Gilles de la Tourette syndrome

Definition

Gilles de la Tourette syndrome is a nervous system disorder which causes a person to make repeated and involutary movements and sounds (vocalizations) called tics. The disorder is commonly called Tourette syndrome.

Alternative Names

Causes

Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. There is strong evidence that Tourette syndrome is passed down through families, although the specific gene has not yet been identified. Some evidence suggests that the syndrome is linked to problems in certain areas of brain and the chemical substances (dopamine, serotonin, and norepinephrine) that help nerve cells talk to one another.

About 200,000 Americans have the most severe form of Tourette syndrome. About 10% of Americans have a milder form. However, actual numbers may vary, since many persons with very mild tics may be unaware of them and never seek medical attention.

Tourette syndrome is four times as likely to occur in boys as in girls.

Famous people with Tourette syndrome include Mahmoud Abdul-Rauf (formerly Chris Jackson), Jim Eisenreich, and Mozart.

Symptoms

Symptoms of Tourette syndrome are usually first noticed during childhood, usually between ages 7 and 10.

The most common initial symptom is a facial tic -- other tics may follow. A tic is a sudden, rapid, repeated movement or voice sound (vocalization). Tics can include eye blinking, repeated throat clearing or sniffing, arm thrusting, kicking, shoulder shrugging, or jumping.

Tics may occur many times a day, but they tend to improve or worsen at different times. The nature of the tics may vary and change with time.

Contrary to popular belief, use of socially inappropriate words or phrases (coprolalia) occurs in only a minority of patients.

Many patients say that the tics are not totally involuntary, but that "things just would not feel right" if they did not do them. This is what makes Tourette syndrome different from obsessive-compulsive disorder (OCD) -- persons with OCD are compelled to do the behaviors out of fear of impending doom.

Exams and Tests

There are no specific lab tests to confirm a diagnosis of Tourette syndrome. However, a thorough examination by a qualified physician should be performed to rule out other causes of these symptoms.

Diagnostic Criteria:

• Both multiple motor and one or more vocal tics have been present at some time, although not necessarily at the same time.
• Tics occur many times a day, nearly every day or intermittently, throughout a period of more than 1 year. During this period there must not be a tic-free period of more than 3 consecutive months.
• The tics started before age 18.

Treatment

Many patients with Tourette syndrome have very minor symptoms. In this case, they are not treated, since the medications have side effects that would be worse than the symptoms.

Medicines called antipsychotics have historically been used to treat Tourette syndrome. These medicines can help control or reduce tics, but they are associated with side effects such as movement disorders and cognitive dulling.

A blood pressure medicine called clonidine has been shown to help control tics. Another drug commonly used is tetrabenazine, but this drug is also linked to movement disorders as well as depression. Many other treatments have been tried with little or no improvement.

Support Groups

Tourette Syndrome Association - www.tsa-usa.org

Outlook (Prognosis)

It is important to note that symptoms of Tourette syndrome vary widely and range from nearly unrecognized minor movements (such as grunts, sniffling or coughing) to persistent, involuntary movements and vocalizations.

The severity and nature of the symptoms typically come and go. Symptoms usually get worse before the mid-teen years. Most patients improve in early adulthood. Although 25% of patients may be symptom-free for a few years, only 8% of patients have symptoms completely go away without returning.

Persons with Tourette syndrome have a normal life expectancy.

Possible Complications

Conditions associated with Tourette syndrome include:

• Obsessive-compulsive disorder
• Attention deficit hyperactivity disorder (ADHD)
• Anger control issues
• Poor social skills

These conditions need to be identified and addressed accordingly.

When to Contact a Medical Professional

Make an appointment with your health care provider if you have tics that are severe or persistent, or if they interfere with your daily life.

Prevention

There is no known prevention.

Singer HS, Szymanski S, Giuliano J. Elevated intrasynaptic dopamine release in Tourette's syndrome measured by PET. Am J Psychiatry. 2002 Aug;159(8):1329-36.