Gilles de la Tourette syndrome

Tourette syndrome

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 gene has not yet been found.

The syndrome may be linked to problems in certain areas of the brain, and the chemical substances (dopamine, serotonin, and norepinephrine) that help nerve cells talk to one another.

Tourette syndrome can be either severe or mild. About 10% of Americans have a mild form. Many people with very mild tics may not be aware of them and never seek medical help.

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

Most people first notice symptoms of Tourette syndrome during childhood, between ages 7 and 10.

The most common first symptom is a facial tic. Other tics may follow. A tic is a sudden, rapid, repeated movement or voice sound (vocalization). Tics can include:

• Arm thrusting
• Eye blinking
• Jumping
• Kicking
• Repeated throat clearing or sniffing
• Shoulder shrugging

Tics may occur many times a day, but they tend to improve or get worse at different times. The tics may change with time.

Contrary to popular belief, use of curse words or other inappropriate words or phrases (coprolalia) occurs in only a small number of patients.

Many patients say that the tics are not totally out of their control (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) -- people with OCD feel as though they have to do the behaviors.

There are no lab tests to diagnose Tourette syndrome. However, a health care provider should do an examination to rule out other causes of these symptoms.

To be diagnosed with Tourette syndrome, a person must:

• Have had many motor tics and one or more vocal tics at some time, although not necessarily at the same time.
• Have tics that occur many times a day, nearly every day or on-and-off, for a period of more than 1 year. During this period there must not be a tic-free period of more than 3 months in a row.
• Have started the tics before age 18.

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

Doctors have prescribed medicines called antipsychotics to treat Tourette syndrome. These medicines can help control or reduce tics, but they have side effects such as movement disorders and cognitive dulling. Anti-epileptic medications are also used sometimes.

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.

Symptoms of Tourette syndrome can range from almost unnoticeable minor movements (such as grunts, sniffling, or coughing) to continuing, uncontrollable movements and sounds (vocalizations).

The symptoms tend to get better and worse. 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.

People with Tourette syndrome have a normal life expectancy.

Conditions related to Tourette syndrome include:

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

These conditions need to be diagnosed and treated.

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.

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.

Goetz CG. Goetz: Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders; 2007.