THE EVOLUTION OF TIC
Tic is, from its nature, highly variable in its evolution; each tic has
a development peculiar to itself. Mental differences among individuals
have their counterpart in physical differences, in health as well as in
disease, and a comprehensive sketch of the evolution of tic is therefore
impracticable. We shall restrict ourselves accordingly to a few general
remarks.
In the great proportion of cases of tic the onset is an insidious one.
We have already made a sufficiently detailed examination into the
pathogenic mechanism to obviate any repetition in this place, but we may
note how unsettled the earliest manifestations are, how a tic may pass
from one muscle or group of muscles to another, and even when its
exciting cause is patent an apprentice stage always precedes its final
establishment. Of the truth of this the history of J. provides an
excellent instance. Another one is from Pitres:
A nine-year-old boy received a severe shock one day through being
pounced on by some companions who were in hiding behind a wood
pile, and though the emotion was of short duration, he commenced a
few days later to exhibit involuntary muscular twitches of the
upper part of his body, and to utter suppressed cries. The
phenomena increased in violence and in frequency, and, in spite of
treatment, a year later he was not freed of them entirely. For an
unknown reason the tics renewed their activity when he was
seventeen and continued so for the next three years, until a spell
of Pitres' respiratory exercises effected a complete cure.
An evolution such as the above may be considered more or less typical of
the great majority of tics.
We have seen that the tic may be localised indefinitely in one and the
same muscle or muscular group, but its site may also vary from day to
day. Two tics may co-exist and coincide, or a third may appear with the
disappearance of the others. Unexpected resurrections may succeed
periods of complete repose.
Tic always shows a tendency to invade; regarded as a functional act, it
moves in the direction of greater complexity.
After involving the orbicularis, for instance, a tic will spread to
neighbouring groups, in particular to those muscles whose synergic
contractions form a special expression of countenance. That is why
tics of the eyelids are associated with movements of the
pyramidales, frontales, and corrugators. Tics of the lips or of the
alae nasi very commonly extend to the corresponding elevators. It
is not surprising that muscular groups accustomed to act in
physiological unison should also be affected together (Noir).
Moreover, the fecund imagination of the victim to tic is calculated to
facilitate the invention of all sorts of modifications, complications,
parodies, and caricatures of the functional acts on which his tics are
grafted.
Tics are constantly varying in amplitude, degree, and frequency; as O.
remarked spontaneously, "We have our good days, and we have our _mauvais
quarts d'heure_." The sedative effect of rest, solitude, silence, and
obscurity may be contrasted with the detrimental results of fatigue,
noise, fear of ridicule, etc.
However incapable S. is of rotating his head to the right when
requested to do so, the movement is executed with the utmost
readiness should his attention be drawn in that direction. But if
he hesitates, even momentarily, before looking round, he cannot
then do so without the preliminary performance of all sorts of
contortions, ending in a twist of his body through a half circle to
the right. Sometimes he actually turns round two or three times,
after the fashion of a dog chasing its tail. Let him have a
pleasant visit, on the other hand, let him engage in a discussion,
or be engrossed in a play, let him administer a rebuke to some one,
and immediately his trouble is forgotten, his speech is accompanied
with animated gestures, the vicious position of his head
vanishes--in short, he becomes normal.
An intercurrent affection may act either as a deterrent or as a
stimulus; with convalescence, however, there is usually a
re-establishment of the mischief. The most potent influence over the
phenomena of tic is wielded by a sense of well-being, to employ Janet's
discriminating expression. Well-being is a panacea for the _tiqueur_ no
less than for the hysteric. The tic of the worried financier disappears,
as we have had occasion to note, under the magic of a rise in stocks or
a knowledge of solvency. The child's happiness is bound up in his
freedom, which explains the cessation, in Tissié's little patient, of
all convulsive movements during the holidays.
Much evidence is forthcoming to support these points, but we must admit
that the why and wherefore of a tic's amelioration or aggravation often
escape us, nor must we forget that both in the child and the adult
spontaneous cure is not unknown.
As has been remarked, the evolution of tic does not lend itself to
systematic description, but there are cases that form an exception,
their course being regularly progressive. Strictly speaking, they are
instances of Gilles de la Tourette's disease.
GILLES DE LA TOURETTE'S DISEASE
Under the title, "Study of a nervous affection characterised by motor
inco-ordination, and accompanied with echolalia and coprolalia,"
Tourette[131] grouped together, in 1885, a certain number of cases
presenting features in common and so enabling him to describe a morbid
entity, specially remarkable for its progressive evolution. He was
followed in the same line by Guinon, who supplied an account in
nosographical form, and since then the disease has figured in all the
text-books.
To obtain a schematic picture of the condition we shall borrow from
Tourette's[132] last communication on the subject:
About the age of seven or eight a little boy or girl--for the sexes
are affected equally--commonly with a wretched family history,
begins to exhibit a series of tics. The attention of the parents is
soon drawn to the fact, but they seldom give much heed at first,
since the twitches are limited preferably to the facial
musculature. At this stage, too, expiratory laryngeal noises are
occasionally superadded.
The movements may be confined for a long time to the face, but
under the influence of causes very difficult to determine they
gradually invade the shoulders and the arms. First one shoulder is
shrugged and then the other, then the trunk is inclined _en masse_
to right or left; then the patient waves his hands or his arms, or
bends backwards and forwards, or jumps up and down, flexing the
knees alternately and tapping with his feet. The muscles of the
larynx sometimes participate in the abnormal functioning, whence it
is that many sufferers from tic give vent to quick expiratory
"hems" and "ahs," which coincide often with the twitches of trunk
and limbs.
The disease may be limited to this stage, but it is not uncommon to
find, a few months or years after the beginning of the facial
movements, that the inarticulate laryngeal sound becomes organised
and develops in a particular direction, thus, in a sense, showing a
pathognomonic value. Under the influence of causes whose action we
are, in the majority of cases, powerless to appreciate, the patient
gives vent one day to a word or short phrase of a quite special
character, inasmuch as its meaning is always obscene. These words
and phrases are exclaimed in a loud voice, without any attempt at
restraint. There must be a complete absence of the moral sense
where there is coprolalia such as this; at the moment of the
ejaculation some irresistible psychical impulse must drive the
patient to utter filthy words unreservedly and with no
consideration for other people.
Another psychical stigma--echolalia--is occasionally, though less
frequently, observed in these cases.
Such, then, is Gilles de la Tourette's disease, a clinical type of which
many examples have been recorded. We do not think, however, that all
tics can be brought under the same category; we lose sight of its
distinguishing features if we make the attempt. Of course _fruste_ and
atypical cases are encountered, but even in them it is rare not to find
a certain degree of mental instability in dependence on which echolalia
and coprolalia rest, so completing the morbid syndrome, and it is
important to recognise the successive development of these various
constituents.
It is, indeed, this evolution of symptoms which is so characteristic of
Gilles de la Tourette's disease. A careful scrutiny of recorded cases of
tic, however, makes it abundantly clear that they do not all belong to
the disease of convulsive tics; their localisation, form, and progress
are so different that the effort to assimilate them to Tourette's
disease would abolish the nosographical value of the latter. One patient
may have an ocular tic all his life, and nothing else; the affection of
another may be limited to a tic of the shoulder and arm; a third blinks
and makes a facial grimace; a fourth is a coprolalic who has never
suffered from tic. Are they all to be considered incomplete cases of the
disease of convulsive tics? To answer in the affirmative is equivalent
to a failure to appreciate the distinctive characters of a judiciously
isolated syndrome, and a refusal to describe tics as they are met with
in everyday life. One questions, in fact, whether some of the cases
allotted to Tourette's disease really conform to it. Take an instance
from Chabbert[133]:
A woman, aged forty-two, had had an injury to the left side of her
face at the age of nine, as a result of which appeared a
convulsive facial tic, accompanied at times by hysterical attacks
which continued for eight years. The tic itself, an abrupt
contraction of the inferior portion of the left orbicularis
palpebrarum, underwent no subsequent change, in degree or extent.
At a later stage a fairly definite tendency to coprolalia became
manifest.
An unvarying post-traumatic palpebral tic in an hysterical subject
cannot be said to constitute the syndrome of Gilles de la Tourette, in
spite of the coprolalia.
In another of his cases the diagnosis is no less open to doubt:
The son of the previous patient was a youth of nineteen, with a bad
heredity on the father's side. In boyhood he had been a
somnambulist. Some months previously to his coming under
observation he developed a convulsive tic limited to the frontalis.
Stigmata of hysteria were present in dyschromatopsia, restriction
of the visual fields, and left hemihyperæsthesia.
A third case reported by the same author does probably belong to the
disease of convulsive tics:
A woman aged forty-four, of a strumous diathesis, exhibited tics of
face and limbs, occurring in the form of attacks sufficiently
violent to cause bruises, attacks which were invariably associated
with coprolalia. In addition, she suffered from echolalia,
echokinesis, and _folie du doute_.
We can only repeat, of course, that each type of tic passes by
insensible gradations into others that precede it or succeed it in the
hierarchy of tics; but we must, provisionally at least, neglect the
links that unite neighbouring groups if we are to avoid losing sight of
admittedly distinctive characters in too comprehensive summaries. It is
desirable to retain the term "disease of convulsive tics" for those
cases whose progressive evolution ends in the generalisation of the
convulsive movements, to the accompaniment of coprolalia and sometimes
of echolalia. This clinical form represents the most advanced degree
attained by the disease; it might be called the tic's apogee. From its
psychical aspect, moreover, the development it undergoes may culminate
in actual insanity.
* * * * *
According to the teaching of Magnan, the disease of convulsive tics does
not constitute an entity, since each and all of its symptoms may occur
separately as episodic syndromes of degeneration. The general
considerations with which we introduced our study are applicable in this
connection, and we shall be content to say with Noir:
We cannot deny the validity of the objections raised by Magnan and
his school; but the fact that these various symptoms may and do
most frequently occur singly is no reason for expunging the disease
of Gilles de la Tourette from the text-books. The combination of
these symptoms constitutes a clinical entity which has a specific
evolution, and while its subjects are degenerates in the sense of
Magnan and of Charcot, they may be ranged by themselves in a very
definite group.
In some cases which apparently come under this category, psychical
disturbance has not been a prominent feature.
Sciamanna[134] is the reporter of a case where a young man with
neuropathic antecedents was afflicted with tics involving various
muscular groups; his intellect, however, was normal, and the only
psychical change was an insignificant disorder of affectivity.
In such a case it would be instructive to know the mental condition
after the lapse of some years.
Two typical examples of Tourette's disease have been described by
Köster[135] as "disease of impulsive tics"; a third case--in which
widespread muscular twitches, the muscles of respiration and the
cremasters included, were coupled with sometimes a monotonous
intonation and sometimes a jerky speech, though psychical functions were
unimpaired--is considered by Kopczynski[136] to be a case of convulsive
tic, which he distinguishes from the "disease of convulsive tics."
A last instance, published by Innfeld[137] as a case of "chronic
progressive muscular spasm," is an unmistakable example of tic, in spite
of the author's declaration that it does not correspond to any known
morbid type and his attempt to liken it to chronic chorea. A boy of
fifteen exhibited convulsive movements which had begun in the facial
musculature and thence spread to the head, shoulders, and hands, and
were accompanied with respiratory noises and involuntary exclamations.
There was no alteration in sensation or in reflectivity, or in
electrical excitability. Sleep banished while emotion aggravated the
movements.
VARIABLE CHOREA OF BRISSAUD
If the disease of Gilles de la Tourette, by reason of the uniformity of
its symptomatology and the regularity of its evolution, justifies its
differentiation as a separate entity among the tics, a comparison of it
with another type, of polymorphic manifestation, irregular evolution,
and uncertain duration, may prove instructive. We refer to the affection
described by Brissaud as variable chorea.
The form of the motor reactions in this condition warrants the
application to it of the term chorea, but the analogies the disease
presents to tic are very close, nevertheless, and sometimes the two
occur in the same individual. Patients suffering from variable chorea
reveal the same mental abnormalities as are found among those who tic,
while the troubles of motility are sometimes so similar to what we meet
with in the latter that Gilles de la Tourette regarded the condition
simply as one form of convulsive tic, the more so that it is
occasionally accompanied by explosive utterance and even coprolalia.
This view, however, is calculated to obliterate the distinctive
characters of the two affections, and ought not to be entertained. We
cannot do better than repeat Brissaud's original description:
The use of the word chorea need occasion no ambiguity: the chorea
consists in the appearance of meaningless and apparently idiopathic
involuntary movements, whose repetition during rest and action
alike is proof of their irrationality and incongruity; the duration
of the symptoms may be limited as in chorea minor or Sydenham's
chorea, or unlimited as in chorea major or Huntington's chorea.
"Variable" is the epithet we apply to the chorea because of the
lack of uniformity in its exteriorisation, the irregularity of its
development, and the inconstancy of its duration. It comes and
goes, waxes and wanes, vanishes abruptly to reappear unexpectedly;
it is a neurosis without a characteristic march.
Notwithstanding the fact that we are dealing with a chorea--that is
to say, with a disease which is almost as readily recognisable by
the public as by any professional--the difficulty of fixing its
onset is paralleled by the difficulty of knowing when it has
ceased. This uncertainty is explained by the facile and changeable
nature of the patient; until the condition is revealed by
unmistakable signs it passes for an insignificant muscular caprice
of no pathological importance, while its disappearance is not
associated with any particular modification of the patient's ways.
There is a natural tendency to identify all "nervous movements"
with myoclonus, but the conception is a remarkably nebulous one,
and means nothing more than "muscular twitch." On the other hand,
it is well understood that "nervous movements" are more or less
sudden movements of limbs, shoulders, face, always involuntary and
generally increasing in force and frequency with the nervous state
of the patient.
Parents say, for instance, that their child has become more
restless and irascible, and at the same time that he has had "more
movements of the nerves." The coincidence is unfailing. Is the
expression "nervous movement" lacking in precision? Yet it
signifies what it is intended to signify. We are concerned neither
with tonic convulsions nor with clonic spasms, nor yet with tics of
habit; what the term stands for is a complex contraction, brisk but
not violent, closely allied to the simplest of automatic acts, such
as a step in advance, a shrug of the shoulders, a frown, a sigh, a
moan, a crack of the fingers, an exclamation--in any case usually a
gesture of impatience. The whole thing, however, is so variable and
fugitive, that it cannot be said to constitute a definite
convulsive phenomenon. The contractions, further, in spite of their
complexity, escape the notice of their originator, who is quite
surprised at being asked the meaning of the movement he has just
made, as he is almost entirely ignorant of it.
Briefly, the "nervous movements" of which we have been speaking do
not belong either to myoclonus or to tic, but owe their
distinctiveness to their multiplicity and inconstancy. At the same
time they are always grafted on a certain neuropathic diathesis
akin to that of chorea; in fact, they are nought else than a form
of chorea themselves.
The psychical peculiarities of the patient with variable chorea may be
summed up in instability of thought and action, combined with mental
infantilism. Hence the terms "polymorphous chorea" and "chorea of
degenerates" are used synonymously for variable chorea.[138] Sometimes
the disorders of the mind include hallucinations, and various forms of
phobia or mania.
One or two examples may be given:
A microcephalic youth of sixteen, a monorchid, developed what
appeared at first to be an ordinary chorea subsequently to an
orchidopexy. The movements, however, varied from day to day and
from hour to hour. Sometimes they disappeared for days at a time,
to reappear suddenly just when the neurosis seemed cured. The
influence exerted on them by the will was both mild and transient.
They constituted, in short, a particular kind of chorea, changing
and changeable, and differing from intermittent chorea in that
neither remissions nor relapses were ever wholly complete. Further,
the condition was implanted on a basis of mental and physical
degeneration, and seemed likely to become established as a
permanent functional stigma.
In another case a peculiar chorea gradually supervened, for no
obvious reason, in an adult female of tardy and imperfect physical
and intellectual development. It was difficult to decide whether
the psychical or the somatic phenomena were preponderant; but to
the material, tangible, and visible signs of constitutional
inferiority was superadded a choreiform instability of the whole
voluntary muscular system, consisting in agitation, gesticulation,
and incorrigible motor restlessness, coupled with a conspicuous
incapacity for rational action.
The steps in the evolution of this functional defect were very
slow, and coincided with final confirmation of the intellectual
insufficiency. As for the chorea, its localisation and its
intensity, its increase and its decrease, its extension and its
limitation, seemed to vary, in a way that could not be foreseen, at
the call of certain undetermined circumstances.
In a third instance we meet with many of the symptoms already noted
among those who tic:
X. is a well-developed boy of fifteen, but there is something
peculiar about his physiognomy which defies analysis. If his
mother's statements can be trusted, he is intelligent, quick,
witty, sound in judgment, and blessed with an excellent memory.
From the very first he has been eccentric, timid, and
hypersensitive, and is to-day as tender-hearted and affectionate to
his people as ever. He has various little "manias" of his own; he
must have a knife, fork, and spoon for himself, and cannot take his
food in comfort if they have been set before some one else. Each
morning he dresses himself with extreme deliberation, then comes
down to breakfast, of which, however, he will not partake unless he
has touched all the door handles on his way. This little matter has
developed into an obsession. His loathing of cold water is so
pronounced that his morning toilet is rather a stormy proceeding,
and as he is too old to be washed by his mother, the inevitable
result is that his face and hands are never clean. At school he is
both attentive and docile, finding pleasure in his study of the
classics, but evincing a perfect passion for German. Anything
German is a source of ineffable joy, so much so that he hugs his
dictionary with childish exuberance. He listens deferentially to
his teachers, but takes no note of what he hears. In German, Greek,
and Latin he is at the head of his class, whereas in history and
mathematics he is at the foot.
The "nervous movements" for which he has been brought to the
consulting-room consist of a series of gesticulations akin both to
tic and to chorea. Some are much more frequent than others,
meaningless gestures executed spontaneously, one might almost say
unconsciously. As he walks to school with his books under his left
arm, his right hand roams over his person; and in the class-room
the movements are repeated. At table he rubs his back against the
chair, and alternately flexes and extends his right leg. Apart from
these "habit tics," he exhibits actual twitches of his muscles
generally, and evidence of the consequent disturbance of his
movements is furnished by a glance at his untidy bedroom, his
disarranged books, his blotted papers, his slovenly clothes. When
he goes out with his parents, he is never at their side, but
lounges along in his own way, then suddenly hurries to regain his
place by them, falling back again and occupying himself by crossing
his legs, knocking his ankles together, shrugging his shoulders,
grimacing, etc. All the movements can be arrested for a time by an
effort of the will. At any one's behest he can maintain
tranquillity for a minute, but the strain is too severe, and the
muscular dance recommences sooner or later.
The movements are highly variable in type and degree, nor can the
mother specify the date of their appearance. It is only during the
last three years that her attention has been more particularly
drawn to them, and their increasing gravity occasions her some
anxiety. The boy has become the laughing-stock of his companions at
school, hence he limits his stay there to the actual hours of his
classes.
Three years later the choreic symptoms vanished. X. is to-day a
stalwart youth, though still timid and eccentric. It is evident
that in his case the variable chorea has been but an episode in
adolescence, to be added to the numerous stigmata of degeneration
enumerated above.
Notwithstanding its slow evolution (says Brissaud), the neurosis,
in so far as it was a disorder of motility, seems to have
completely disappeared. The importance of this for prognosis is
fundamental, but from the point of view of diagnosis it is no less
significant, seeing that the nature and form of the movements
suggested chronic or Huntington's chorea.
A case described by Gilles de la Tourette[139] as disease of the tics
seems really to have been one of variable chorea.
A woman of twenty-two, who had never been very strong, had an
attack, at eight years, of involuntary movements of face and arms
which prevented her feeding herself, and at the hospital a
diagnosis of chorea was made. Two months later cessation of the
movements allowed of her return to school, but a second attack
followed after two years, and a third a year later. At the time of
observation she was in the throes of her sixth relapse. Every one
who had seen her considered the condition as chorea.
Tourette, however, was dissatisfied with the diagnosis. There was no
suggestion of its being Sydenham's chorea, or hysterical chorea, still
less of its belonging to Huntington's variety. According to the author,
the muscular twitches were amorphous and indefinite, and characterised
by extreme variability in form, expression, and intensity.
In our opinion the clinical picture is that of variable chorea, and we
are confirmed in our opinion by a consideration of the patient's mental
condition.
She comes of a pronounced neuropathic stock. One of her two sisters
is nervous and impressionable, and probably a neurasthenic, while
the other is subject to hysterical attacks. She herself is of a
profoundly nervous temperament; she cannot go to bed without
assuring herself several times that no one is concealed beneath it;
she suffers from fears and dreads and obsessions of all sorts; she
is, in fact, an "unstable," a degenerate.
In one of our patients the symptoms were unilateral, constituting a
variable hemichorea.
It is a matter of some difficulty to furnish an adequate
description of the movements of the right arm. We note, first of
all, that their activity depends on whether the arm is free or held
in a fixed position. Voluntary movements are carried out stiffly,
but are interrupted by sudden deviations, sometimes of rather a
wide range, and highly irregular in distribution. Notwithstanding
these breaks, the end to which the movement is directed is always
attained with precision.
While L. was an apprentice dressmaker, she occasionally used to
make various contortions with her arm, though if her attention was
diverted they did not occur, and as a matter of fact she did her
work well enough. Once she became familiar with the mechanical act
of sewing, the involuntary performances ceased. Before her disease
asserted itself, she had commenced to learn the piano, and she
continued to make unimpeded progress, as her teacher discovered a
method of holding her elbow which checked all convulsive twitches.
The involuntary movements of the right leg were so insignificant as
to be almost negligible; they united to produce a sort of irregular
tremor which became appreciable only when the patient was very
tired or very annoyed. Sometimes a long walk was followed by a
certain hesitation in putting the right foot to the ground, and by
defective inhibition of the antagonists of the desired movement.
Sometimes one foot was knocked against the other, and sometimes the
right appeared to assume an equinovarus position. On the other
hand, we have seen L. walking in the street with her father, when
no anomaly could be detected in her gait. The distraction of any
occupation such as dancing or playing a game has the effect, for
the time being, of banishing the greater part, if not all, of the
spasmodic phenomena.
This is undoubtedly a case of Brissaud's variable chorea of a unilateral
type, and a consideration of the symptoms confirms the intimate
relationship between it and tic.
Various intermediate forms have been noted. In one of Brissaud's cases,
variable chorea and multiple tics co-existed. Féré[140] reports a case
of variable chorea preceded by tic, and Bernard another in which
starting, trembling, facial tic, variable chorea, etc., were associated.
Tics of phonation are often superadded to the gesticulations of variable
chorea. Brissaud refers to the case of a girl of sixteen in whom
involuntary movements resembling those of this type of chorea were
coincident with a sort of hiccough, and a more or less inarticulate cry;
at a later stage the movements became very infrequent, the hiccough was
more constant, and the cry developed into a coprolalic ejaculation.
Variable chorea and variable tic are obviously very closely allied. The
movements of the latter, however, are distinguished by their greater
abruptness and smaller variety. They are tics by reason of their
systematisation and co-ordination; they are variable because they pass
from one region of the body to another. There is no necessary relation
between them; each has an individuality of its own and is independent of
the rest. In variable chorea, on the other hand, one movement passes
insensibly into another, and the variants of any particular one are
legion.
However easy it is, then, to separate the two clinically, it is none the
less true that they spring from the same soil of mental defect. Variable
chorea differs in nature from other choreas, though its form is the
same; it may be distinguished from tic by the type of movement, but in
essence it is identical.