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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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Taming Tourette's tics & twitches - includes related articles
From FDA Consumer, 9/1/88 by Evelyn Zamula

Taming Tourette's Tics & Twitches

Poor Danny. In the doghouse again. Teacher sent him out of the room because he's disrupted his second-grade class just once too often this morning. She knows he's being treated for hyperactivity--and she can cope with his occasional frenetic outbursts--but lately something new has been added. He blinks his eyes and twitches his nose continually, and his feet are never still. Every once in a while he'll let out a series of yelps, which the children find hilarious, but which also makes it impossible for her to teach. He has told her he can't help making these noises. She is beginning to wonder. Can it be that Danny has that disease she heard about on TV a few years ago? The one in which the victims uncontrollably bark like dogs and curse and scream?

The disease she's thinking of is called Tourette syndrome, and her suspicions about Danny are correct.

People with Tourette syndrome have recurrent involuntary muscle contractions called tics, such as eye-blinking, nose-twitching, facial grimacing, head-shaking, or shoulder shrugging. TS usually begins in childhood, between the ages of 2 and 15, with most cases clustered around the age of 7.

TS is seen throughout the world, in all races. Because TS often is not diagnosed correctly, accurate figures are hard to come by, but experts estimate that as many as one in 2,000 persons may be affected by the disorder, with males outnumbering females approximately three to one. It is believed that about 100,000 people in the United States have the disease.

Although childhood tics are common--about a fourth of all children have them at one time or another--they usually disappear spontaneously within a year. In patients with TS, however, the tics usually persist for life, except for a lucky few who experience complete remission during adolescence.

The tics may affect different parts of the body at different times, and get better or worse over weeks or months. The first tics to appear are motor tics, usually in the face, with eye-blinking the most common. The facial tics may vanish, only to be replaced by new ones that may involve the neck, arms and legs in the form of head-tossing, neck-stretching, arm flailing, shoulder-shrugging, jumping, or similar purposeless movements. The motor tics may either go away or be joined by vocal tics--grunts, throat-clearing, barks, sniffs, whistles. About a third of the victims feel compelled to use explicit four-letter words. This is called coprolalia, which means "babbling about feces." Some people with Tourette syndrome repeatedly echo other people's words (echolalia) or imitate others' movements (echopraxia).

Those with severe cases may practice self-mutilating behavior, such as lip-biting or head-banging, while others may be caught up in obsessive-compulsive behavior such as excessive handwashing or elaborate bedtime rituals. Their lot is not a happy one.

TS people can sometimes control their tics for minutes to hours, but sooner or later the urge becomes irresistible, and the tics must be allowed to play themselves out. Tics get worse when TS patients are anxious and improve when they concentrate on something. Since many patients try to keep a tight rein on their tics at school or at work, they are inclined to "let it all hang out" at home where they're comfortable.

The cause of TS is unknown. For years some scientists argued that the disease was a psychiatric disorder. Freud himself believed that tics were a neurosis, resembling hysteria, but that TS was hereditary. Psychoanalysts who came after him used familiar Freudian terms to explain TS, in which words like eroticism, sadism, aggression, anal, narcissism and the like figured prominently.

Other scientists, including Gilles de la Tourette, the physician who described the disease definitely in 1885 and after whom the disorder is named, believed TS had a physiological basis, which is also the current thinking. No doubts at all existed in the minds of a group of inquisitors in 1489, when a priest with motor and vocal tics was diagnosed as being possessed by the devil and was subjected to the rite of exorcism.

The story goes that he was cured and thus avoided being burned at the stake. But, as Arthur K. Shapiro, M.D., and Elaine Shapiro, Ph.D., point out (in a July 1982 article in the American Journal of Psychotherapy), exorcism seems to have lost its clout through the ages. Inspired by the book and movie "The Exorcist," about two dozen people with TS symptoms have undergone exorcism in the last few years, but no cures have been reported. The Shapiros and others believe that "The Exorcist" was an exaggerated description of an actual TS patient. The young girl in the story displayed a number of TS symptoms: vocal tics (animal sounds, like barking and mewing); purposeless movements (whirling and flinging); obscene language; self-mutilation (scratching and biting); and an abnormal degree of neck-stretching, culminating in that hair-raising scene in the movie where her head appears to completely turn around.

The best evidence suggests that, although TS has psychological manifestations, the disease is a neurological disorder. Researchers believe that there is a chemical abnormality in the part of the brain called the basal ganglia. This affects the neurotransmitter systems used by the brain to regulate movement and behavior. (Neurotransmitters are chemicals that carry signals between nerve cells.) Many people with TS show signs of neurologic abnormalities, such as hyperactivity, poor coordination, and attention deficit disorder (characterized by impulsiveness, hyperactivity, poor concentration, and inattention).

A certain type of TS is inherited. A high percentage of Tourette patients have relatives with motor tics, and some families have more than one member with TS. Recent genetic studies indicate that the three types of tic disorders (childhood tics, chronic tics, and Tourette syndrome) are all genetically related, making TS a common hereditary illness. Scientists do not yet know why some people get milder forms while others get the full-blown syndrome with its motor and vocal symptoms.

Just as the world of psychiatry was turned upside down when it was discovered that many disorders presumed to be "psychiatric" in fact had a physiological basis and could be treated with drugs, so was the argument that TS had an organic, rather than a psychologic, basis made more compelling by the fact that certain drugs were also found that controlled tics in a large number of cases.

But a disease in which the body is made to do things it doesn't want to do is bound to cause psychological problems, too. Though many Tourette people with mild symptoms adjust quite well to their disability and can hold jobs, marry, and live normal lives, those with more serious symptoms that interfere with normal relationships may need psychological help to cope with this socially crippling illness.

This is especially true for Tourette people who demonstrate obsessive-compulsive behavior, an example of a classic psychiatric symptom arising, in this case, from neurological disease. It may be almost impossible for obsessive-compulsive people to keep a job. People who arrange or rearrange papers on their desks all day long or touch their toes repeatedly are not likely to get much work done.

Some TS people never even make it out the front door in the morning. They spend the day taking innumerable showers, or feel compelled to touch and retouch certain objects in the house a certain number of times before leaving. Acting out the compulsions gives them no pleasure, because they know the behavior is senseless. Some obsessive-compulsive patients have recurrent, protracted thoughts about death or sex that haunt them night and day. Depression, embarrassment, and despair are an everyday part of life for these unfortunate people. A small number of people with severe TS symptoms have been institutionalized, either because they've been incorrectly diagnosed or ineffectively treated.

TS children often have problems in school because other children may make fun of them or are even afraid of them. As a result, TS students are often forced to use all of their energy and concentration trying to control or mask their symptoms. Teachers may become exasperated by TS students' tendency to perseverate--that is, to ask the same question over and over again, even though they know the answer. Some teachers incorrectly interpret the motor and vocal tics as misbehavior or attention-getting devices, and may punish a child for symptoms he or she can't control.

Parents, to their dismay, often make the same mistake. "From the time I was 7 until I was 11, I spent a lot of time in my room," says Michelle. "My parents thought my eye-blinking was deliberate, so they punished me by sending me up to my room. Relations between me and my parents were very bad at that time. It was only by chance that we discovered what the real problem was. We were at a party with some other families, when I noticed one of my father's friends staring at me. By this time, I was not only blinking my eyes, I was twitching my nose, jerking my arms, and doing some grunting. He told my father that his partner's son was making the same kinds of movements, and when they took him to the doctor, his parents found out he had Tourette syndrome. I was taken to a neurologist a week later and I was diagnosed with the same thing. My parents felt terrible, but I was kind of relieved. At least I had something that had a name."

Many TS children have difficulty fitting in socially. Many simply have no friends. Some, like Michelle, miss so much school because of twitching, that they don't get a chance to form close relationships. Many are left out of social activities, and tend not to be chosen for team sports, or elected class officers, or asked out on dates. To avoid psychological damage, it is important that the disease be diagnosed and treated early. When a child is told often enough that he's crazy, in time he'll begin to believe it. Educating classmates of TS children about the disorder also helps lessen teasing and ridicule. Videotapes and pamphlets can be used to teach children to be more tolerant of TS symptoms.

Tourette children are usually of normal intelligence, and most can attend regular school. But some may need to be in special classes, or smaller classes, because besides motor and vocal tics, they often have attention-deficit disorder, learning disorders, and speech problems, such as stuttering. Children with more severe TS may become such behavior problems that they must go to special schools.

Most need the help of compassionate teachers, who will allow for some flexibility in the normal school routine. This may include a private study area, extra time to complete assignments, privately held exams, or oral exams if the child has difficulty writing. Reading disorders are six to seven times more common in TS children than in the general population, so timed tests often throw them into a panic.

In the past, all kinds of treatments were tried on Tourette patients, including acupuncture, shock treatment, insulin shock therapy, hypnosis, tranquilizers, psychotherapy--even frontal lobotomy. The modern era of treatment began in 1961 when Italian and French researchers discovered that haloperidol, a major tranquilizer used to treat severe mental disorders such as schizophrenia, also suppressed tics. Haloperidol is believed to block transmission of dopamine, a neurotransmitter. It is not clear to researchers whether the brain's dopamine systems are overactive in TS or whether the dopamine receptors in the brain are hypersensitive. Other neurotransmitters (serotonin and norepinephrine) may also be involved.

Haloperidol reduces tics in as many as 86 percent of Tourette patients, but has to be stopped in most cases because of formidable side effects--depression, weight gain, difficulty in concentration, drowsiness, a feeling of being "spaced out," and Parkinsonian symptoms of tremor, rigidity, lack of facial expression, and slow movement. (Since Parkinson's disease is characterized by abnormally low levels of brain dopamine, there's obviously a fine line between too much and too little dopamine.)

Those who can't tolerate haloperidol may be switched to other drugs, among them pimozide, another major tranquilizer, or clonidine, a blood pressure medication that is used experimentally in treating the obsessive-compulsive symptoms of TS. Side effects of pimozide are similar to, though not as severe as those of, haloperidol, and clonidine may cause dry mouth.

Pimozide, incidentally, played a major role in the passage in 1983 of the Orphan Drug Act, a law that made it easier to get drugs for rare disorders on the market in the United States. (See "Medicine's 'Orphans': Drugs for Rare Diseases" in the February 1988 FDA Consumer.) Though approved in Canada, pimozide was unavailable here because the manufacturer, McNeil Laboratories, did not find it economically feasible to go through the tests required for FDA to approve the drug. Consequently, about 100 patients were illegally smuggling the drug in from Canada. The unavailability of "legal" pimozide was called to the attention of several congressmen by families of TS patients and the Tourette Syndrome Association. After a series of congressional hearings investigating the plight of those with so-called "orphan diseases"--diseases affecting fewer than 200,000 people--Congress passed the Orphan Drug Act in 1983. Under this law, drug companies receive special incentives to develop unprofitable drugs for small patient populations. The law also added a fast-track drug approval system in FDA. Pimozide was approved in 1984.

When Tourette patients start drug therapy, they usually must see their physicians often, because considerable fine-tuning of medications is necessary. a drug that works initially may lose its effectiveness, so another drug may have to be tried. Dosage may be increased when symptoms worsen or decreased when the disease is in remission. However, many patients find significant relief in drug therapy, with few or no side effects, and may see their doctors only once a year. Others with mild symptoms may require no medication. Very often, if a patient can cope psychologically--even with severe symptoms--drug therapy is not necessary.

Michelle has had her ups and downs with drug therapy. "In the 10th grade I was out a whole semester because I was ticcing like mad. My doctor took me off Haldol [haloperidol] for two weeks to see if it was the drug that was making me tic so much. Those two weeks were unbearable. You know, Haldol is a major tranquilizer, and when I was off the drug, I just couldn't sleep. My tics would keep me up until 4 in the morning. The doctor put me back on a half-dose of Haldol and I was O.K. until last summer when I developed blepharospasm [rapid twitching of the eyelids] that was so bad that I couldn't see. Altogether, I was on Haldol for 10 years. Now I'm on Orap [pimozide] and Prolixin [fluphenazine, a drug used experimentally in the treatment of TS]. My doctor is gradually decreasing the Orap and increasing the Prolixin. He wants me to be off drugs completely by this summer, though, but he's doing it very slowly so that I don't get withdrawal symptoms. He thinks my body is becoming immune to drugs and I need a 'drug vacation.' Besides, he wants to see if TS is causing the tics or maybe it's the drugs. I'm scared witless about going off drugs completely."

Maybe Michelle has less to fear than she thinks. Consider Danny's story. Danny is 10 now and has been through some rough times. His tics became worse, eventually involving his upper arms and shoulders and then progressing to complex tics that caused him to leap from his desk and throw himself on the floor. In addition to yelping and screaming, he began using obscene language.

But by that time, Danny's disorder was finally diagnosed correctly. The stimulant medication that he was given in the mistaken belief that he was simply hyperactive was withdrawn. (Some TS experts believe that stimulant medication, such as methylphenidate or pemoline, used to treat children with hyperactivity and attention-deficit disorder, may actually bring on tics in those genetically vulnerable to TS. The labeling of both drugs warns about giving them to children with tics or children who have relatives with tics.) Drug therapy effectively controlled his symptoms, but made him a "zombie" in school, to use his own description. All medication was then stopped. Danny's tics and hyperactivity continue, but supportive counseling has taught him to cope with these problems. At his physician's recommendations, Danny's teachers are trying to minimize stress in the classroom and provide special education services. His schoolmates have been shown a videotape about TS and are much more sympathetic. Members of his family have also undergone counseling and have a better understanding of his illness. They maintain close contact with his teachers. Changes in the home and school environment have made all the difference in the world for Danny, who is now a happy, well-adjusted child.

Research on TS has been neglected until recent years. One difficulty is that there is no dignostic test for TS. It is recognized only when certain symptoms occur together. But interest in this unique disease has revived because biomedical researchers feel there's much to be learned about TS that will cast light on other neurological disorders affecting movement behavior. Major medical institutions throughout the country are participating in TS research, which is being supported by the National Institute of Neurological and Communicative Disorders and Stroke, the National Institute of Child Health and Human Development, and the National Institute of Mental Health.

Because very few autopsies have been done on TS victims, studies of brain tissue have been unavailable until recently. Now, with the help of sophisticated new diagnostic tools, such as positron emission tomography (PET scans) and magnetic resonance imaging, it may be possible to locate more precisely areas of the living brain that are malfunctioning.

Researchers also are conducting genetic studies, including some using twins, to identify families at risk for TS, and to determine exactly how the gene is transmitted. Also, because many TS victims do not respond to haloperidol or cannot tolerate its side effects, investigators are searching for other drugs that may help. At least half of TS research money is being devoted to projects focusing on neurotransmitters and the operation of the synapses--the junction where neurotransmitter chemicals carry impulses from one nerve to another.

For more information, readers may contact the Tourette Syndrome Association, a nonprofit organization located at 42-40F Bell Boulevard, Bayside, N.Y. 11361. The telephone number is 1-800-237-0717, except for residents of New York state, who can phone 718-224-2999.

COPYRIGHT 1988 U.S. Government Printing Office
COPYRIGHT 2004 Gale Group

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