Tourette syndrome

"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, 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.

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

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.

Tourette Syndrome patients may exhibit symptoms of other conditions along with their physical or vocal tics, such as Attention Deficit Disorder (ADD), Attention Deficit Hyperactivity Disorder (ADHD), Obsessive-Compulsive Disorder (OCD), Non-OCD Anxiety Disorders, Executive Dysfunction, Depression, Bipolar Disorder, Autism Spectrum disorders including Asperger's Syndrome, "Rage Attacks," as well as Sensory Integration issues, and Sleep Disorders. Tourette's, when it occurs along with some of these other conditions, has been colloquially referred to as "Tourette Syndrome Plus", which was a term coined to explain that not all of the symptoms experienced by persons with tics are related to Tourette's syndrome, but may be more accurately explained by other diagnoses.

Studies have shown no correlation with tic severity and the onset of puberty, but multiple studies have shown that most tics remit or subside as one passes through adolescence. [1] Some well-known persons with Tourette's are Dr. Samuel Johnson, Jim Eisenreich, and Tim Howard. [2]

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 required that the disturbance must cause distress or impairment in the individual's normal 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.

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

As it is a spectrum disorder, the severity of the condition can range vastly. Those with mild cases are often highly functioning, so much so that others would 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.

Other commonly associated disorders are obsessive-compulsive disorder (OCD) and attention deficit hyperactivity disorder (ADHD).

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.

Researchers are also investigating the use of nicotine patches as a treatment. Researchers are seeking a substitute that can target brain disorders in the same way, without the risks. (BBC News)

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.

In any case, the condition in many individuals improves as they mature. Multiple studies have shown that most children improve with maturity. [3] [4] [5] 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. In a few cases, complete remission, as stated previously above, occurs after adolescence, and in most cases, tic severity subsides as one passes through adolescence. [6]

Living with TS, with a supportive environment and family, generally gives one survival 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!

Cognitive Behavioural 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.

Genetics

Recent research by Doctor Matthew State at Yale University suggests that Tourette Syndrome may be caused by an inversion defect on chromosome 13 of gene SLITRK1. This means that Tourette Syndrome can be inherited or caused by mutation. However, this research 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. [7]

Other genetic studies indicate tic disorders, including TS, are inherited as a dominant gene(s) that may produce varying symptoms in different family members. 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 behaviours 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, TS may not be inherited; these cases are identified as "sporadic" TS because a genetic link is missing.

Famous people With Tourette's

• Mahmoud Abdul-Rauf (formerly Chris Jackson) - former NBA player. Featured in the TS documentary Twitch and Shout.
• Jim Eisenreich - former major league baseball player.
• Lowell Handler - photo journalist. Narrates and featured in the TS documentary Twitch and Shout.
• Tim Howard - goalkeeper for Manchester United Football Club.
• Howard Hughes - aviator, film director, producer, hotel owner and at one time, one of the most wealthy people in the world; thought to have Tourette Syndrome Plus due to his compulsive behavior.
• Dr. Samuel Johnson, lexicographer, certainly had Tourette Syndrome as evidenced by the writings of James Boswell.
• Mike Johnston - relief pitcher for the Pittsburgh Pirates.
• Tobias Picker - composer
• Amy Wilensky - playwright/author.
• Michael Wolff - Jazz musician.

Those Who May Have Had Tourette Syndrome or Tourette Syndrome Plus

• Howie Mandel - actor. Some neurologists think may have Tourette Syndrome Plus due to his long-standing germ obsession.
• Howard Stern - Although Stern claims to have cured himself of the OCD he suffered with as a child, some experts suspect he may have had Tourette Syndrome with OCD - otherwise known as Tourette Syndrome Plus.
• Thelonious Monk - Some neurologists think may have had Tourette Syndrome.
• Wolfgang Amadeus Mozart - Some historians think Mozart may have had Tourette Syndrome, although many experts on Tourette Syndrome disagree (see external link below).

References in the entertainment industry

The entertainment industry has often depicted those with TS as being social misfits whose only tic is coprolalia, which has led to the general public's misunderstanding of TS sufferers 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. An infamous incident of disinformation about coprolalia and Tourette's involved Dr. Laura Schlessinger.

In fiction

• Matchstick Men's protagonist (Nicolas Cage) is a neurotic con artist with Tourette's and OCD.
• 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.
• 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 The Boondock Saints, an elderly pub bartender suffers from Tourette's,he constantly yells "fuck ass".
• 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 continously 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, Michael Sheen, Shirley Henderson. 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.
• 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.

On the Internet

• Danny, the subject of the website TourettesGuy.com, is promoted to have a form of Tourette's Syndrome. Although many believe this is not true, the videos on the website suggest that he may in fact have a form of Tourette's, but his alcohol problem (he's drinking in many of the videos) might have a great deal to do with his outbursts as well.

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 their 1993 Gold Against The Soul album titled "Symphony Of Tourette", the lyric being sung from the perspective of being a sufferer. The album sleeve also contains a brief description of the condition and its symptoms following the lyrics for the song.

External links

• The Tourette Syndrome Association Inc. - The US advocacy group.
• The Tourette Syndrome (UK) Association - The UK advocacy group.
• Tourette Syndrome Association of Australia - The AU advocacy group.
• Tourette Syndrome Foundation of Canada - The Canadian advocacy group.
• The TIC: Tourette's Information Center.
• Tourette Syndrome Online