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Dr. George Gilles de la Tourette

 

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Tourette's Syndrome

Tourette's Syndrome

Synonyms: Gilles de la Tourette syndrome; GTS

 

What is Tourette's Syndrome?

The syndrome is characterized by repetitive compulsive involuntary stereotyped movements or vocalizations termed 'tics'. In 1885, Dr. Georges Gilles de la Tourette described nine patients with motor (movement) and vocal tics, some of whom had echo phenomena (a tendency to repeat things said to them) and coprolalia (utterances of obscene or aggressive phrases). He incorrectly thought that the condition was likely related to a group of startle disorders that included "the jumping Frenchman of Maine," described by Beard in 1880.

The syndrome was initially considered to be a psychological or psychiatric one.

The observation in the 1960s that certain drugs called neuroleptics were effective in treating GTS refocusing attention from a psychological to an organic central nervous system cause. Tics are relatively common, occurring in up to 28% of children in some studies. The tics may be transient and last less than 1 year (Transiet tic disorder) or chronic (Chronic tic disorder). To be diagnosis with GTS further criteria have to be fulfilled including:1) a history or finding of multiple motor tics, (2) a history or finding of one or more sonic (vocal) tics, (3) onset prior to 18 years of age, (4) duration of more than 1 year, and (5) tics cause marked distress or significant impairment in daily functioning (American Psychiatric Association 1994).

 

Are tics the only sign of GTS?

GTS may be characterized just by the tics. Many children however, mostly male, with GTS have attention deficit - hyperactivity disorder characterized by a shortened attention span, distractibility, impulsivity, and hyperactivity.

Other associated problems include learning disabilities such as dyslexia, depression, anxiety disorders, phobias, antisocial behavior, sleep disorders, and personality disorders.

 

What causes GTS?

Studying affected families with GTS suggests that in the great majority of people the trait is inherited in what is called an autosomal dominant pattern with variable expression. This means that a person with GTS has a 50% chance of passing the trait on to their offspring, but that to what degree the offspring is affected varies widely. The inherited trait may not cause any disorder or may manifest as GTS, chronic multiple tic disorder and/or obsessive-compulsive disorder. Current evidence suggests that the underling problem is the overactivity of a brain chemical called  dopamine within a certain are of the brain (striatum). The observed benefit from drugs that are inhibitors of another brain chemical called serotonin suggests that this chemical  system may be the one disturbed for patients with associated obsessive-compulsive disorder.

 

How is it diagnosed?

There is no specific laboratory test for GTS. The diagnosis is made by your doctor from your story, family history and examination. Occasionally testing may be done to rule out other conditions which may mimic GTS.

 

How is it treated?

Although severe tics can be socially distressing, for most GTS patients the principal difficulties come not from tics but from the associated features of obsessive-compulsive symptoms, attention deficit hyperactivity disorder, and other behavioral disturbances.

Because many of the medications used to treat the tics may cause side effects of their own including chronic movement disorders, most patients are simply counseled and observed. This also allows for a better appreciation of the baseline disorder and the difficulties encountered at home, in school, at work, and with peers.

Occasionally medication is required to control the tics. The standard therapy is low doses of a group of drugs known as neuroleptics (e,g, haloperidol). These drugs can be quite effective but risk the development of chronic movement disorders. The complications of neuroleptics can be sometimes be avoided by using another class of medications called presynaptic catecholamine depletors (e.g. reserpine and tetrabenazine).

If you have GTS and you think that your tics require treatment see your doctor and consider your options carefully. Contact a support group and talk over treatments with other people in a similar situation.

 

Dr. R. O'Brien

_____________________

This data is provided for informational purposes only. It does not substitute for individualized advice from a qualified physician. Although attempts have been made to ensure the material is accurate and up to date it is provided in an 'as is' state. Neither the author nor neurology BC assumes any liability for errors or omissions or any problems that might arise due to them. Always consult your physician or qualified health professional before acting on information that concerns your health.

Further Reading

A Physician's Guide to the Diagnosis and Treatment of Tourette Syndrome. Bruun RD, Cohen D, Leckman J. , Tourette Syndrome Association Inc., New York, New York, © 1995

Other web links

Tourette Syndrome Foundation of Canada

Tourette Syndrome Association (USA)

NINDS Tourette Page

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This page last modified 08/21/08