Kitabı oku: «Tics and Their Treatment», sayfa 3

CHAPTER II
HISTORICAL

WE have just become acquainted with an individual who may, we believe, be considered the type of a species, and have described all his tics. What is a tic, then?

Its etymology has not much information to furnish. The probability is that the word was originally onomatopœic, and conveyed the idea of repetition, as in tick-tack. Zucken, ziehen, zugen, tucken, ticken, tick, in the dialects of German, tug, tick, in English, ticchio in Italian, tico in Spanish, are all derivatives of the same root. It matters little, in fact, since the term is in general use and acceptable for its shortness and convenience. In popular language every one knows what is meant by a tic: it is a meaningless movement of face or limbs, "an habitual and unpleasant gesture," as the Encyclopædias used to say. But the definition lacks precision.

A glance at the history of the word will reveal through what vicissitudes it has passed. We need but remind the reader of its exhaustive treatment in the Dictionaries, and refer him for an elaborate bibliography to a recent work by R. Cruchet,¹ to which we shall have occasion to return.

There is no justification for regarding the risus sardonicus of the ancients as a tic. All that we can say is that the phrase apparently stood for a complex of facial "nervous movements," whether accompanied by pains and paralyses or not. Nor can the rictus caninus or the tortura oris have been other than spasms or oontractures of the face.

Previous to its introduction as a technical term, the word tique, ticq, tic, was in current use in France, and applied in the first place to animals. In 1655 Jean Jourdin described the tique of horses. In eighteenth-century literature tic appears in the sense of a "recurring, distasteful act" – as expressed by the Encyclopædia– especially in individuals revealing certain eccentricities of mind or character. This old-time opinion is worth remembering, particularly in view of latter-day theories.

Once adopted by the eighteenth-century physicians, the application of the word was extended in various directions. André (1756) was the first to mention tic douloureux of the face, an affection excluded to-day by common consent from the category of true tics. Simple, painless convulsive tic, spreading from face to arms, and to the body as a whole, was differentiated by Pujol in 1785-7. During the earlier half of the nineteenth century no solid progress was achieved by the work of Graves, François (of Louvain), Romberg, Niemeyer, Valleix, or Axenfeld. It is to the clinical genius of Trousseau that we owe the rediscovery of tic, the careful observation of its objective manifestations, and the recognition of accompanying mental peculiarities.

In spite of the fact that he considered it a sort of incomplete chorea, and classed it² nosologically with saltatory and rotatory choreas and with occupation neuroses, Trousseau's original account remains a model of clinical accuracy:

Non-dolorous tic consists of abrupt momentary muscular contractions more or less limited as a general rule, involving preferably the face, but affecting also neck, trunk, and limbs. Their exhibition is a matter of everyday experience. In one case it may be a blinking of the eyelids, a spasmodic twitch of cheek, nose, or lip; in another, it is a toss of the head, a sudden, transient, yet ever-recurring contortion of the neck; in a third, it is a shrug of the shoulder, a convulsive movement of diaphragm or abdominal muscles, – in fine, the term embodies an infinite variety of bizarre actions that defy analysis.

These tics are not infrequently associated with a highly characteristic cry or ejaculation – a sort of laryngeal or diaphragmatic chorea – which may of itself constitute the condition; or there may be a more elaborate symptom in the form of a curious impulse to repeat the same word or the same exclamation. Sometimes the patient is driven to utter aloud what he would fain conceal.

The advantage of this description is its applicability to every type of tic, trifling or serious, local or general, from the simplest ocular tic to the disease of Gilles de la Tourette. Polymorphism is one of the tic's distinguishing features.

Apart from his studies in objective localisation, Trousseau, as we have seen, recognised that the tic subject was mentally abnormal, but the credit of demonstrating the pathogenic significance of the psychical factor is Charcot's. Tic, he declared,³ was physical only in appearance; under another aspect it was a mental disease, a sort of hereditary aberration.

Advance along the lines thus laid down has been the work more especially of Magnan and his pupils, of Gilles de la Tourette, Letulle, and Guinon. A meritorious contribution to the elucidation of the question is the thesis of Julien Noir, written under the inspiration of Bourneville and published in 1893. The still more recent labours of Brissaud, Pitres, and Grasset in France, and of others elsewhere, have added materially to our knowledge.

Confining ourselves for the present to the discussion of the latest interpretations put on the word tic, we may be allowed the remark that if the influence of Magnan's teaching has been instrumental in making our idea of tic conform more to the results of observation, nevertheless his view is not without its dangers.

In the opinion of Magnan and his pupils, Saury and Legrain⁴ in particular, the tics do not form a morbid entity; they are nought else than episodic syndromes of what Morel called "hereditary insanity," that is to say, of what is usually designated nowadays "mental degeneration."

Now, if by degeneration be meant a more or less pronounced hereditary psychopathic or neuropathic tendency which betrays itself by actual physical or psychical stigmata, then tic patients are unquestionably degenerates. If degeneration unveils itself in multifarious psychical or physical anomalies, the subjects of the tic are undoubtedly degenerates. If a degenerate may suffer from one or other variety of aboulia, or phobia, or obsession, the man with tic is a degenerate too.

Thus understood, the epithet may be applied to all individuals affected with tic. In fact, they must be degenerates, if the word is to be employed in its most comprehensive sense. But the explanation is insufficient, inasmuch as the converse does not hold good; all degenerates do not tic.

We may be safe in maintaining, then, that tic is only one of the manifold expressions of mental degeneration, but we are not much enlightened thereby. Obsessions and manias similarly are indications of mental deterioration, yet the fact conveys very scanty information as to their real nature. Physical anomalies – ectrodactyly, for instance – betoken physical degeneration, no doubt; but are inquiries to cease with this categorical assertion? Such certainly was not the idea of those observers whose is the praise for having demonstrated the common parentage of the heterogeneous manifestations of degeneration. Synthesis cannot exclude the work of analysis, and in practice there is scarcely a case to which this doctrine is not pertinent.

Every physical and every mental anomaly is the fruit of degeneration; every individual who is a departure from the normal is a degenerate, superior or inferior as the case may be. As instances of the latter we may specify the dwarf and the weak-willed; of the former, the giant and the exuberant. This sane and comprehensive conception of the subject must command universal acceptance as a synthetic dogma, but it cannot supplant the description and interpretation of individual facts. However legitimate be our representation of tic as a sign of degeneration, it is obviously inadequate if we rest content with styling its subject a degenerate.

Unfortunately the inclination too often is to be satisfied with the term, and to imagine that therewith discussion terminates. Still more unfortunately, in concentrating their attention on the mental aspect of the disease, some have altogether lost sight of one of its fundamental elements, viz. the motor reaction, and have conceived the possibility of its occurrence without any tic at all. Cruchet actually postulates the existence of an exclusively psychical tic, with no external manifestation.

To these questions, however, we shall return. The present introductory sketch is intended merely to demonstrate the ease with which ambiguity arises, and the desirability of its removal. We are fully conscious of the value of the work of Magnan and his school in emphasising a phase of the subject the exposition of which can only result in gain.

The investigation of the motor phenomena of tic is no less encircled with perplexities. Not only are the troubles of motility boundless in their diversity and correspondingly difficult to classify, but they also bear so close a resemblance to a whole series of muscular affections that one is tempted to describe a special symptomatology for each individual case.

For several years there has been, more especially outside of France, a manifest tendency to aggregate all convulsions of ill-determined type into one great class, under the name "myoclonus"; and into this chaotic farrago, it is to be feared, will tumble a crowd of conditions which should be studiously differentiated: the tics, electric and fibrillary choreas, paramyoclonus multiplex, etc., etc.

In the present state of our knowledge, according to Raymond,⁵ we must be guided by the lessons of clinical experience, which teach us, first, that the varying modalities of myoclonus develop from the parent stock of hereditary or acquired degeneration; and, secondly, that transitional forms which do not fall into any of the received categories are of common occurrence.

From a general point of view, the deductions are entirely reasonable. There is a suggestive analogy between these conditions and the muscular dystrophies in the persistence with which their multiplicity seems to defy the efforts of classification. The analytic stage witnessed the rapid evolution of such clinical types as the facial, the facio-scapulo-humeral, the juvenile, the pseudo-hypertrophic, not to mention others that bear the name of their observer; but it has been succeeded by the synthetic stage, whose function it is to incorporate all the former myopathies in the comprehensive group of "muscular dystrophy."

Yet here, again, peril lurks in too hasty a generalisation. To give the disease a name is not equivalent to pronouncing a diagnosis. The denominations "myoclonus," "muscular dystrophy," "degenerate," are alike inconvenient. Their scope is at once too inclusive and too exclusive. They may be indispensable; they are assuredly not sufficient.

The possibilities of misapprehension do not end here.

The manifestation of each and every tic – be it a flicker of the eyelid, a turn of the head, a cry, a cough – is through the medium of a muscular contraction. On the very nature of this contraction opinion is divided.

To its distinctive features of abruptness and momentariness is due the epithet "convulsive" habitually assigned it, but the qualification is not secure. Since the time of Willis the word convulsion has been employed in a double sense, to signify clonic muscular contractions (the "convulsion" of popular parlance) and tonic muscular contractions (a meaning attached to the term only by the scientist).

For our part, we can raise no valid objection to the specification of tics as convulsive, provided always that the existence of clonic convulsive tics and of tonic convulsive tics be recognised. As a matter of fact, clinical observation supplies instances of both sorts.

Nevertheless, attention has been confined by a majority of authors to the consideration of the former variety only, so much so that a whole order of facts which in derivation, essence, and external characteristics ought to be identified with the tics has been passed over in silence. Even on the assumption that the proposal to recognise the two classes cannot be entertained, at the least it is advisable to predetermine the import of the word convulsion, and to speak of clonic convulsive tics. This is the formula of Ferrand and Widal in their article "Convulsion" in the Encyclopædic Dictionary of the Medical Sciences. Similarly, Troisier⁶ says that the convulsive tic properly so called is characterised by clonic movements, in which opinion Erb and most German observers concur. Tonic tic appears to have been forgotten, and we have thought it our duty to resuscitate it.

Cruchet has quite recently approached the subject in a critical fashion:

To extend the term tic to tonic spasms such as mental torticollis, mental trismus, or permanent blepharospasm, is singularly to outstep the limits of its significance. We believe Erb, Troisier, and Oppenheim are warranted in restricting convulsive tic to clonic convulsions, and the consequent simplification and elucidation of the question lead us to adopt the same view.

If it be solely a matter of terminology, and if universal consent reserve tic for convulsions whose expression is clonic, we shall be the first to withdraw the phrase "tonic tic," making the single proviso that some other designation be found for a condition which differs from the clonic tic only in its external features, and not in origin, pathogeny, or treatment.

What is this other name to be? Are these tonic muscular contractions to be regarded as synonymous with contractures? If so, do we mean myotetanic contracture – to utilise the excellent division imagined by Pitres – as in hysteria, or myotonic contracture, as in Parkinson's disease? The state of muscular contraction in tonic tic does not correspond accurately to either, though it is certainly more akin to the myotonic form; but myotonia is a sort of caput mortuum for the too facile classification of cases in reality difficult to place, and we are afraid the term is not calculated to ensure precision of ideas.

Should we be reproached with straining the primary meaning of the word tic by applying it to a contraction of a certain duration, we find ample justification ready at hand in the pages of Cruchet himself. "It was probably in 1656," he says, "that tique appeared in the French language, in the works of Jean Jourdin." Now, in the quaint description of the horse's tique given by that writer, the signs of the disease are said to be cocking of the ears, rolling of the eyes, clenching and gnashing of the jaws, stiffening of the tail, nibbling at the bit, etc. What else are these than persistent contractions or tonic tics, alternating or co-existing with jerking movements or clonic tics?

We have no desire, of course, to over-estimate the argumentative value of this passage, the interest of which is mainly historical; but we find ourselves wholly in accord with Cruchet when he remarks of the scientific distinction formulated by Willis, and again by Michael Etmüller, between continuous, permanent tonic convulsions, and intermittent, momentary clonic convulsions, that it is uninvolved, practical, and of universal applicability.

In 1768 certain tics were classified among the tonic convulsions by Boissier de Sauvages. Marshal Hall⁷ gave an account of various tonic facial convulsions to which Valleix refers as non-dolorous tics or idiopathic convulsions of the face. Coming nearer to our own times, we find the distinction of which we have been speaking again elaborated by Jaccoud,⁸ in 1870, and accepted also by Rosenthal.

Doubtless physiologists and pathologists are not invariably at one as regards the proper characters of the two, and subdivisions into continuous tonic contractions as opposed to intermittent tonic contractions have been deemed necessary; but without burdening the subject with a plethora of detail, we think it simple, suggestive, and clinically satisfactory to uphold Willis's generalisations and to enlist their help in the exposition of the tics. Hence, unless under special circumstances, we consider recourse to the epithet "convulsive" superfluous, and we shall employ the word tic by itself, except when there may be occasion to indicate the form of muscular contraction. The gain in conciseness is not likely to be neutralised by any loss of precision.

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.