In 1884-5 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 base – 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 ‘I’.
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 ‘I’, 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 New York Times, 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 seeing 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 until it is recognised (and, thereafter, 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 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 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 ‘I’ 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 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 me!’ ‘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.
Under an ebullient, eruptive, clownish surface, he was a deeply serious man – and a man in despair. He had never heard of the TSA (which, indeed, scarcely existed at the time), nor had he heard of haldol. He had diagnosed himself as having Tourette’s after reading the article on ‘Tics’ in the New York Times. When I confirmed the diagnosis, and spoke of using haldol, he was excited but cautious. I made a test of haldol by injection, and he proved extraordinarily sensitive to it, becoming virtually tic-free for a period of two hours after I had administered no more than one-eighth of a milligram. After this auspicious trial, I started him on haldol, prescribing a dose of a quarter of a milligram three times a day.
He came back, the following week, with a black eye and a broken nose and said: ‘So much for your fucking haldol.’ Even this minute dose, he said, had thrown him off balance, interfered with his speed, his timing, his preternaturally quick reflexes. Like many Touretters, he was attracted to spinning things, and to revolving doors in particular, which he would dodge in and out of like lightning: he had lost this knack on the haldol, had mistimed his movements, and had been bashed on the nose. Further, many of his tics, far from disappearing, had simply become slow, and enormously extended: he might get ‘transfixed in mid-tic’, as he put it, and find himself in almost catatonic postures (Ferenczi once called catatonia the opposite of tics – and suggested these be called ‘cataclonia’). He presented a picture, even on this minute dose, of marked Parkinsonism, dystonia, catatonia and psychomotor ‘block’: a reaction which seemed inauspicious in the extreme, suggesting, not insensitivity, but such over-sensitivity, such pathological sensitivity, that perhaps he could only be thrown from one extreme to another – from acceleration and Tourettism to catatonia and Parkinsonism, with no possibility of any happy medium.
He was understandably discouraged by this experience – and this thought – and also by another thought which he now expressed. ‘Suppose you could take away the tics,’ he said. ‘What would be left? I consist of tics – there’d be nothing left.’ He seemed, at least jokingly, to have little sense of his identity except as a ticqueur: he called himself ‘the ticcer of Little Neck Parkway’, and spoke of himself, in the third person, as ‘witty ticcy Ray’, adding that he was so prone to ‘ticcy witticisms and witty ticcicisms’ that he scarcely knew whether it was a gift or a curse. He said he could not imagine life without Tourette’s, nor was he sure he would care for it.
I was strongly reminded, at this point, of what I had encountered in some of my post-encephalitic patients, who were inordinately sensitive to L-DOPA. I had nevertheless observed in their case that such extreme physiological sensitivities and instabilities might be transcended if it were possible for the patient to lead a rich and full life: that the ‘existential’ balance, or poise, of such a life might overcome a severe physiological imbalance. Feeling that Ray also had such possibilities in him, that, despite his own words, he was not incorrigibly centred on his own disease, in an exhibitionistic or narcissistic way, as is the case, alas, with a number of severe Touretters, who become ‘professional patients’ and lacerated, demonic exhibitionists of their disease, I suggested that we meet weekly for a period of three months. During this time we would try to imagine life without Tourette’s; we would explore (if only in thought and feeling) how much life could offer, could offer him, without the perverse attractions and attentions of Tourette’s; we would examine the role and economic importance of Tourette’s to him, and how he might get on without these. We would explore all this for three months – and then make another trial of haldol.
There followed three months of deep and patient exploration, in which (often against much resistance and spite and lack of faith in self and life) all sorts of healthy and human potentials came to light: potentials which had somehow survived twenty years of severe Tourette’s and ‘Touretty’ life, hidden in the deepest and strongest core of the personality. This deep exploration was exciting and encouraging in itself and gave us, at least, a limited hope. What in fact happened exceeded all our expectations and showed itself to be no mere flash in the pan, but an enduring and permanent transformation of reactivity. For when I again tried Ray on haldol, in the same minute dose as before, he now found himself tic-free, but without significant ill-effects – and he has remained this way for the past nine years.
The effects of haldol, here, were ‘miraculous’ – but only became so when a miracle was allowed. Its initial effects were close to catastrophic: partly, no doubt, on a physiological basis; but also because any ‘cure’, or relinquishing of Tourette’s, at this time would have been premature and economically impossible. Having had Tourette’s since the age of four, Ray had no experience of any normal life: he was heavily dependent on his exotic disease and, not unnaturally, employed and exploited it in various ways. He had not been ready to give up his Tourette’s and (I cannot help thinking) might never have been ready without those three months of intense preparation, of tremendously hard and concentrated deep analysis and thought.
The past nine years, on the whole, have been happy ones for Ray – a liberation beyond any possible expectation. After twenty years of being confined by Tourette’s, and compelled to this and that by its crude physiology, he enjoys a spaciousness and freedom he would never have thought possible (or, at most, during our analysis, only theoretically possible). His marriage is tender and stable – and he is now a father as well; he has many good friends, who love and value him as a person – and not simply as an accomplished Tourettic clown; he plays an important part in his local community; and he holds a responsible position in ‘the City’. Yet problems remain: problems perhaps inseparable from having Tourette’s – and haldol.
During his working hours, and working week, on Wall Street, Ray remains ‘sober, solid, square’ on haldol – this is how he describes his ‘haldol self’. He is slow and deliberate in his movements and judgments, with none of the impatience, the impetuosity, he showed before haldol, but equally, none of the wild improvisations and inspirations. Even his dreams are different in quality: ‘straight wish-fulfilment,’ he says, ‘with none of the elaborations, the extravaganzas, of Tourette’s’. He is less sharp, less quick in repartee, no longer bubbling with witty tics or ticcy wit. He no longer enjoys or excels at ping-pong or other games; he no longer feels ‘that urgent killer instinct, the instinct to win, to beat the other man’; he is less competitive, then, and also less playful; and he has lost the impulse, or the knack, of sudden ‘frivolous’ moves which take everyone by surprise. He has lost his obscenities, his coarse chutzpah, his spunk. He has come to feel, increasingly, that something is missing.
Most important, and disabling, because this was vital for him – as a means both of support and self-expression – he found that on haldol he was musically ‘dull’, average, competent, but lacking energy, enthusiasm, extravagance and joy. He no longer had tics or compulsive hitting of the drums – but he no longer had wild and creative surges.
As this pattern became clear to him, and after discussing it with me, Ray made a momentous decision: he would take haldol ‘dutifully’ throughout the working week, but would take himself off it, and ‘let fly’, at weekends. This he has done for the past three years. So now, there are two Rays – on and off haldol. There is the sober citizen, the calm deliberator, from Monday to Friday; and there is ‘witty ticcy Ray’, frivolous, frenetic, inspired, at weekends. It is a strange situation, as Ray is the first to admit:
Having Tourette’s is wild, like being drunk all the while. Being on haldol is dull, makes one square and sober, and neither state is really free ... You ‘normals’, who have the right transmitters in the right places at the right times in your brains, have all feelings, all styles, available all the time – gravity, levity, whatever is appropriate. We Touretters don’t: we are forced into levity by our Tourette’s and forced into gravity when we take haldol. You are free, you have a natural balance: we must make the best of an artificial balance.
Ray does make the best of it, and has a full life, despite Tourette’s, despite haldol, despite the ‘unfreedom’ and the ‘artifice’, despite being deprived of that birthright of natural freedom which most of us enjoy. But he has been taught by his sickness and, in a way, he has transcended it. He would say, with Nietzsche: ‘I have traversed many kinds of health, and keep traversing them ... And as for sickness: are we not almost tempted to ask whether we could get along without it? Only great pain is the ultimate liberator of the spirit.’ Paradoxically, Ray – deprived of natural, animal physiological health – has found a new health, a new freedom, through the vicissitudes he is subject to. He has achieved what Nietzsche liked to call ‘The Great Health’ – rare humour, valour, and resilience of spirit: despite being, or because he is, afflicted with Tourette’s.