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Gilles de la Tourette's syndrome

Tourette syndrome — also called Tourette's syndrome, Tourette's disorder, or Gilles de la Tourette syndrome — is a neurological or neurochemical disorder characterized by tics: involuntary, rapid, sudden movements or vocalizations that occur repeatedly in the same way. more...

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The eponym was bestowed by Jean-Martin Charcot after and on behalf of his resident, Georges Gilles de la Tourette, (1859 - 1904), French physician and neurologist.


Symptoms include multiple motor and one or more vocal tics present at some time during the disorder although not necessarily simultaneously; the occurrence of tics many times a day (usually in bouts) nearly every day or intermittently throughout a span of more than one year; the periodic change in the number, frequency, type and location of the tics, and in the waxing and waning of their severity; symptoms disappearing for weeks or months at a time; and onset before the age of 18.

Vocal tics may fall into various categories, including echolalia (the urge to repeat words spoken by someone else after being heard by the person with the disorder), palilalia (the urge to repeat one's own previously spoken words), lexilalia (the urge to repeat words after reading them) and, most controversially, coprolalia (the spontaneous utterance of socially objectionable or taboo words or phrases, such as obscenities and racial or ethnic slurs). However, according to the Tourette Syndrome Association, Inc., only about 10% of TS patients suffer from this aspect of the condition. There are many other vocal tics besides those categorized by word repetition: in fact, a TS tic can be almost any possible short vocalization, with common vocal tics being throat clearing, coughing, sniffing, grunts, or moans. Motor tics can be of an endless variety and may include hand-clapping, neck stretching, shoulder shrugging, eye blinking, and facial grimacing.

The term "involuntary" has been used to describe TS tics, since it is known that most people with TS do have limited control over the expression of symptoms. Immediately preceding tic onset, individuals with TS experience what is called a "premonitory urge," similar to the feeling that precedes yawning. The control which can be exerted (from seconds to hours at a time) may merely postpone and exacerbate the ultimate expression of the tic. Children may be less aware of the premonitory urge associated with tics than are adults, but their awareness tends to increase with maturity. Tics are experienced as irresistible (like a yawn or sneeze or itch) and must eventually be expressed. People with TS often seek a secluded spot to release their symptoms after delaying them in school or at work. It is not uncommon for children to suppress tics during a visit to the doctor or while at school. Typically, tics increase as a result of tension or stress (but are not solely caused by stress) and decrease with relaxation or concentration on an absorbing task. In fact, neurologist and writer Oliver Sacks has described a man with severe TS who is both a pilot and a surgeon.


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Exorcising a damnable disease - Tourette syndrome
From Discover, 12/1/86 by Glenn Garelik

In the beginning it wasn't the words: it was the tics. It was the blinking and the squeezing of his eyelids, the spasmodic brushing of his pants with his fingertips. Then it was the twitching of his nose and the raising of his brows; the jaw-dropping, the teeth-grinding, and the grimacing.

It was the wrenching, joint-snapping jerks of his head that he couldn't help. It was the violent shrugging of his shoulders, the flinging of his arms, the kicking and the sudden stamping; the convulsive contracting of his belly, the arching of his back, and the plucking at his privates.

It wasn't the words, in the beginning, but the noises -- the snorting, the sniffing, and the grunting; the throat-clearing, the hissing, the barking; the humming and panting and gurgling; the rasping and burping and braying; the clicking and yipping and yelping. It was the lip- smacking. It was the ear-splitting shrieks.

Only later came the words. They were obscene eruptions, and they came -- fuck, WHORE! -- explosively. When they came, they came -- kike, WOP! -- in the course of ordinary talk, senseless ejaculations of ethnic epithets.

And all along there were the learning problems: the short attention span, the hyperactivity, the disruptiveness in school, the obsessiveness, the compulsions.

The affliction that racks Marshall Wolfe, 32, is a severe case of Gilles de la Tourette syn- drome. It's a mysterious and humiliating disorder, only recently recognized as organic, named for the French physician who first defined it, in 1885, in, among others, a woman who was so mortified by her paroxysms of gestures and invective that for almost 70 years she had isolated herself from social contact. Just as Tourette syndrome crippled her with shame and turned her days to wretchedness, it threatened to do the same to Wolfe, who lived until he was 25 without so much as knowing that there was a name for what he had.

His symptoms began when he was seven -- ''brushing my pants, shaking my head, snorting till my nose bled, jerking my hands till the teacher would actually have to hold them down.'' In Wolfe's case, as with most people who have Tourette, the affliction first appeared as a single, simple tic -- the term doctors use to describe rapid, senseless, involuntary contractions of muscle groups. Taken singly, the tics of Tourette sufferers are no different from the ''transient,'' or ''habit,'' or ''nervous'' tics that a quarter of all children experience at one time or another.

But the tics don't come singly in Tourette, which may afflict as many as 100,000 people in the U.S. (out of a total of perhaps 3.5 million with chronic tic disorders). The first sign of the syndrome, which in nearly 95 per cent of cases arises by age eleven, is often something as simple as an exaggerated blink. But it isn't long before other parts of the body become involved. A cheek twitches. A shoulder. A knee. A leg. Then the ''vocal tics'' -- the noises -- may begin, similarly abrupt, involuntary, and inappropriate.

Once the tics start, their number, type, and intensity wax and wane. One tic may plague a person for months, even years, only to be suddenly replaced by a new one. The tics may increase with anxiety, or in periods of solitude or stress. Sometimes they can be briefly inhibited by force of will; more often they subside on their own when the person is absorbed in activity, preoccupied or concentrating, playing music or acting, or in the company of strangers. It isn't clear why, but the tics seem to disappear during sleep and orgasm.

One of the most vexing symptoms, and perhaps the most embarrassing, is coprola lia, a vocal tic that consists of the uncontrollable uttering of obscenity -- more broadly, of anything vulgar, blasphemous, or socially unacceptable -- and sometimes a compulsion to make obscene gestures and touch one's genitals or those of others. Sometimes people with Tourette echo, without mockery, the words or gestures of the person they're with, or they repeat their own words, endlessly -- endlessly endlessly endlessly endlessly.

Not everyone with Tourette has all of these symptoms -- for example, only slightly more than half ever suffer from cop rolalia -- but those who don't manifest one symptom or another can't be sure they won't get it some day. Tourette disappears spontaneously in a small number of cases, but for most victims, it's a lifelong affliction -- though it's neither life- threatening nor degenerative.

Medical researchers are beginning to understand that Tourette syndrome, like many other conditions once thought to be psychological, is neurochemical in origin -- and that in fact it has no emotional component at all. Indeed, some researchers feel that in its peculiar way, Tourette -- lying, it would appear, at the intersection of instinct and intellect -- has much to tell about things as basic to the human species as language.

Though its causes may be purely physical, the condition's effects can be devastating socially, and, in turn, emotionally. It's not only that the tics, both nonvocal and vocal, often turn Tourette suffer- ers into outcasts; as children, many of them also have to live with the learning disabilities and obsessive-compulsive behavior that often accompany the syndrome: they read a sen- tence again and again, for example, or before going to bed they feel compelled to touch their toes three times or keep tracing the edge of the bathroom mirror.

The tics, as such, don't frighten the people who have them, nor are they painful, but many ''Touretters'' complain that their muscles ache as the result of the violent jerking and constant movement. Although the tics are involuntary, many sufferers are able to hold them at bay -- ''storing them up'' is what they call it -- for various lengths of time, and they complain of soreness from the struggle to suppress them.

Although most people with Tourette find their tics so unexpected and explosive as to be beyond control, one sufferer, Joseph Bliss, wrote a few years ago that in his case ''each movement is the result of a capitulation to a demanding and relentless urge, [like] he moment before a sneeze ex- plodes.'' Bliss hid his condition from even his children for 62 years by ''storing up'' -- forcing ''every thought and muscle to hold back the blinking of an eye,'' -- or by secluding himself when he felt a tic coming on.

Noises and words are rare among the ''nervous'' tics, says psychiatrist Arthur Shapiro, who, with his wife, Elaine, a psychologist, directs the Tourette and Tic Laboratory and Clinic of the Mount Sinai School of Medicine in New York. However, vocal tics -- whether or not they're articu- late speech -- are by definition part of Tourette. Beyond that, says Arthur Shapiro, ''when words are involved, they're almost always the unacceptable, intruding on conversation and activities like four-letter slaps in the face. But they don't have to be obscene. A child may say 'doodie,' if that's a bad word to him.

''A man I treated, who was anything but a racist, used to shout 'nigger!' whenever he passed through Harlem. It was appalling. But the exclamation carried no content, either intellectual or emotional. One boy I saw used to touch his mother's breasts or genitals and, occasionally, his father's. One day, at the age of four, while his mother cradled him in her arms, he looked up at her face and spat out 'Fuck!' She was struck dumb.''

''How does it feel?'' asks Orrin Palmer. Despite suffering from severe Tourette, Palmer, 29, graduated from New York Medical College a year ago. ''I feel like you would if there were a powerful man who had you by the throat and just kept tightening his grip. I end my days exhausted because of the energy I expend both ticquing and warding off the tics. In school, taking a test was utterly draining. I had to coordinate my muscles so I could write, and at the same time I had to think carefully to answer the questions. And that effort seemed to make the ticquing worse.''

For Wolfe, until recently, the effort to control his tics wasn't enough to permit him to live a normal life. Although today he has a family and a job, his odyssey from illness to diagnosis was a black comedy. As he talks about his affliction, his right leg shakes with nervous energy, and his quickness of thought is surprising. It's striking how well adjusted he seems: good-natured, optimis- tic, even boyish. Only once in a while does a look of exhaustion -- nothing so strong as ex- asperation -- cross his face.

At twelve, having become alienated from his father and afraid that his ''weirdness'' (his name for his condition at the time) had caused his stepmother to reject him, too, he ran away from home in Brooklyn. He bummed around the country until he was 16. ''I was a pretty hip little kid,'' Wolfe says, explaining how a boy his age could survive on the road.

At truckstops he met sympathetic drivers who would give him a lift and a meal; he found churches willing to provide runaways with shelter; he even temporarily joined a Hare Krishna group with the idea of getting good meals. In the South he worked in restaurants and in the fields alongside illegal aliens. ''I did so much apple picking, and picking, and picking, it could have been apple ticquing,'' he says.

When he was unable to get work, he fished and scavenged to feed himself. Twice he was picked up for vagrancy, detained, and sent home, where he still felt unwelcome. He took off again. Ultimately he decided to go back to his family anyway, but when he turned 17, his father insisted that Mar- shall join the service to get the discipline he thought his son needed. Wolfe enlisted in the Navy -- and wonders to this day how on earth he passed the physical.

Boot camp was misery: ''Ima gine this -- everybody's standing at attention, every helmet is still, and mine's moving a mile a minute. 'You spastic maggot!' my petty officer yells at me.''

Serving in Vietnam was a little easier, thanks to an understanding officer: ''He took me aside one day and said, 'Listen, I know what you're doing, and I'm really proud of you.' He thought I was an addict who was trying to withdraw on my own. When I saw that that made them leave me alone, I just let them go on thinking it.''

Like many of those with Tourette, Wolfe found that the only thing that would diminish his ticquing was alcohol. ''After leaving the service, with maybe a quart a day I'd slow down to about what you'd call normal,'' he says of the bourbon that sent him to the hospital with bleeding ulcers -- and more. Vomiting, which some doctors now think may have been caused by an internal tic, occurred day af- ter day, as many as 30 or 40 times a day, and by mid-fall of 1980, Wolfe had lost 90 of his 240 pounds. Test results showed he didn't have cancer, or indeed anything else. Mysti- fied, the hospital gave up and sent him for psychiatric examination. Finally, when he thought he was near death, he decided to undergo neurological tests. He heard that Mount Sinai had a good neu- rology department.

There he was told he had Tourette. ''It was an incredible relief,'' Wolfe says. ''I mean, I thought, 'Wow! I'm not psychotic, and I've been goofed on all my life.' I'd had a pretty big inferiority complex because of it. I felt I couldn't be out in public, the whole bit. But when they said it was Tourette, and told me what that meant, I said, 'My God, it's something in the medical books. It's not something I invented!' Twenty years I'd suffered.''

With the diagnosis came not only understanding but also a measure of treatment. At Sinai, after unsuccessfully trying 36 different drugs on his first Tourette patient, Arthur Shapiro had hit upon haloperidol, a strong tranquilizer ordinarily pre- scribed for schizophrenia. The drug dramatically reduced that patient's ticquing -- as it has for more than three-quarters of his patients since.

But haloperidol doesn't help some patients, and it often produces devastating side effects. They include, most frighteningly, muscle spasms that can seize the entire body: the head pulls to the side, the back arches, the eyes roll back, and the tongue protrudes. Just as bad is a condition that mimics Parkinson's disease: slow shuffling of the feet, drooling, tremor, and zombie-like loss of expression. In other cases, instead of lethargy and slowness of thought, the patient experi- ences an uncontainable restlessness. Sometimes there's mild depression, or an inability to concentrate, or a hugely increased appetite, leading to gross obesity. Worst of all, because it's irreversible, is a condition called tardive dyskinesia, which produces uncontrollable writhing and jerking. And although another drug, Cogentin, mitigates some of haloperidol's side effects, it sometimes makes urinating difficult, and can cause visual hallucinations.

''When the ticquing gets bad, I'd rather just wait it out,'' says Wolfe, and he has been able to suppress his tics for as long as 20 hours. But he pays a price: once he lets go at home, his tics go into overdrive, and sometimes his ear-shattering screams have brought neighbors, and even the police, rushing in thinking he was beating his wife. (Wolfe got married in 1976. ''I was attracted by his sensitivity and his sense of humor,'' his wife says.)

Of course there are times when he can't store up the tics. He tells about being at a restau- rant one evening, just a table away from ''this huge black guy and is wife.'' He erupted with ''Who-ore nigga!''

''We're in trouble,'' he managed to whisper to his wife before anyone had a chance to react. ''Let's get outta here.''

Too late.

'''What did you call my wife?' the guy asked. We were still sitting there.

''He swung at me. I told him what my problem was, but the guy swung at me!''

Tourette exacts its price at work, too. Wolfe has had so many jobs, so many bosses who've run out of patience with him, that he has lost count. One year, he remembers, he had 46 W-2s.

The war between Wolfe's intelligence, which is considerable, and the ways in which some mysterious other part of his brain has undermined his potential has often left him frustrated. It was dealing with these difficulties -- not Tou rette, but its side effects -- that led Wolfe, like many others with the syndrome, to develop a winning sense of humor.

''I have a way with people, if you don't count bosses,'' he says. ''They learn to accept me right away. I'm a comedian -- you know, I cover up that way. I'm a center-of-attention kind of guy, either using my tics to be funny or diverting attention from them with comedy. I'm compensating, overcompensating. But at the end of the day I'm wiped out. And people I know imitate this stuff. Not to hurt me -- it's just sort of in- fectious. I have to deal with this all the time.

''My friends decided to have an Academy Awards, for example. The best tics of the year win prizes, like the one that was most imitated by the group. I've won a few awards in my day, too -- believe me, I've won a few.''

It's no wonder that Wolfe and many other Touretters prefer their tics and their verve to a drug-induced relief: if the tics aren't exactly fun, at least they're familiar. Says Julius Wechter, a successful percussionist whose symptoms disappeared while he was taking haloperidol some years ago, ''It was sort of like losing my arm. I'd lived with this thing for so long that it felt strange not to have it.''

Another musician said to Oliver Sacks, a neurologist at New York's Albert Einstein College of Medicine and a best-selling author, ''Suppose you could take away the tics, what would be left? I consist of tics -- there'd be nothing left.'' When this patient was on haloperidol, says Sacks, he found he was ''musically dull, average, competent, but lacking energy, enthusiasm, extravagance -- and joy.''

Indeed, there's something primal about the behavior that Tourette evokes. Inevitably, Sacks says, plumbing the secrets of Tourette will lead to ''insights into the nature of cursing and joking,'' and it's already revealing the workings of ''instinctual and behavioral structures of a primitive kind.''

Although the brains of perhaps half a dozen Tourette sufferers have been autopsied, no anatomical locus for the disease has been implicated with certainty. But it's there, Shapiro thinks. ''It's as if there's an organic short-circuiting of a place in the brain where so cially unacceptable ideas are stored,'' he says. And recent PET scans of people with Tourette have shown special activity in the basal ganglia, a pair of deep-lying structures that facilitate physical coordination by helping to relay signals from the cerebral cortex to the brain stem.

Victims of Tourette deny that they're consciously responsible for their cursing -- deny even that the sounds have an emotional content -- but they're still forced to acknowledge that there's a pattern to their outbursts. ''It's not random,'' says Palmer, ''at least in the sense that I know I shouldn't do it. The common denominator is the forbidden -- that's what comes out, in the middle of fifteen million other things.''

According to Sacks, the late Russian neuropsychologist A. R. Luria, to whom he often sent his observations on Tourette, wrote him that ''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.''

That's a bold statement about a disease so rare that until not long ago most doctors had never heard of it, much less seen it. Even the victims had no name for their condition. As recently as 1972 Faruk Abuzzahab and Floyd Anderson, University of Minnesota psychiatrists, could only identi- fy -- by searching through the medical literature -- a worldwide total of about 750 cases in the past hundred years. According to the Shapiros, even by the early 1980s the average time between the onset of the tics and diagnosis was more than a decade. Those who suffered from the syndrome did so in the dark, never even realizing their affliction was a subject for medical treatment.

However, the first document referring to what was probably a case of Tourette dates from 1489. The diagnosis was possession by the Devil, and the treatment, prescribed by the church, was exorcism:

''Look speedily and snatch from damnation and from this Devil of our times this man who was created in your image and likeness. Throw your terror, Lord, over the Beast who is destroying what belongs to you!''

When exorcism failed, victims were sometimes burned as witches.

Indeed, those who have Tourette in its full-blown form do seem possessed, if not by a demon, then by something altogether alien -- and something that speaks. The obscenities, the barks, the screams, the violent contortions are so at odds with the victim's personality that it's easy to understand why the symptoms have been seen as invasion by an evil spirit.

A letter published in the Journal of the American Medical Association in 1974 suggested that the case that was the basis for The Exorcist involved a youngster with Tou rette. After seeing the movie, a couple of dozen Touretters thought they recognized themselves and appealed to the Catholic church to have the rite of exorcism performed.

Others resort to even more primitive expedients. A fundamentalist minister in Missouri repeatedly beat his daughter to get rid of the devil he thought was within her. She was subse- quently diagnosed as having Tourette.

In the nineteenth century, consistent with the tendency of the time, people with Tourette were characterized as ''higher degenerates'' of ''neuropathic heredity.'' They were treated, too, in the fashion of the day: incarceration in lunatic asylums.

Even twentieth-century med icine has inflicted treatments more harrowing than the disease. The more advanced the methods, in fact, the more grotesque they become when they're misapplied: insulin coma, carbon dioxide inhalation and prolonged sipping of water (presumably on the old hiccough paradigm), hypnotherapy, allergic desensitization, behavior modification, lobotomy, electroshock.

The most widespread -- and, thanks to its apparent gentleness, often the most insidiously destructive -- technique of our time has been psychotherapy. Freud characterized Tourette and tics as neuroses resembling hysteria. Later variations on the psychoanalytical theme also followed the fashions of the moment: ''displaced unconscious muscular eroticism toward the father''; ''nar- cissistic onanism,'' or, worse still, ''masturbatory conflict''; ''defense against auto-pleasur- able thumb-sucking''; and ''inhibited aggression,'' in which ''swearing becomes a substitute for physical assault.''

Other therapie at least started from the assumption that the cause of Tourette was tangible; if they failed, they failed. But psychotherapy assumed otherwise: that the condition was of emotional origin, and alterable through chats with a doctor. Since the disorder turned out to be organic, failure was as inevitable with psychotherapy as with other, less subtle, treatments. But the difference is that psychotherapeutic failure typically generates more therapy -- perpetuating, often after first implanting, a confidence-wrecking burden of guilt both in the patient and (when the patients are children) his parents. The financial burden to the families is also staggering.

A typical example of the psychoanalytic approach to Tourette dates from 1949, the heyday of misapplied Freudianism. A 14-year-old boy was under the treatment of a Vienna-born psychiatrist at Johns Hopkins. According to a press account, ''[the doctor believed] that the patient's family background always contains at least one domineering parent . . . The unprintable four-letter words are directed against the parent or some authority. In the most dramatic manifestation of the disease, the victim is impelled to get up in church and denounce the clergyman in resounding obscenities.''

In the 1960s a London psychiatrist claimed that the cause of seal-like barking and physical spasms in one of his patients, who was naturally left-handed, was the result of the man's having been forced to write with his right hand. He treated the patient -- successfully, he felt -- by prescribing a quarter of a gram daily of chlorpromazine, a tranquilizer with the power of a sledgehammer.

In 1966 a psychologist in Scotland claimed he'd ''cured'' a 22-year-old man by getting him to repeat his usual obscenities as loud and fast as he could until he was exhausted. The use of alternative words or flagging from a metronome-paced speed of up to 200 curses a minute was discouraged by mild electric shocks to the back of the hand. ''Of course, restraining the symptoms may not be curing the disease,'' a report on the case concluded. ''But ulcers are certainly more socially acceptable than dirty words.''

Still, these people may have been lucky compared to those who were left to the mercies of their families. The Shapiros recall the case of a boy who had been diagnosed as schizophrenic and confined with a roommate who was instructed to beat him up every time he made a noise. Another case involved a boy whose parents locked him up in a trailer in their back yard because they couldn't stand to live with him.

Palmer says that schoolmates used to call him ''chicken head'' because of the way he used to jerk his head back and forth. His parents begged and even punished him to get him to stop. His teachers, who felt he had a behavior problem, sent him repeatedly to the principal's office. ''There was no question in their minds -- or, finally, in my own -- that it was a childhood tic, and that childhood tics are caused by psychological problems,'' he says. ''Therefore, presumably, I could control it. If I didn't, the fault was mine.'' Says Arthur Shapiro, ''The burden of pa- tients with Tourette syndrome is so immense, it's a wonder to us and a credit to them that they don't become psychotic.''

Not until Tourette was diagnosed as a neurological disorder, caused by something chemically amiss in the brain, did victims begin to receive appropriate help. That the syndrome is no longer considered a psychiatric affliction can be credited largely to Shapiro, who 20 years ago determined that a patient of his named Brenda had an organic rather than a psychiatric disorder. He made this diagnosis partly because he connected the snorting, cursing, grimacing young woman with an incident he'd witnessed 15 years earlier in a Greenwich Village bookshop.

While he was browsing through the stacks, he suddenly heard a barrage of grunts, hoots, and curses. He was stunned when he caught a glimpse of the person who emitted them. ''When I looked around, I saw a nice young woman, very conventional, politely perusing the collected works of Keats,'' he says. ''Years later, when I first saw Brenda, I was struck by the way her behavior resembled the other woman's. After treating her, I decided she wasn't suffering from any psychiatric disorder I'd seen. I began to believe I was looking at a neurological rather than a psychological problem.''

Shapiro began treating Bren da with drugs -- anticonvulsants, tranquilizers, even anti- depressants -- until he came upon haloperidol, which had been used abroad for several years to treat schizophrenia. ''Within a week,'' he says, ''every symptom disappeared.''

Since then, Shapiro has given haloperidol to most of the 1,700 patients he and his wife have diagnosed as having Tou rette. They've found that it has not only mitigated the symptoms; it has also pointed to Tourette's organic nature -- and to its possible mechanism and seat in the brain.

Says Ken Bonnet, an associate professor of psychiatry at New York University Medical Center, ''The pharmacology of haloperidol not only gives us ways of managing the disease, albeit imperfectly; it's also a very valuable probe with which we can infer some of the biochemical architecture of the disorder. I think we're coming to the point now where we can put together biochemistry, neuroanatomy, and function in understanding Tourette and related disorders.''

Since haloperidol is known to decrease dopamine, a neurotransmitter that's active in the basal ganglia, the obvious explanation for its effectiveness with Touretters was that they must suffer from excessive dopamine production.

There seems to be an optimum level of dopamine: too little, as in Parkinson's disease, and movements become sluggish; too much, and they're hyperactive or out of control.

But because many other drugs that decrease dopamine aren't effective against Tou rette, scientists began to think that the original hypothesis was too simple. For one thing, it seems probable that more than one neurotransmitter may be involved. Even as far as dopamine is concerned, says Shapiro, ''it's probably not excess dopamine, per se, that matters as much as a patho- logical sensitivity to normal amounts of it.''

Arnold Friedhoff, Bonnet's supervisor at NYU and director of the Millhauser Labora tories there, says that ''there's a balance in the brain between the cells from which dopamine is released and the cells, on the other side of the synaptic gaps, that have the dopamine receptors. If there are too many receptors on the postsynaptic cell, then in the normal situation the brain may compensate by reducing the amount of dopamine being produced in the presynaptic cell.'' But with Touretters, the compensation may not be enough.

Says Bonnet, ''Just because Tourette patients respond to antidopamine medication doesn't mean we're dealing purely with a dopamine system at all; it says we're entering a complex system, and we're tweaking one of the important keys in it.''

Studies at Yale indicate that whether Tourette is a dysfunction of dopamine release or uptake, or something more complex, it's genetically determined. David Pauls, assistant professor of human genetics in the Child Study Center at Yale, has discovered by following 120 familiesin which one member has Tourette that about 90 per cent of people with the syndrome have other family members with Tourette or tics. He has also learned that the condition affects four times as many boys as girls.

Moreover, in a recent study that included a large Mennonite family in Canada, Pauls discovered an apparent link between ''pure'' Tourette, which manifests itself in simple, eruptive tics, and

more complex, obsessive-compulsive behavior, such as repeated toe-touching. hat remains is to find the part of the brain that might be responsible, when it malfunctions, for both sets of behavior. PET scans, like the inferences drawn from the effects of haloperidol, point to the bas- al ganglia. But by combining neuroanatomical and biochemical information with sophisticated EEG data, Bonnet puts his money on a neighboring area, deep within the inner forebrain -- the cingulate cortex.

He presents his theory as just that: an untested but altogether logical alternative to the basal ganglia hypothesis. How did he arrive at it? Not just by looking at the action of halo- peridol, he says, but by ''going back to the basic symptoms and trying to make some sense of their anatomy.

''If we assume that haloperidol really is effective,'' he says, '' -- and it may be that it doesn't correct the symptoms so much as mask them -- then it still doesn't tell us anything about where it's effective. There are a number of dopamine systems that it blocks, after all, and it can't be all of them: many of the symptoms just aren't right for that to be the case. For example, hyperdopamine function is one of the models we have for psychoses like schizophrenia. But these guys aren't schizophrenic -- not by any stretch of the imagination.

''Now, if you see a movement disorder, the first thing you usually do is go to the motor cortex. And if you have vocalization, you go to the speech area. I say both of those are the wrong things to do, because when you stimulate the motor cortex electrically, the movement patterns you get aren't the kind you see in Tourette. And when you stimulate the speech area, you get articulat- ed speech -- not the kind of hoots, grunts, screeches, and yelps you so often get out of a Tourette patient. And even though we tend to focus on the more dramatic components, like coprolalia, these aren't often the early symptoms, and they're not the most common.''

Bonnet also points to the curious fact that the symptom pattern varies not only from person to person but within each individual. How can this be explained by a single lesion in the brain? ''It can,'' he says, ''but only if there's a single area that's capable of eliciting the large variety of tics that you see in Tourette.''

For instance, by repeatedly stimulating the cingulate cortex with low-intensity elec- trical current in non-Tourette patients undergoing brain surgery, Bonnet points out, researchers in France induced movements that are ''part of the classical symptom cluster of Tourette, including inar-

ticulate vocalizations. What's more, the movements and vocalizations occasionally intruded upon what the patients were doing, just as in Tou rette.'' At times the patients couldn't suppress the tics, and at times they could forestall them briefly.

''Patients like Orrin Palmer,'' Bonnet says, ''frequently end a tic with both eyes moving sharply and suddenly to the upper right. Now, there are three areas of the brain that can be stimulated to cause that -- including the cingulate cortex. But the only area that can al- so account for Tourette-like movements and speech patterns is the cingulate cortex.

''It's an attractive area too if you go back to the haloperidol premise,'' he says -- that since haloperidol blocks dopamine, and haloperidol seems to help ameliorate Tourette, then dopamine might be a culprit in Tourette -- ''because the cingulate cortex has a very high rate of input of dopamine. It also has a very high sensitivity to clonidine'' -- an antihypertensive, of all things, that's being used by some doctors for Tourette.

Bonnet's theory also takes into account the obsessive or compulsive component frequently seen with Tourette. One situation in which it manifests itself is in reading. ''It isn't that people with Tourette are unable to read,'' he says. ''They're perfectly able to read.'' But if they're left alone, without distraction, many will sit down at a book and stare at the same page for ten or fifteen minutes, reading unproductively, unable to get unstuck from the page. ''It makes sense to look at this persistence, this obsessiveness,'' he says, ''not as a personality disorder but as a piece of the things that come in and out of the circle that we call Tourette. The intriguing part is that in the 1950s cingulectomy was used to treat extreme obsessive-compulsive disorder. Today the cingulate cortex is still implicated, but pharmacology has supplanted surgery.

''Likewise, you find that when there's anxiety, symptoms get worse. Well, it also turns out that one of the targets for control of chronic anxiety by surgery was again that cingulate area. There are just continual reinforcements of the pattern.

''Now, there's a large fiber bundle, called the cingulum, that goes up through the cingulate cortex from some limbic areas, which play a role in the emotions, and dumps into the frontal lobes.''

In Bonnet's view, Tourette may be a ''disorder of compartmentalization'' in the forward part of the cingulum, so that when one area of it fires up, others that shouldn't do so fire up at the same time. He says it may ultimately be shown that the reason that haloperidol and other dopamine-blocking agents seem to work is that the forward end of the cingulum feeds into what he calls ''the richest dopamine-dependent area of the cerebral cortex.''

Bonnet also has a linguistic argument. For one thing, he says, it's most common in Tourette to utter words that are monosyllabic and that rely on particular sounds. For another, he points to a suggestion, by Marc Nuwer at the Reed Neurological Center at UCLA, that random computer generation of chains of high- probability sounds in English will, with ''remarkable frequency,'' come up with combinations that resemble the so- called four-letter words that are emitted in Tourette. What we call swearing, in other words, may be nothing more than vocal tics -- devoid of content or meaning -- that are formed of the language's most common sounds and that most quickly release a build-up of neuromuscular tension. For the same reason that a normal person chooses those exple- tives when he's too angry to think selectively, the Touretter may just be producing the sounds that come most easily.

''First of all,'' Bonnet says, ''we don't hear Tourettes saying dirty words every time they utter something. It may be every third or fifth uncontrolled vocal outburst that has something articulate -- and, of those articulated outbursts, a fairly high number are vulgar. But the rest of what you hear in Tourette people isn't articulated speech -- it's shrieks, and grunts, and so on.''

Although articulate speech is considered a function of the cerebral cortex, Bonnet points to an interesting model -- one with what he calls ''Tourette- like intensity'' -- for subcortical ''speech.'' ''Thereare German studies,'' he says, ''that show that if you stimulate certain species of monkey in the areas on either end of the cingulum bundle, you get socially relevant calls, or eating calls, or aggressive calls, or sexual calls, and along with some of them you get gestures appropriate to the context in which the calls usually occur naturally. The kind of 'speech' you get out of these regions is very much like the kind of 'speech' you most often get out of Tou rette: not articulated speech, but shrieks, yells, cackles, and grunts.''

Then again, he says, ''when Touretters do say words, why are they so often the offensive ones? Why don't they say 'pretty' instead of 'prick,' or 'ship,' not 'shit'?''

Kenneth Kidd, a professor of human genetics at Yale, has a theory that draws these observations together. ''There's one bit of evidence that gives me an idea for a possible explanation of coprola lia,'' he says. ''There's a kind of aphasia -- loss of ability to speak -- that results from a stroke, in which people still have a lot of language capability left. They can understand, and they can write down their thoughts, but they lose the ability to convert those thoughts into spoken words. But many people with this kind of aphasia, if they become extremely angry, upset, or emotional, can swear fluently.''

Like Bonnet, Kidd thinks ''it's possible there are two different pathways that initiate speech -- one in an area of the brain that's under conscious control, and another that's not, but that's highly charged emotionally. It's possible that in Tourette patients it's the second neural pathway that's being stimulated and that's resulting in vocal twitches and expletive language.''

Shapiro demurs. ''Wherever these outbursts originate,'' he says, ''if it's not cortical, it at least has to be modulated at some point by the cortex.'' He points out that the only times symptoms are absent are during sleep and orgasm, when people ''go biological,'' or when, as while they're

playing a musical instrument, they ''give up cortical thinking.'' What's more, he says, ''Tourette isn't found in animals.''

Friedhoff, for his part, emphasizes a disorder in the ability to inhibit behavior. ''Just keep track throughout a day of how many things you think about that you inhibit yourself from saying,'' he says. ''Tou rette people can't always do that. As soon as something arises in their minds, it comes out of their mouths. Speech involves a very sensitive balance between impulses activating the vocal cords and impulses that are constantly censoring them. When there's a lack of inhibitory control, the normally suppressed thoughts are going to come out first.''

''Certainly,'' says Shapiro, ''though the tics carry no emotion themselves, the condition worsens when there's excitement of the emotions.'' After all, says Sacks, ''the disturbance seems to lie in the highest parts of the 'old brain,' where the instinctual deter minants of personality are lodged -- a sort of missing link between mind and body.''

In that, Luria's poetic vision may be right: by understanding Tourette, we may learn, some- day, not just about the nature of cursing but about the nature of our humanness, and of our deepest selves.

COPYRIGHT 1986 Discover
COPYRIGHT 2004 Gale Group

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