What is Tourette Syndrome?

Tourettes Syndrome is an inherited, neurological disorder characterized by repeated involuntary movements and uncontrollable vocal sounds called tics. In some cases, such tics can include inappropriate words and phrases.

This syndrome is named for Dr. Georges Gilles de la Tourette, a pioneering French neurologist who, in 1885, described the condition in an 86-year-old French noblewoman.

The natural course of Tourettes Syndrome varies from patient to patient. Although Tourettes symptoms range from very mild to quite severe, the majority of cases fall in the mild category.

What are the symptoms?

The first symptoms of Tourettes Syndrome are usually facial tics. Often seen first is eye-blinking, however, facial tics can also include nose twitching or grimaces. With progression of the disorder, other motor tics may appear such as head jerking, foot stamping, or body twisting and bending.

It is not uncommon for a person with Tourettes Syndrome to continuously clear his or her throat, cough, sniff, grunt, yelp, bark, or shout. Patients may even utter strange and unacceptable sounds, words, or phrases.

People with Tourettes Syndrome may involuntarily shout obscenities (coprolalia) or constantly repeat the words of other people (echolalia). A few patients with severe Tourettes Syndrome demonstrate self-harming behaviors such as lip and cheek biting and head banging against hard objects. However, these behaviors are extremely rare.

How are tics classified?

There are two categories of tics: simple and complex.

What causes TS?

Although the basic cause of Tourettes Syndrome is unknown, current research suggests that there is an abnormality in the gene(s) affecting the brain's metabolism of neurotransmitters such as dopamine, serotonin, and norepinephrine. Neurotransmitters are chemicals in the brain that carry signals from one nerve cell to another.

How is Tourettes Syndrome diagnosed?

Generally, Tourettes Syndrome is diagnosed by observing the symptoms and evaluating family history. For a diagnosis of Tourettes Syndrome to be made, both motor and phonic tics must be present for at least 1 year. Neuroimaging studies, such as magnetic resonance imaging (MRI), computerized tomography (CT), and electroencephalogram (EEG) scans, or certain blood tests may be used to rule out other conditions that might be confused with TS. However, Tourettes Syndrome is a clinical diagnosis. There are no blood tests or other laboratory tests that definitively diagnose the disorder.

In many cases, parents, relatives, friends, or even the patients themselves become aware of the disorder based on information they have heard or read in the popular media.

What is the treatment for Tourettes Syndrome?

Because symptoms do not impair most patients and development usually proceeds normally, the majority of people with Tourettes Syndrome require no medication. However, medications are available to help when symptoms interfere with functioning. Unfortunately, there is no one medication that is helpful to all persons with TS, nor does any medication completely eliminate symptoms; in addition, all medications have side effects.

Some patients who require medication to reduce the frequency and intensity of the tic symptoms may be treated with neuroleptic drugs such as haloperidol and pimozide. These medications are usually given in very small doses that are increased slowly until the best possible balance between symptoms and side effects is achieved.

Clonidine, an antihypertensive drug, is also used in the treatment of tics. Studies show that it is more effective in reducing motor tics than reducing vocal tics. Fatigue, dry mouth, irritability, dizziness, headache, and insomnia are common side effects associated with clonidine use. Fluphenazine and clonazepam may also be prescribed to help control tic symptoms.

Other types of therapy may also be helpful. Although psychological problems do not cause TS, psychotherapy may help the person better cope with the disorder and deal with the secondary social and emotional problems that sometimes occur. Psychotherapy does not help suppress the patient's tics.

What is the prognosis?

There is no cure for TS; however, the condition in many individuals improves as they mature. Individuals with Tourettes Syndrome can expect to live a normal life span. Although the disorder is generally lifelong and chronic, it is not a degenerative condition. Tourettes Syndrome does not impair intelligence.

Tics tend to decrease with age, enabling some patients to discontinue using medication. In a few cases, complete remission occurs after adolescence. Although tic symptoms tend to decrease with age, it is possible that neuropsychiatric disorders such as depression, panic attacks, mood swings, and antisocial behaviors may increase.

References:
National Institutes of Health
Tourette Syndrome; Iowa Health Book: Psychiatry University of Iowa Department of Psychiatry
MRI of Tic Suppression in Tourette Syndrome. Abstracts: Arch Gen Psychiatry