Tourette's Disorder

Tourette's disorder is a rare disorder in which the child demonstrates multiple involuntary motor and vocal tics. A tic is a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalization.

Epidemiology

Tourette's disorder affects 0.4% of the population.
There is a 3: 1 male-female ratio.

Etiology

Tourette's disorder is highly familial and appears to frequently co-occur with obsessive-compulsive disorder. Despite evidence of genetic transmission in some families, no gene (or genes) has yet been discovered to explain the etiology of the disorder.

Clinical Manifestations

History, Mental Status and Physical Examinations
The patient or family usually describes an onset in childhood or early adolescence before age 18. Vocal tics are usually loud grunts or barks but can involve shouting words; the words are sometimes obscenities (coprolalia). The patient describes being aware of shouting the words, being able to exert some control over them, but being overwhelmed by an uncontrollable urge to say them. Motor tics can involve facial grimacing, tongue protrusion, blinking, snorting, or larger movements of the extremities or whole body.
Motor tics typically antedate vocal tics; barks or grunts typically antedate verbal shouts. The motor tics are not painful.

Differential Diagnosis

A careful neurologic evaluation should be performed to rule out other causes of tics. Wilson's disease and Huntington's disease are the principal differential diagnostic disorders. An EEG should be performed to rule out a seizure disorder. Careful evaluation for other comorbid psychiatric illnesses should be performed. Stimulants used to treat other psychiatric disorders may unmask tics.

Management

Treatment typically involves the use of low doses of high-potency neuroleptics such as haloperidol or pimozide. The child and his or her family should receive education and supportive psychotherapy aimed at minimizing the negative social consequences (e.g., embarrassment, shame, isolation) that occur with this disorder.

KEY POINTS 1. Tourette's disorder is a tic disorder. 2. It is rare and more common in males (3: 1). 3. When diagnosing Tourette's disorder, Wilson's and Huntington's diseases must be ruled out. 4. It is treated with high-potency neuroleptics and patient/family support.