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Gilles De La Tourette Syndrome - Tourette Syndrome


Overview, Causes, & Risk Factors

Tourette syndrome is an inherited disorder that causes a person to make involuntary movements and sounds.

What is going on in the body?

A person with Tourette syndrome doesn't show any unusual behavior early in life. During the teenage years, however, he or she may begin to blink a lot and make facial grimaces or twitches, called tics. The person may also begin to experience uncontrolled movements of the head and limbs.

Later, a person with Tourette syndrome will begin to make unusual sounds or blurt out inappropriate words. The words may be curse words and are often repeated several times.

What are the causes and risks of the disease?

Many cases of Tourette syndrome occur randomly, but other cases tend to run in families.

Tourette syndrome affects 1 in 2,500 males. Females are affected less frequently.


Symptoms & Signs

What are the signs and symptoms of the disease?

Symptoms of the disorder include making many involuntary movements. These include:

  • facial twitches, or tics
  • twitching or jerking of the head and shoulders
  • jerking of the arms and legs
  • Other symptoms include making involuntary sounds, such as:

  • grunts
  • yelping
  • coughs
  • hissing
  • loudly blurting out words that are inappropriate
  • An affected person may also have compulsive behavior, repeating a particular activity to relieve fear or anxiety.


    Diagnosis & Tests

    How is the disease diagnosed?

    Tourette syndrome is diagnosed by a healthcare provider who takes a careful history of the symptoms. The provider may also observe the tics and other inappropriate movements or vocal sounds.


    Prevention & Expectations

    What can be done to prevent the disease?

    Nothing can be done to prevent Tourette syndrome.

    What are the long-term effects of the disease?

    The effects of Tourette syndrome may vary over the course of the lives of affected persons. At times, the effects may almost disappear. Then, they return as pronounced as ever. Some affected persons are able to suppress the tics and sounds for very short periods of time.

    What are the risks to others?

    Tourette syndrome is not contagious. It is an inherited disorder that can be passed from affected persons to their children. Genetic counseling is valuable for the relatives of affected persons.


    Treatment & Monitoring

    What are the treatments for the disease?

    Various medications have been tried to treat Tourette syndrome. Haloperidol is used most often, but pimozide is also effective. These drugs may be used alone or with other drugs to treat specific symptoms of the disease.

    What are the side effects of the treatments?

    Persons being treated for Tourette syndrome may be depressed or drowsy or have blurred vision. Persons may also have less control over their regular, voluntary movements.

    What happens after treatment for the disease?

    Symptoms return if treatment is stopped.

    How is the disease monitored?

    Tourette syndrome is monitored by observation. If the syndrome is being treated with medications, the level of the drugs in the blood is monitored by blood tests for therapeutic drug levels.


    Attribution

    Author:Ronald J. Jorgenson, DDS, PhD, FACMG
    Date Written:
    Editor:Smith, Mary Ellen, BS
    Edit Date:06/11/00
    Reviewer:Eileen McLaughlin, RN, BSN
    Date Reviewed:05/07/01

    Sources

    Buyse ML: Birth Defects Encyclopedia. Blackwell Scientific Publishers, 1990.

    Goodman RM and Motulsky AG: Genetic Diseases among Ashkenazi Jews. Raven Press, 1979.