Gilles de la tourette syndrome


Neurological disorder characterized by facial grimaces and tics and movements of the upper body and grunts and shouts and coprolalia. There is some confusion about the correct name for this condition. The author’s complete family name is Gilles de la Tourette, and the correct term is thus Gilles de la Tourette’s syndrome, although in everyday speech it is commonly referred to as just «Tourette’s». The disease is also known as Brissaud’s disease, for the French physician Edouard Brissaud (1852-1909) who, in 1896, gave a more detailed description. Jean-Martin Charcot preferred the more euphonic eponym Gilles de la Tourette’s syndrome to the more factual maladie des tics - disease or syndrome with unvoluntary tics. Thus Gilles de la Tourette was ensured of perpetual glory. Part of the symptomatology was described by Jean Marc Gaspard Itard (1774-1838) in 1825 and by George Miller Beard (1839-1883) in 1880.

A rare psychoneurological disorder with onset in childhood, usually at the age of 7 to 10 years, but onset may occur in early adolescence. It is now assumed to be a neurological rather than a psychiatric disease. Symptoms are echolalia (the automatic repetition by lunatics of what is said to them; a sign of schizophrenia), pallilalia (repeating the sentences of others) and coprolalia (the compulsory saying of dirty words), a want for touch, and stottering.

A characteristic trait is lack of muscular coordination with unvoluntary and purposeless movements, which may consist of mild facials spasms and blinking with the eyes, and/or violent tics in eyes, head, arms, and legs, or other parts of the body. Often there is also echopraxia (involuntary mimicking of the movements of others), incoherent grunts and barks which may represent suppressed obscenities. Coprolalia only occurs in the presence of other people, and may by some patiens be camouflaged by coughing. Patients with this syndrome find it difficult to concentrate, are easily distracted, and tend to withdraw from social intercourse. Periodic cases occur. but it is usually chronic, lasting for life.

In puberty the obscenities may be annoyingly unsuppressed, with uncontrolled saying of all kinds of foul words. This aspect of the disease complicates social adjustment. There may also be sleep disturbances and self mutilation. The symptoms are absent during sleep and do not manifest in the patient’s written work.

The course of the illness varies. In some patients, the disorders tend to abate, whereas in others they progres to severe disability. Approximately 1/3 of cases are serious. The disease affects boys three times more often than girls. The syndrome occurs most frequently in Ashkenazie Jews and persons of Mediterranean extraction, rarely in black children. Transmission as an autosomal dominant trait is suspected.



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