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Tourette syndrome

The Tourette Syndrome Association helps Tourette sufferers by educating the public and physicians about the organic basis of and available treatments for this neurological disorder.

 

Gilles de la Tourette Syndrome, now most commonly referred to simply as Tourette Syndrome (TS), is a nervous system disorder that is characterized by repeated involuntary body movements (“tics”) and uncontrollable vocalizations (“vocal tics” or “phonic tics”). The physical and vocal tics may be either severe (“florid”) or mild, and over time they may unpredictably grow more severe (“waxing”) or less severe (“waning”). Onset of symptoms is usually between two and fifteen years of age, with a mean age of seven.

In many Tourette patients, symptoms grow less severe over the course of adulthood, and by middle age many have achieved complete or near-complete remission of their ticcing behaviors, although a significant minority continue to experience mild symptoms, and a smaller percentage continue to experience severe symptoms.

Tourette Syndrome gets its name from the nineteenth-century French physician Georges Gilles de la Tourette, who described a tic disorder in a landmark study in 1885, but what is now recognized as Tourette Syndrome is not precisely the same condition described by Tourette. As understanding of Tourette Syndrome has improved, the classification and presumed etiology of the disorder has been significantly modified, as have recommended treatments.

Until the early 1970s, Tourette Syndrome was treated primarily as a psychiatric disorder, and psychoanalysis was the preferred intervention. However, for over a century some alert physicians had associated Tourette Syndrome with a variety of choreas (from “Khoreia,” the Greek word for “dance”). Choreas are disorders characterized by involuntary movements, but in which the tics are less violent, irregular, and asymmetrical. The most well-known among these choreas is Sydenham’s chorea, also known as St. Vitus’s Dance.

Such choreas, especially St. Vitus’s dance, were known to be associated with a prior bout of infection, most often infection by Group A beta hemolytic streptococcal bacteria (GABHS). It had been posited that when infection spread to areas of the basal ganglia that control voluntary motor activity, it could trigger in genetically predisposed individuals the involuntary movements labeled choreas.

A worldwide epidemic of infectious encephalitis from 1918-1926, which led to a subsequent epidemic of tic disorders, renewed interest in the possibility that such movement disorders, including Tourette Syndrome, might have an organic rather than a psychogenic origin.

But at the time psychoanalytic theory was so dominant, both in Europe and in North America, that such correlations were either dismissed entirely or explained away with the claim that even if there was an organic component to the condition, that would not in itself be sufficient to produce Tourette Syndrome.

According to psychiatrists of that time, the patient must also be suffering from unresolved psychological disturbances--most likely psychosexual conflicts--and the tics were a way of “acting out” those conflicts. Thus, psychiatric intervention was still considered the preferred method of treatment, and patients and their families (especially their mothers) were told that their own psychological maladjustments were to blame for their distressing symptoms.

Naturally, such a diagnosis added to the burden carried by the patients and their families, but did nothing to relieve the tics or vocal outbursts of the Tourette patient.

The turning point in the diagnosis and treatment of Tourette Syndrome occurred in 1965, when Dr. Arthur K. Shapiro treated a twenty-four-year-old Tourette patient with the neuroleptic drug haloperidol (Haldol), which has a tranquilizing effect in reducing dopamine transmission in the substantia nigra to neuron receptors in the basal ganglia. Hypersensitiiivity of these neuron receptors to the neurotransmitter dopamine has been posited as a cause of the involuntary movements of Tourette Syndrome.

In a 1968 article, Dr. Shapiro and his wife, Elaine Shapiro (Ph.D.), reported the successful treatment of this Tourette patient with haloperidol. In that article they also severely criticized the practice of psychoanalytic psychiatry and its complete failure with Tourette patients, even blaming psychoanalytic intervention for causing many of the psychopathologies associated with the condition.

In 1978, with psychiatrist Ruth Bruun and neurologist Richard Sweet as co-authors , the Shapiros published a landmark book on the disorder, “Gilles de la Tourette Syndrome.”

The Shapiros were also instrumental in the formation of the American Tourette Syndrome Association (TSA), which was organized by patients and their families starting in 1971, to lobby the public, the government, and physicians to recognize Tourette Syndrome as an organic disorder. The first (very small) meeting of the TSA took place early in 1972.

With the help of the Shapiros and a number of other physicians, the TSA became adept at winning grants and shaping media treatment of Tourette Syndrome. As a result of their intense media campaign, the medical establishment’s view of Tourette Syndrome was freed from its century-long submission to discredited psychoanalytic theory.

Perhaps even more impressive was the TSA’s success in getting information to people with Tourette Syndrome and their families, as well as to the public at large. Even today, a diagnosis of Tourette Syndrome is often prompted by the patient and his or her family, as a result of information they have gleaned from the lay media (as a result of the TSA’s tireless media campaign) and through the TSA.

Eventually many people with Tourette Syndrome found that the side effects of haloperidol were intolerable--even less tolerable than the ticcing behaviors that the drug relieved in most patients. Even such substitute drugs as pimozide (Orap). which was better tolerated by many Tourette patients, had unacceptable side effects for many of them.

A number of Tourette sufferers have decided that they prefer their tics to the side effects of the currently available drugs, especially since the efforts of the TSA have done so much to increase public awareness and understanding of the condition.

Now that the tics and vocalizations that characterize Tourette Syndrome are widely recognized, Tourette patients do not suffer the same degree of public opprobrium they once faced, and the TSA provides both information and support to those who must deal with the symptoms of Tourette Syndrome, either in themselves or in their loved ones.

COPROLALIA AND COPROPRAXIA

As a consequence of media exposure, the public is particularly familiar with the most florid Tourette symptoms, in particular “coprolalia” and “copropraxia.” “Coprolalia” is the vocalization of obscene or other socially unacceptable words and phrases. “Copropraxia” means to engage in obscene behaviors.

Despite the public’s familiarity with coprolalia and copropraxia, only a small minority of Tourette sufferers manifest these symptoms. In fact, all of the most florid Tourette symptoms represent a statistical minority within the range of Tourette diagnosis, though they are the patients most likely to be referred for diagnosis and treatment because of the prominence of their symptoms and their interference with the individual’s ability to function normally.

For those who do manifest the most florid ticcing behaviors and vocalizations, the fact that their condition has a name, and that their tics and vocalizations are now widely recognized as involuntary, has helped to alleviate the social isolation and distress that result from speaking and behaving in ways usually considered to be socially unacceptable.

COMORBIDITY

Certain other neurological conditions are often associated with Tourette Syndrome, and it has been posited that such comorbid conditions may be additional symptomatic manifestations of the underlying organic problem that produces Tourette symptoms. Among the conditions frequently found to be comorbid with Tourette Syndrome are Attention Deficit/Hyperactivity Disorder (ADHD) and Obsessive Compulsive Disorder (OCD). Tourette sufferers may also have difficulties with impulse control (disinhibition), sleep disorders, and various learning disabilities, such as dyslexia. Individuals with pervasive developmental disorders (e.g., autistic spectrum disorder) are also often diagnosed with Tourette Syndrome.

DIAGNOSTIC CRITERIA

--Multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily at the same time.

--Tics occur frequently during a twenty-four-hour period (usually in bouts), nearly every day or intermittently, throughout a period of more than one year. The individual does not experience a tic-free period of more than three consecutive months.

--Periodic changes in the number and type of tics, in their frequency and location, and in their severity (waxing and waning). Symptoms may even disappear for weeks or months at a time.

--onset occurs before eighteen years of age.

PREVALENCE

The prevalence of Tourette Syndrome is estimated at approximately 2% of the general population. However, this may be a conservative estimate, since many people with very mild tics may not be aware of them or may not be bothered by them, and thus never seek medical attention. Tourette Syndrome seems to be far more common in boys than in girls. In fact, three to four times as many boys as girls are diagnosed with Tourette Syndrome. But this may be the result of an ascertainment bias. If TS symptoms in girls are often more often mild in their manifestation, as may be the case, then boys with Tourette Syndrome would be more likely to be referred for diagnosis and treatment.

HEREDITY

Research has determined that a significant genetic component is involved in susceptibility to Tourette Syndrome. Environmental insults, such as GABHS (strep) infection, are more likely to produce Tourette or other movement disorders in genetically susceptible individuals. The Tourette Syndrome Association has aided research into the genetic component of Tourette Syndrome by putting scientists in touch with families in which several members have Tourette Syndrome or other related movement disorders. The TSA also provides significant amounts of funding for such research.

ARE TICS TRULY INVOLUNTARY?

It is not entirely accurate to say that tics are “involuntary.” It would perhaps be more precise to say that they are a response to an irresistible inner compulsion, like the urge to scratch an itch or to sneeze. Like the urge to sneeze, the impulse to tic may be resisted or suppressed for a limited time, but at some point, usually sooner rather than later, it will be released, and that release will be more explosive if the tic has been suppressed.

Many individuals with Tourette Syndrome are able to suppress their tics for a variable amount of time, but when they do, they usually undergo a lengthy explosion of suppressed tics once they are free to release their ticcing impulses. That is why people with Tourette Syndrome often experience pronounced episodes of ticcing while relaxing at home after a day spent attempting to suppress their tics in public situations.

This variable ability to suppress tics for a limited time under certain circumstances is responsible for much of the misunderstanding faced by Tourette sufferers, especially children. Because they can suppress their tics sometimes, parents, teachers, and other observers often assume that ticcing behavior is actually willful misbehavior, that the child could stop if he wanted to. The unpredictable waxing and waning of tics, and the fact that tics often disappear, only to be replaced by other tics, also make it hard for others to believe that the tics of a Tourette sufferer are not deliberate--or even just bad habits that the individual is making no attempt to overcome.

ENVIRONMENTAL INFLUENCES ON THE SEVERITY OF TICCING BEHAVIORS

When a Tourette sufferer is engaged in enjoyable or satisfying activities that require intense concentration, tics may disappear altogether, as in the case of the surgeon with Tourette Syndrome who never experiences tics while performing surgery. Unfortunately, even this phenomenon has led to the blaming of children with Tourette Syndrome. When parents or teachers see that a child can play video games for several hours without ticcing, they assume that the child has control over his tics and only displays them when he wants attention.

On the other hand, tics often become more numerous and severe when the individual is under unusual stress, when he is depressed, or when he is overexcited.

PROBLEMS WITH DEVELOPMENT AND SOCIAL ADJUSTMENT

Many of the problems faced by children with Tourette Syndrome are caused not by their tics but by the fact that they are subject to negative emotional and social feedback at important points in their development. Their academic development may also be impaired if their ticcing behaviors prevent them from functioning well in school. These problems are exacerbated by the fact that Tourette Syndrome is often comorbid with other disorders, including attentional deficits and learning disabilities.

Although Tourette Syndrome is not a psychiatric disorder, it can lead to social and emotional problems, including depression, low self-esteem, and family dysfunction, that may require psychological counseling.

THE TOURETTE SYNDROME ASSOCIATION OF AMERICA (TSA)

The Tourette Syndrome Association of America (TSA) was started in 1972 by families of Tourette sufferers. Their purpose was to disseminate information about Tourette Syndrome--to the public, to medical practitioners, and to Tourette sufferers and their families--in order to counter the widespread ignorance that stigmatized people with Tourette Syndrome and prevented them from leading normal, productive lives.

The TSA also became adept at winning grants to help fund research into the causes and treatment of Tourette Syndrome. In addition to their support for research and their educational program and media campaign, the TSA has also spearheaded patients’ rights legislation. One of their most notable successes in this area is the Orphan Drug Act, which enables drug companies to economically develop and win FDA approval for drugs to help patients with rare diseases. Before passage of the Orphan Drug Act, it had not been economically feasible for pharmaceutical companies to develop such medications.

Although its other work is undeniably significant, the educational function of the TSA remains its single most important activity. Public education about Tourette Syndrome is an ongoing process. Even today some individuals with Tourette Syndrome find themselves disadvantaged and oppressed by the ignorance of others.

For example, Tourette children are sometimes still expelled from classes or suspended from riding school buses because of what is perceived as willful misbehavior. But because of the TSA’s continued efforts to educate both patients and the public about the nature of this condition, TS children encounter such problems far less frequently than in the past, and when they do, they and their families are far more aware of their legal right to reasonable accommodation of their condition.

SIMPLE AND COMPLEX TICS

--Tics are classified as being simple or complex.

SIMPLE TICS: movements or vocalizations that involve only one muscle group and that are apparently meaningless

COMPLEX TICS: movements or vocalizations which make use of more than one muscle group or that appear to be meaningful

COMMON EXAMPLES OF SIMPLE TICS

MOTOR--blinking, head jerking, shrugging, facial contortions, licking, squinting, tongue thrusting, nose-twitching, foot tapping, ankle flexing, clapping, leg jerking.

VOCAL--Throat clearing, yelping, hissing, puffing, shouting, squealing, squeaking, sniffing, snorting, tongue clicking, coughing, grunting, gurgling.

COMMON EXAMPLES OF COMPLEX TICS

MOTOR: banging, kissing, jumping, hopping, pinching, somersaults, skipping, tearing things, throwing things, touching other people or things, smelling, twirling about, walking on toes, pulling clothes, hair twisting, copropraxia (engaging in obscene gestures or behaviors), and even (in some rare cases) self-injurious actions such as hitting or biting oneself.

VOCAL: uttering ordinary words or phrases out of context, echolalia (repeating a sound, word or phrase just heard), coprolalia (vocalizing socially unacceptable words), animal sounds, calling out, laughing, muttering to oneself, whistling, humming, palilalia (repeating one’s own words).




Written by Tina Blue - © 2002 Pagewise


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