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At a glance

Tourette Syndrome is a common neuropsychiatric disorder with onset in early childhood. It is characterized by multiple involuntary motor tics with a wax and wane pattern that can be suppressed temporarily. Affected individuals must present at least one vocal (phonic) tic. These tics are eye blinking, coughing, throat clearing, sniffing, and facial movements. The tics are often associated with vocalization. Intelligence and life expectancy are not affected by this condition.

Synonyms

Tourette Syndrome; Tourette’s; Brissaud Syndrome; Maladie des Tics; Tic Impulsif; Chronic Motor Tics; Syndrome des Tics Impulsifs.

History

Georges Albert Edouard Brutus Gilles de la Tourette was a French physician and neurologist (1857-1904) who described nine patients with this psychoneurologic disorder in 1885. The condition was named after Jean-Martin Charcot (1825-1893) on behalf of his resident who observed the condition. Gilles de la Tourette became Doctor in Paris in 1879. The onset is reported in childhood usually at 7 to 10 years of age. It is characterized by echolalia, palilalia, coprolalia, stuttering, and a craving to touch.

Incidence

Common; estimates range from 0.05 to 1% in the general population. Up to 20% of schoolchildren may have a transient tic disorder at some points. About 1% of school-age children and adolescents have Tourette’s. It was once considered a rare and bizarre syndrome, most often associated with coprolalia (the utterance of obscene words or socially inappropriate and derogatory remarks), but this symptom is present in only a small minority of people with Tourette’s.

Genetic inheritance

Controversial; probably autosomal dominant with incomplete penetrance and variable expression. Environmental factors seem to influence the risk, severity, and course of the disorder.

Pathophysiology

Tourette’s is defined as part of a spectrum of tic disorders, which includes provisional, transient, and persistent (chronic) tics. While the exact cause is unknown, it is believed to involve a combination of genetic and environmental factors. Postulated to be a result of dopamine excess or supersensitivity of the postsynaptic dopamine receptors in the basal ganglia and frontal cortex. Another hypothesis is a neurophysiologic deficit secondary to neurotransmitter abnormalities, resulting in failed inhibition of frontal-subcortical motor circuits.

Diagnosis

Diagnostic criteria for Gilles de la Tourette Syndrome recommended by the American Psychiatric Association include both multiple motor and vocal tics over a period of more than 1 year, voluntary suppression of symptoms, a waxing and waning course, and onset between 2 and 15 years of age.

Clinical aspects

Initial symptoms usually are involuntary, tic-like movements. The disorder progresses with development of echolalia (repeating another’s words or phrases), grunting, and coprolalia (use of obscene words). Most cases are mild and do not come to medical attention. Waxing and waning course, with one tic appearing and ...

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