The man who mistook his wife for a hat (14 page)

   'Dangerous wellness', 'morbid brilliance', a deceptive euphoria with abysses beneath-
this
is the trap promised and threatened by

   excess, whether it be set by Nature, in the form of some intoxicating disorder, or by ourselves, in the form of some excitant addiction.

   The human dilemmas, in such situations, are of an extraordinary kind: for patients are here faced with disease as seduction, something remote from, and far more equivocal than, the traditional theme of illness as suffering or affliction. And nobody, absolutely nobody, is exempt from such bizarrenesses, such indignities. In disorders of excess there may be a sort of collusion, in which the self is more and more aligned and identified with its sickness, so that finally it seems to lose all independent existence, and be nothing but a product of sickness. This
fear
is expressed by Witty Ticcy Ray in Chapter Ten when he says: 'I consist of tics-there is nothing else', or when he envisages a mind-growth- a 'Tourettoma'-which might engulf him. For him, with his strong ego, and relatively mild Tourette's syndrome, there was not, in reality, any such danger. But for patients with weak or undeveloped egos, coupled with overwhelmingly strong disease, there is a very real risk of such 'possession' or 'dispossession'. I do no more than touch on this in 'The Possessed'.

   
10

   
Witty Ticcy Ray

   In 1885 Gilles de la Tourette, a pupil of Charcot, described the astonishing syndrome which now bears his name. Tourette's syndrome', as it was immediately dubbed, is characterised by an excess of nervous energy, and a great production and extravagance of strange motions and notions: tics, jerks, mannerisms, grimaces, noises, curses, involuntary imitations and compulsions of all sorts, with an odd elfin humour and a tendency to antic and outlandish kinds of play. In its 'highest' forms, Tourette's syndrome involves every aspect of the affective, the instinctual and the imaginative life; in its 'lower', and perhaps commoner, forms, there may be little more than abnormal movements and impulsivity, though even here there is an element of strangeness. It was well recognised and extensively reported in the closing years of the last century, for these were years of a spacious neurology which did not hesitate to conjoin the organic and the psychic. It was clear to Tourette, and his peers, that this syndrome was a sort of possession by primitive impulses and urges: but also that it was a possession with an organic basis-a very definite (if undiscovered) neurological disorder.

   In the years that immediately followed the publication of Tourette's original papers many hundreds of cases of this syndrome were described-no two cases ever being quite the same. It became clear that there were forms which were mild and benign, and others of quite terrible grotesqueness and violence. Equally, it was clear that some people could 'take' Tourette's, and accommodate it within a commodious personality, even gaining advantage from the swiftness of thought and association and invention which went with it,

   while others might indeed be 'possessed' and scarcely able to achieve real identity amid the tremendous pressure and chaos of Tourettic impulses. There was always, as Luria remarked of his mnemonist, a fight between an 'It' and an T.

   Charcot and his pupils, who included Freud and Babinski as well as Tourette, were among the last of their profession with a combined vision of body and soul, 'It', and T, neurology and psychiatry. By the turn of the century, a split had occurred, into a soulless neurology and a bodiless psychology, and with this any understanding of Tourette's disappeared. In fact, Tourette's syndrome itself seemed to have disappeared, and was scarcely at all reported in the first half of this century. Some physicians, indeed, regarded it as 'mythical', a product of Tourette's colourful imagination; most had never heard of it. It was as forgotten as the great sleepy-sickness epidemic of the 1920s.

   The forgetting of sleepy-sickness
(encephalitis lethargica)
and the forgetting of Tourette's have much in common. Both disorders were extraordinary, and strange beyond belief-at least, the beliefs of a contracted medicine. They could not be accommodated in the conventional frameworks of medicine, and therefore they were forgotten and mysteriously 'disappeared'. But there is a much more intimate connection, which was hinted at in the 1920s, in the hyperkinetic or frenzied forms which the sleepy-sickness sometimes took: these patients tended, at the beginning of their illness, to show a mounting excitement of mind and body, violent movements, tics, compulsions of all kinds. Some time afterwards, they were overtaken by an opposite fate, an all-enveloping trance-like 'sleep'-in which I found them forty years later.

   In 1969, I gave these sleepy-sickness or post-encephalitic patients L-Dopa, a precursor of the transmitter dopamine, which was greatly lowered in their brains. They were transformed by it. First they were 'awakened' from stupor to health: then they were driven towards the other pole-of tics and frenzy. This was my first experience of Tourette-like syndromes: wild excitements, violent impulses, often combined with a weird, antic humour. I started to speak of 'Tourettism', although I had never seen a patient with Tourette's.

   Early in 1971, the
Washington Post,
which had taken an interest in the 'awakening' of my post-encephalitic patients, asked me how they were getting on. I replied, 'They are ticcing', which prompted them to publish an article on 'Tics'. After the publication of this article, I received countless letters, the majority of which I passed on to my colleagues. But there was one patient I did consent to see-Ray.

   The day after I saw Ray, it seemed to me that I noticed three Touretters in the street in downtown New York. I was confounded, for Tourette's syndrome was said to be excessively rare. It had an incidence, I had read, of one in a million, yet I had apparently seen three examples in an hour. I was thrown into a turmoil of bewilderment and wonder: was it possible that I had been overlooking this all the time, either not seeing such patients or vaguely dismissing them as 'nervous', 'cracked', 'twitchy'? Was it possible that everyone had been overlooking them? Was it possible that Tourette's was not a rarity, but rather common-a thousand times more common, say, than previously supposed? The next day, without specially looking, I saw another two in the street. At this point I conceived a whimsical fantasy or private joke: suppose (I said to myself) that Tourette's is very common but fails to be recognised but once recognised is easily and constantly seen.* Suppose one such Touretter recognises another, and these two a third, and these three a fourth, until, by incrementing recognition, a whole band of them is found: brothers and sisters in pathology, a new species in our midst, joined together by mutual recognition and concern? Could there not come together, by such spontaneous aggregation, a whole association of New Yorkers with Tourette's?

   Three years later, in 1974, I found that my fantasy had become a reality: that there had indeed come into being a Tourette's Syndrome Association. It had fifty members then: now, seven years

   ( A very similar situation happened with muscular dystrophy, which was never seen until Duchenne described it in the 1850s. By 1860, after his original description, many hundreds of cases had been recognised and described, so much so that Charcot said: 'How come that a disease so common, so widespread, and so recognisable at a glance- a disease which has doubtless always existed-how come that it is only recognised now? Why did we need M. Duchenne to open our eyes?'

   later, it has a few thousand. This astounding increase must be ascribed to the efforts of the TSA itself, even though it consists only of patients, their relatives and physicians. The association has been endlessly resourceful in its attempts to make known (or, in the best sense, 'publicise') the Touretter's plight. It has aroused responsible interest and concern in place of the repugnance, or dismissal, which had so often been the Touretter's lot, and it has encouraged research of all kinds, from the physiological to the sociological: research into the biochemistry of the Tourettic brain; on genetic and other factors which may co-determine Tourette's; on the abnormally rapid and indiscriminate associations and reactions which characterise it. Instinctual and behavioural structures, of a developmentally and even phylogenetically primitive kind, have been revealed. There has been research on the body-language and grammar and linguistic structure of tics; there have been unexpected insights into the nature of cursing and joking (which are also characteristic of some other neurological disorders); and, not least, there have been studies of the interaction of Touretters with their family and others, and of the strange mishaps which may attend these relationships. The TSA's remarkably successful endeavours are an integral part of the history of Tourette's, and, as such, unprecedented: never before have patients led the way to understanding, become the active and enterprising agents of their own comprehension and cure.

   What has emerged in these last ten years-largely under the aegis and stimulus of the TSA-is a clear confirmation of Gilles de la Tourette's intuition that this syndrome indeed has an organic neurological basis. The 'It' in Tourette's, like the 'It' in Parkinsonism and chorea, reflects what Pavlov called 'the blind force of the subcortex', a disturbance of those primitive parts of the brain which govern 'go' and 'drive'. In Parkinsonism, which affects motion but not action as such, the disturbance lies in the midbrain and its connections. In chorea-which is a chaos of fragmentary quasi-actions-the disorder lies in higher levels of the basal ganglia. In Tourette's, where there is excitement of the emotions and the passions, a disorder of the primal, instinctual bases of behaviour, the disturbance seems to lie in the very highest parts of the

   'old brain': the thalamus, hypothalamus, limbic system and amygdala, where the basic affective and instinctual determinants of personality are lodged. Thus Tourette's-pathologically no less than clinically-constitutes a sort of 'missing link' between body and mind, and lies, so to speak, between chorea and mania. As in the rare, hyperkinetic forms of
encephalitis lethargica,
and in all post-encephalitic patients over-excited by L-Dopa, patients with Tourette's syndrome, or Tourettism' from any other cause (strokes, cerebral tumours, intoxications or infections), seem to have an excess of excitor transmitters in the brain, especially the transmitter dopamine. And as lethargic Parkinsonian patients need more dopamine to arouse them, as my post-encephalitic patients were 'awakened' by the dopamine-precursor L-Dopa, so frenetic and Tourettic patients must have had their dopamine lowered by a dopamine antagonist, such as the drug haloperidol (Haldol).

   On the other hand, there is not just a surfeit of dopamine in the Touretter's brain, as there is not just a deficiency of it in the Parkinsonian brain. There are also much subtler and more widespread changes, as one would expect in a disorder which may alter personality: there are countless subtle paths of abnormality which differ from patient to patient, and from day to day in any one patient. Haldol can be an answer to Tourette's, but neither it nor any other drug can be
the
answer, any more than L-Dopa is
the
answer to Parkinsonism. Complementary to any purely medicinal, or medical, approach there must also be an 'existential' approach: in particular, a sensitive understanding of action, art and play as being in essence healthy and free, and thus antagonistic to crude drives and impulsions, to 'the blind force of the subcortex' from which these patients suffer. The motionless Parkinsonian can sing and dance, and when he does so is completely free from his Parkinsonism; and when the galvanised Touretter sings, plays or acts, he in turn is completely liberated from his Tourette's. Here the T vanquishes and reigns over the 'It'.

   Between 1973 and his death in 1977, I enjoyed the privilege of corresponding with the great neuropsychologist A.R. Luria, and often sent him observations, and tapes, on Tourette's. In one of his last letters, he wrote to me: 'This is truly of a tremendous

   importance. Any understanding of such a syndrome must vastly broaden our understanding of human nature in general … I know of no other syndrome of comparable interest.'

   When I first saw Ray he was 24 years old, and almost incapacitated by multiple tics of extreme violence coming in volleys every few seconds. He had been subject to these since the age of four and severely stigmatised by the attention they aroused, though his high intelligence, his wit, his strength of character and sense of reality enabled him to pass successfully through school and college, and to be valued and loved by a few friends and his wife. Since leaving college, however, he had been fired from a dozen jobs-always because of tics, never for incompetence-was continually in crises of one sort and another, usually caused by his impatience, his pugnacity, and his coarse, brilliant 'chutzpah', and had found his marriage threatened by involuntary cries of 'Fuck!' 'Shit!', and so on, which would burst from him at times of sexual excitement. He was (like many Touretters) remarkably musical, and could scarcely have survived-emotionally or economically- had he not been a weekend jazz drummer of real virtuosity, famous for his sudden and wild extemporisations, which would arise from a tic or a compulsive hitting of a drum and would instantly be made the nucleus of a wild and wonderful improvisation, so that the 'sudden intruder' would be turned to brilliant advantage. His Tourette's was also of advantage in various games, especially ping-pong, at which he excelled, partly in consequence of his abnormal quickness of reflex and reaction, but especially, again, because of 'improvisations', 'very sudden, nervous,
frivolous
shots' (in his own words), which were so unexpected and startling as to be virtually unanswerable. The only time he was free from tics was in post-coital quiescence or in sleep; or when he swam or sang or worked, evenly and rhythmically, and found 'a kinetic melody', a play, which was tension-free, tic-free and free.