is sometimes the case Tourette subsequently admitted, but he still professed their frequent actual inco-ordination.

This divergence of opinion is entirely attributable to difference of interpretation. Littré's definition of muscular inco-ordination is, "A condition occurring in various diseases of the nervous system, in which the patient cannot co-ordinate the necessary muscular movements for walking, grasping an object, etc." In this sense the term is applicable indiscriminately to the gesticulations of choreic, athetotic, or tic patients; to the ataxia of tabetics and others; to the tremor of disseminated sclerosis or paralysis agitans, etc. An expression so general is not merely of no diagnostic value; it leads to positive confusion.

It is precisely in the type of inco-ordination that the difference lies. As rigorous a distinction must be drawn between the gestures of chorea and the gesticulations of the sufferer from tic as between the tremor of insular sclerosis and of Parkinson's disease.

In assigning an exact meaning to the term muscular inco-ordination, we cannot do better than quote the remarks of Guinon:

The tabetic who throws his legs to right and left, who as he sits at table cannot carry his spoon to his mouth, furnishes an instance of true motor inco-ordination. On the other hand, the subject of tic performs his voluntary actions with perfect assurance; though his infirmity occasion all sorts of ridiculous involuntary arm movements, he never brings his fork against his ear or his cheek, nor does he spill a drop from his glass; his walk may be interrupted by a sudden halt to bend his knees and kneel, or to strike his foot violently on the ground, but he never trips one leg over the other and never falls.

In his article in the Dictionnaire Jaccoud, Letulle distinguishes two kinds of tics:

The convulsive tic consists of a series of partial convulsions, while the co-ordinated tic is the expression of some complex act by a sequence of muscular contractions for that purpose. In the former case the resulting movement is irregular, abnormal, and useless; it is a muscular "shock" evolved without reason and continued without effect… The normal individual usually possesses in potentia all the elements for the genesis of a co-ordinated tic. Some little trick or mannerism, arising perhaps from the necessity of gaining time for reflection, or from the desire of concealing some innate timidity, or of dissimulating some preoccupation, becomes sooner or later involuntary and automatic, and though maintaining its regularity and co-ordination, passes insensibly into the realm of pathology.

The distinction, however, is far from being absolute. Letulle himself admits it is a question of degree rather than of kind; the co-ordinated tic differs from the first variety only in its greater extent, complexity, and duration. Now, the convulsive tic may be a local, partial, irregular, abnormal convulsion, yet these characteristics are not sufficient to differentiate it: biting the lips is classed by Letulle as a co-ordinated tic, but it is surely a local, partial, irregular, abnormal muscular act; and the explosive laryngeal "ahem!" he would similarly place, yet it cannot be said to be a phenomenon characterised by its extent, complexity, and duration.

According to Guinon, a further distinguishing feature of the convulsive tic is its frequent though inopportune reproduction of some reflex or automatic purposive movement of everyday life, whereas we have just seen that one of the elements in Letulle's co-ordinated tic is its purposiveness. In a word, these observers apply the same epithet to two varieties of tic which they are endeavouring to separate.

The explanation of the apparent contradiction is simple. A gesture which seems meaningless and useless to-day becomes intelligible and logical to-morrow, when we learn the reason for it. In the course of an attack of conjunctivitis a patient acquires the habit of winking his eye, and though the inflammation subsides, the habit persists. If we are ignorant of its cause, are we to call the tic convulsive since it appears to us needless? And if we do know its origin; can we say it is co-ordinated when one muscle only is involved in the contraction?

The distinction drawn by Letulle between the two groups may hold good in some cases, but certainly not in all, and in our opinion it is preferable to abstain entirely from the attempt to base a classification on variation in muscular contraction. Noir remarks very justly that intermediate forms occur which are difficult to place in one or other category. In face of the confusion to which an illogical division inevitably leads, we may safely leave this question aside. In our view, the motor phenomena of the disease are always systematic, co-ordinated movements, directed for the attainment of some definite object. We exclude all simple bulbar or spinal reflexes, and all spasms, since the cardinal feature in these conditions is the absence of any functional systematisation.

THE GENESIS OF TIC

We have seen how various purposive, co-ordinated movements may, by dint of education and voluntary repetition, become automatic and be automatically repeated should occasion arise. Imagine some such act recurring involuntarily without any apparent reason and for no apparent object; what does such an anomaly signify?

Take, for instance, the case of a young girl who inclines her head on her shoulder to relieve the pain of a dental abscess. The act is called forth by a real exciting cause; the muscular response is voluntary, deliberate, undeniably cortical in origin: the patient wills to appease the pain by pressing and warming her cheek. Should the abscess persist, the movement will be repeated less and less voluntarily, more and more automatically; but as the why and the wherefore still remain, there is nothing pathological about it.

With the healing of the abscess, however, and the consequent relief of the pain, the girl still inclines her head on her shoulder from time to time, albeit cause and purpose have ceased to operate. Her primarily volitional, co-ordinate, systematic, motor reaction is now automatic, inopportune, and meaningless: it is a tic.

Charcot11 has given us an excellent description of the process:

However complex and bizarre may appear the convulsive phenomena known as tics, they are not always as irregular, inco-ordinate, and contradictory as superficial examination might lead one to believe. On the contrary, they are, as a general rule, systematised; in a given case they recur always in an identical manner, reproducing, and simultaneously exaggerating, complex, automatic, purposive movements which are essentially physiological; they are in a sense the caricatures of ordinary acts and gestures. The tic is not in itself absurd; it appears so only because it occurs inappositely, without obvious motive. Source of irritation is absent, yet the patient scratches himself; he blinks, but no foreign body is to be detected in his eye.