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"Tourette's" redirects here. For the Nirvana song, see Tourette's (song).

Tourette syndrome
Specialty	Neurology

Tourette syndrome — also called Tourette's syndrome, Tourette's disorder, TS, or Gilles de la Tourette syndrome (GTS) — is a movement disorder with onset in childhood, and characterized by the presence of multiple motor tics and at least one phonic tic, which characteristically wax and wane. Tourette syndrome was once considered a rare and bizarre syndrome. It is no longer considered rare, but is often undetected because of the wide range of severity.

The eponym was bestowed by Jean-Martin Charcot after and on behalf of his resident, Georges Gilles de la Tourette, (1859 - 1904), a French physician and neurologist, who published an account of nine patients with Tourette's in 1885.

Symptoms

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The hallmark of Tourette's syndrome are repetitive, involuntary movements and utterances that frequently change in number, frequency, severity, and anatomical location. Tics can be thought of as fragments of normal motor and vocal movements and sounds that occur repetitively and unvoluntarily.

Tics are classified in several ways: simple vs. complex, and motor vs. phonic. Motor tics are movement-based tics affecting discrete muscle groups. Phonic tics are involuntary sounds produced by moving air through the nose, mouth, or throat. They may be alternately referred to as verbal tics, vocal tics, or phonic tics, but some diagnosticians prefer the term phonic tics, to reflect the notion that the vocal chords are not involved in all tics that produce sound. Simple motor tics are typically sudden, brief, meaningless movements, such as eye blinking or shoulder shrugging. Complex motor tics are typically more purposeful-appearing and of a longer nature. Motor tics can be of an endless variety and may include hand-clapping, neck stretching, and facial grimacing. A simple phonic tic can be almost any possible sound or noise, with common vocal tics being throat clearing, coughing, sniffing, grunts, or moans. Complex 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). However, according to the Tourette Syndrome Association, Inc., only about 10% of TS patients exhibit this symptom. Complex tics are rarely seen in the absence of simple tics.

The tics of Tourette's, in contrast to the movements of some other movement disorders, are temporily suppressible and preceded by a premonitory urge. The ability to suppress tics varies among individuals, and may be more developed in adults than children. Sometimes, a person with Tourette's can feel as if their Tourette's Syndrome has totally taken control of their body, and they almost feel as if they are standing outside of themselves. Sometimes a person with TS may not feel the urge to tic at all, but most of the time the urge to tic is somewhere in the middle of two extremes.

Immediately preceding tic onset, individuals with TS experience what is called a "premonitory urge," similar to the feeling of needing to breathe after holding one's breath for a long time. 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.

People with TS can suppress their tics, but doing so is like putting a lid on a boiling pot of water. Tics are experienced as irresistible 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.

Children with Tourette's may suffer socially if their tics are seen as weird or bizarre. It is common for children to suppress tics during a visit to the doctor or while at school. Typically, tics increase as a result of high energy emotions, which can include negative emotions, such as anxiety, but positive emotions as well, such as excitement. Relaxation may also result in a tic decrease, while concentration in an absorbing activity may lead to a decrease in tics. In fact, neurologist and writer Oliver Sacks has described a man with severe TS who is both a pilot and a surgeon.

Tourette Syndrome patients may exhibit symptoms of other conditions along with their physical or vocal tics. Associated conditions include attention-deficit hyperactivity disorder (ADD and ADHD), Obsessive-Compulsive Disorder (OCD), learning disabilities and sleep disorders.

Diagnosis

According to the DSM-IV-TR, TS is indicated when a person exhibits both multiple motor and one or more vocal tics (although these do not need to be concurrent) over the period of 1 year, with no more than 3 consecutive tic-free months. Previous versions of the DSM included a requirement for distress or impairment in social, occupational, or other important areas of functioning, but this requirement has been removed from the most recent version of the DSM, in recognition that not everyone with the diagnosis has distress or impairment to functioning. The onset must have been before the age of 18, and cannot be attributed to the use of a substance or another medical condition. Hence, other medical conditions which include tics or tic-like movements (such as autism) must be ruled out before conferring a Tourette's diagnosis.

Around 4 or 5 people in 10,000 have Tourette's Syndrome. It is estimated that as many as 1 in 200 experience some form of tic disorder, which includes transient tics, chronic tics, or Tourette's Syndrome. Males are affected 3 to 4 times more often than females. Most cases decrease in severity or cease entirely upon reaching adulthood.

Prognosis

Tourette's syndrome is a spectrum disorder, which means that the severity of the condition can range widely. Those with mild cases are often minimally impacted by symptoms, to the extent that others might not know of their condition. Severe cases (which are the rare minority) can inhibit or prevent the individual from engaging in common activities such as holding a job, having a fulfilling social life, or maintaining his/her basic needs.

Multiple studies have demonstrated that the condition in most children improves with maturity. Tics may be at their highest severity at the time that they are brought to diagnostic awareness, and often improve with understanding of the condition. The statistical age of highest tic severity is typically between 8 and 12, with most individuals experiencing steadily declining tic severity as they pass through adolescence. The same studies have shown no correlation with tic severity and the onset of puberty. In many cases, complete remission of tic symptoms occurs after adolescence. Regardless of symptoms, individuals with TS can expect to live a normal life span. Although TS symptoms may be lifelong and chronic for some, it is not a degenerative condition and is not life-threatening.

Living with TS, with a supportive environment and family, generally gives one skills to manage the disorder. Many persons with Tourette's syndrome have learned to cover-up the more socially inappopriate tics or use them to their advantage - such as in the case of musicians. Some drummers, in particular, have found that tics give them a certain 'flair' or 'special sound' to their drumming.

Treatment

Knowledge and understanding is the best treatment available. There are no medications specifically designed to target the symptoms of Tourette's, and most available medications are associated with adverse side effects. The majority of people with TS require no medication, but medication is available to help when symptoms interfere with functioning. TS medications are only able to help reduce specific symptoms. Neuroleptic and antihypertensive drugs can have long- and short-term adverse effects, and use of stimulants is controversial. Current prescribed stimulant medications include: methylphenidate (Ritalin®, Metadate®, Concerta®), dextroamphetamine (Dexedrine®), and mixed amphetamine salts (Adderall®). The alpha 2-adrenergic agonists include clonidine (Catapres®) and guanfacine (Tenex®). SSRIs, a class of antidepressants, may be prescribed when a TS patient is triggered by symptoms of OCD.

Cognitive Behavioral Therapy (CBT) can be used to try to disrupt the automatic chain of events underlying the tics. Relaxation techniques may also be useful in relieving the stress that may aggravate tics.

Nicotine

Researchers also investigated the use of nicotine patches as a treatment. Neal Swerdlow, MD, PhD, and Chairman of the Tourette Syndrome Association Scientific Advisory Board, explains that nicotine showed preliminary promise in case reports, but these effects were not reproduced in well-controlled trials several years later. Studies of nicotine derivatives (mecamylamine, inversine) also showed that they were not effective as monotherapy for the symptoms of Tourette's. Researchers are seeking a substitute that can target brain disorders in the same way, without the risks. (BBC News)

Neuroleptic drugs

Neuroleptic anti-psychotic drugs, such as Haldol or Orap, have historically been and continue to be the most common drugs prescribed to control tics. The drugs work by blocking dopamine receptors. However, these drugs are neurotoxic and have caused permanent brain damage to some people taking them, sometimes as severe as Tardive dyskinesia, which develops in 4% of the people under the age of 45 taking Haldol every year, and to an even higher percentage of older people taking Haldol. Haldol#side-effects.

New neuroleptic atypical antipsychotics are believed to cause Tardive dyskinesia "somewhat less frequently" but "they may cause serious metabolic disorders, e.g., diabetes, frequently enough to make them equally dangerous."

Beta Blockers

Beta Blocker drugs, such as Clonidine pills, work on about a third people with TS. Dr. David Comings, who had a Tourette' Syndrome and ADHD clinic in Southern California, had a higher rate of success with Clonidine patches, but other doctors have not reported the same rate of sucess with Clonidine patches that Dr. Comings did. Originally developed to treat high blood pressure, beta-blockers could be a safer alternative to neuroleptics drugs for the one-third of the people with TS that respond to them. Side effects can include sedation, fatigue, headaches and dizziness. Guanafacine, another beta blocker, may be just as useful as Clonidine in treating TS.

Marijuana

Some people with Tourette's syndrome report that marijuana helps reduce tics. Very little research has been done on smoked marijuana. Research into three case reports showed that Marijuana helped reduce tics, although authors cautioned that subjects may have been reporting a reduction in anxiety rather than tics. In an unblinded, uncontrolled study, other researchers interviewed 13 people with TS and found that 85% of them reported that marijuana caused a marked improvement in their symptoms. Study authors report that longer-term controlled studies are needed to validate results.

Marinol Treatment

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Dronabinol, synthetic THC sold in pill form (brand name Marinol has shown some promise in treating Tourette's Syndrome:

Researchers in Texas published a study that showed that Marinol and nicotine can be used as an effective adjunct to neuroleptic drugs in treating TS.

In 1998 the Tourette's Syndrome Association funded research to see if Marinol treatment alone was effective for treating tics. (TSA Research Awards) The studies were done in Germany.

The German studies showed promise for Marinol treatment for TS. Research on twelve patients showed that Marinol reduced tics with no significant adverse effects.(Muller-Vahl)

The Germans then did a larger and longer six-week controlled study to determine if Marinol worked to reduce tic severity. The results showed the patients taking Marinol had a significant reduction in their tic severity and Marinol did not cause serious adverse effects. The longer TS patients took the Marinol, the more significant the reduction in their tic severity became, in comparison to the placebo group.

Research was done to address the concern that Marinol could cause long term cognitive impairment. In a study to see if Marinol impaired the cognition of TS Patients taking after taking Marinol for 6 weeks, both during the 6 weeks they were taking Marinol and the five to six weeks afterwards. According to the researchers "no detrimental effect was seen on learning curve, interference, recall and recognition of word lists, immediate visual memory span, and divided attention. Measuring immediate verbal memory span, we even found a trend towards a significant improvement during and after treatment."

Some researchers hypothesized that Tourette's may be caused by mutations in the cannibinol receptors. ) Further research showed that this was not true. The authors of the studies state that well-controlled studies of longer duration on larger groups are warranted. The TSA has not funded subsequent studies on Marinol.

Dr. Kate Kompoliti, a member of TSA's Scientific Advisory Board, stated in the TSA newsletter in 2003 moted that Marinol is addictive and that she is concerned that Marinol may lead to long term cognitive impairment or selective long-term memory deficits. However, the rxlist says that addiction is "very unlikely to occur," and it does not say that there is a possibility of long term cognitive impairment, selective long-term memory deficits or anything similar could be caused by Marinol.

Brain Surgery

In February 2004, surgeons in the US successfully carried out a brain surgery in which tiny electrodes, powered by batteries inserted in the chest, were placed beside the thalamus in each cerebral hemisphere. Within half a minute of activating the electrodes, the patient could walk normally and displayed a complete lack of symptoms. This surgery is not a cure; it is regarded as an experimental and dangerous procedure, and is unlikely to become widespread. TSA

Genetics

Genetic studies indicate tic disorders, including TS, are inherited as a dominant gene(s) that may produce varying symptoms in different family members. Tourette Syndrome is thought to be polygenic (the result of several genes), although the gene(s) have not yet been identified. It is also a condition of variable penetrance, meaning even family members with the same genetic makeup may show different levels of symptom severity. Non-genetic, environmental factors do not cause Tourette's, but they can impact upon the expression of the severity of the disorder. As one example of non-genetic factors, twin studies have shown that the lower birth weight twin is the one more likely to display more symptoms.

A person with TS has about a 50% chance of passing the gene(s) to one of his/her children. However, the gene(s) may express as TS, as a milder tic disorder, or as obsessive compulsive symptoms with no tics at all. It is known that a higher than usual incidence of milder tic disorders and obsessive compulsive behaviors are more common in the families of TS patients. The sex of the child also influences the expression of the gene(s). The chance that the child of a person with TS will have the disorder is at least three times higher for a son than for a daughter. Yet only a minority of the children who inherit the gene(s) will have symptoms severe enough to ever require medical attention.

In some cases, tics may not be inherited; these cases are identified as "sporadic" TS (also known as tourettism) because a genetic link is missing.

Recent research by Doctor Matthew State at Yale University suggests that a small number of Tourette Syndrome cases may be caused by an inversion defect on chromosome 13 of gene SLITRK1. Some cases of tourettism (tics due to reasons other than inherited Tourette's syndrome) can be caused by mutation. The finding of a chromosomal abnormality appears to apply to a very small minority of cases (1 - 2%), and studies to locate all of the genes implicated in Tourette's syndrome are ongoing.

Famous people with Tourette's

• Mahmoud Abdul-Rauf (formerly Chris Jackson) - former NBA player.
• Jim Eisenreich - former major league baseball player.
• Tim Howard - goalkeeper for Manchester United Football Club.
• Dr. Samuel Johnson, lexicographer, certainly had Tourette Syndrome as evidenced by the writings of James Boswell.
• Mike Johnston - relief pitcher for the Pittsburgh Pirates.
• Andre Malraux - French author, adventurer and statesman.
• Tobias Picker - composer
• Jeremy Stenberg - Motocross rider (nicknamed "Twitch")
• Michael Wolff - Jazz musician.

• Benjamin Simkin, M.D., endocrinologist emeritus at Cedars-Sinai Medical Center, Los Angeles, in his book "Medical and Musical Byways of Mozartiana," put forth an arguement that Mozart may have had Tourette Syndrome. Tourette syndrome experts and neurologists disagree.

References in the entertainment industry

The entertainment industry has often depicted those with TS as being social misfits whose only tic is coprolalia (the involuntary utterance of inappropriate or taboo phrases), which has led to the general public's misunderstanding of persons with Tourette's as "people who can't help yelling swear words a lot". However, this is merely a clinomorphism, as coprolalia is a relatively rare symptom compared to other types of tics, and severe Tourette's in adulthood is rare. Talk shows, like Dr. Phil, have furthered this stigmatization, focusing on rare and sensational aspects of the condition. An infamous incident of disinformation about coprolalia and Tourette's involved Dr. Laura Schlessinger.

In documentary

• HBO has produced an acclaimed documentary, "I Have Tourette's but Tourette's Doesn't Have Me," featuring children between the ages of 6-13, and addressing what it's like to grow up with Tourette's syndrome.

• "John's not Mad" - A documentary about the daily life of a Scottish teenager who suffers from an extreme case of Tourette's Syndrome and coprolalia.

• "What Made Mozart Tic" - A documentary by composer and Tourette's Syndrome sufferer James McConnel which argues that Mozart did have Tourette's Syndrome.

In fiction

• An episode of the television show Quincy, M.E. has Quincy arguing with the drug companies, lawyers and the Food and Drug Administration to promote research into the syndrome. This was a seminal moment in the history of Tourette's, as it led to many people who didn't know what they had was Tourette's getting a name for their symptoms.
• The Tic Code stars Gregory Hines as a saxophone player with TS who befriends a 10 year old boy who wants to be a jazz pianist (and also has TS). Written by Polly Draper, and produced with her husband Michael Wolff who has Tourette's in real life.
• Matchstick Men's protagonist (Nicolas Cage) is a neurotic con artist with Tourette's and OCD.
• In Niagara, Niagara, Robin Tunney plays a unconventional girl with TS who goes on a road trip with a guy she meets in a drugstore.
• In Wedding Crashers, John Beckwith (Owen Wilson) excuses Jeremy Grey's (Vince Vaughn) cursing at a wedding as a case of Tourette's.
• In The Wedding Singer, when Adam Sandler's character states that his nephew "...might have Tourette's, we're looking into it" after the aformentioned young nephew walks up to his Adam's finacee and says "Linda, you're a bitch"
• In Curb Your Enthusiasm, Season 3, Episode 10, "The Grand Opening", with only days until the opening of his new Restaurant, Larry hires a new chef with Tourette's syndrome
• In Not Another Teen Movie, a girl who tries out for the cheerleading squad has Tourette's.
• In The Big White, the wife has Tourette syndrome.
• In Deuce Bigalow: Male Gigolo, one of Deuce's (Rob Schneider) crazy dates is a character with Tourette Syndrome who continuously yells curse words to people on the street.
• In What About Bob, Bob (Bill Murray) pretends to have Tourette Syndrome
• In The New Guy, Dizzy Harrison / Gil Harris (DJ Qualls) has tourette syndrome.
• In Dirty Filthy Love, tells the story of Mark Furness (Michael Sheen) with Obsessive Compulsive Disorder (OCD) and Tourette's negotiating his way through divorce, his best friend's matchmaking efforts and a woman who introduces him to therapy, filth and unconditional love.
• In The Simpsons, Season 4, Episode 7, "Marge Gets a Job", Ms Krabappel is recounting all the diseases and illnesses Bart has claimed to have to excuse himself for a test. The last excuse she says "and that unfortunate case of Tourette's Syndrome" to which Bart tries to pretend he still might have it by cursing and rambling. The mention of Tourette's caused many complaints upon the episode airing and the line was changed to "and that unfortunate case of Rabies". It has been claimed that the syndicated version was changed back to the original line but the episode on The Simpsons DVDs season 4 set retained the rabies line.
• Marty Fisher in Shameless has Tourette's.
• The main character, and narrator, of the novel Motherless Brooklyn by Jonathan Lethem has Tourette syndrome.
• Ron Silver's character in episode 7x12 of the The West Wing claims Josh Lyman, "has the political equivalent of Tourette's Syndrome."

On the Internet

• Hiding Tics to Gain Friends CBS News
• Documentary looks at Tourette's Syndrome News 10 Now
• Living with Tourette Syndrome The Insider

In music

• The grunge band Nirvana recorded a song on the In Utero album titled "tourette's"—a song with the lyrics intensely shouted rather than sung, perhaps to mimic the syndrome.

• UK Britpop band Manic Street Preachers recorded a song on the Gold Against The Soul album titled "Symphony Of Tourette", the lyric being sung from the perspective of a person with Tourette's. The album sleeve also contains a brief description of the condition and its symptoms following the lyrics for the song.

In upcoming films

• Oscar-winning actor Edward Norton announced that he was going to write, direct and star in a film adaptation of Motherless Brooklyn in 1999 right before Fight Club was released. As of January 2006, the movie has not even started filming. In May 2005 Norton said he was "almost finished" writing the screenplay and the movie "might" be made in 2006.

External links

• The Tourette Syndrome Association, Inc. - A New York based advocacy group.
• The Tourette Syndrome (UK) Association - A UK advocacy group.
• Tourette Syndrome Association of Australia - An AU advocacy group.
• Tourette Syndrome Foundation of Canada - A Canadian advocacy group.
• Tourette Scotland - A Scottish support & information group.
• Tourette Syndrome Plus - A website on Tourette Syndrome plus comorbid conditions.
• Tourette's Syndrome: minimizing confusion - A Tourette's Syndrome blog written by Roger D. Freeman, M.D.
• Tourette Spectrum Disorder Association, Inc A US advocacy group for Tourette Syndrome and co-occurring conditions.

• Eponymous diseases
• Neurological disorders