TOURETTE'S SYNDROME

also called Tourette's Disorder, or Gilles De La Tourette's Syndrome, rare neurological disease characterized by recurrent motor and phonic tics (involuntary and purposeless muscle spasms and vocalizations). The syndrome occurs worldwide in all races, is usually inherited, and is three times more prevalent in males than in females. It is named for Georges Gilles de la Tourette, who first described it in 1885; it has been suggested that the English author Samuel Johnson may have suffered from a form of the disease, based on contemporary descriptions of his facial tics and of the strange vocalizations interrupting his normal speech. Tourette's syndrome usually begins between the ages of 2 and 15 and usually continues into adulthood. Motor tics occur first in about 80 percent of the cases, while compulsions to utter abnormal sounds precede motor tics in the remaining cases. Individuals with milder forms may exhibit either motor or phonic tics but not both. Coprolalia, the compulsion to utter obscenities, was once considered a characteristic symptom, but it is often absent. Echolalia (a compulsion to repeat words heard) and palilalia (spontaneous repetition of one's own words) are two distinctive symptoms of Tourette's syndrome. Other vocalizations that may occur include grunts, barks, hisses, whistles, and other meaningless sounds. Motor tics may be simple actions that are virtually unnoticeable. More complex tics usually involve the shoulders, head, and face and may include apparently intentional behaviour, such as hopping, clapping, and fist clenching. Sleep, intense concentration, and physical exertion tend to suppress the symptoms, while emotional stress exacerbates them. Unlike other forms of compulsive behaviour, Tourette's syndrome has a neurological rather than a psychiatric origin and may improve under treatment with psychoactive drugs; haloperidol has been the traditional agent, but pimozide and fluphenazine among the dopamine blockers and clonidine have also been effective. Abnormalities of one or more chemical neurotransmitters in the brain are implicated in Tourette's syndrome, but the disease's underlying cause remains uncertain, as does its relationship to other, similar disorders.