Tourette syndrome

Joseph Jankovic MD (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released April 19, 1993; last updated March 6, 2017; expires March 6, 2020

Overview

Tourette syndrome is a chronic disorder consisting of motor and sonic tics. The onset is in childhood, and as many as 2% of children are affected, but epidemiological studies have shown that 20% to 30% of children exhibit tics at some time during childhood. Its peak severity usually occurs just prior to adolescence. For most patients, Tourette syndrome is a lifelong condition. It is associated with numerous neurologic comorbidities, most importantly obsessive-compulsive disorder and attention deficit hyperactivity disorder. In this article, the author provides a succinct but thorough review of current knowledge regarding the etiology and pathogenesis of Tourette syndrome.

Key points

 

• Tourette syndrome is a neurobehavioral disorder chiefly manifested by motor and phonic tics.

 

• Most patients with Tourette syndrome have a variety of behavioral comorbidities, including obsessive-compulsive behavior, attention deficit disorder with or without hyperactivity, and impulse control disorder.

 

• Although no consistently present gene mutation has been identified, Tourette syndrome is considered a genetic disorder, often inherited bi-lineally (from both parents).

 

• Treatment of Tourette syndrome must be individualized and tailored to the needs of each patient.

Historical note and terminology

Georges Gilles de la Tourette was a French neurologist, a trainee of Charcot at the Salpetriere (Kramer and Daniels 2004). In 1885 he described 9 patients with motor and phonic tics, some of whom had echo phenomena, repeating other people's words and phrases (echolalia) and uttering or shouting obscenities and profanities (coprolalia). He considered the condition to be closely related to a group of startle disorders that included "the jumping Frenchmen of Maine,” described by Beard in 1880. For many years, the etiology of Tourette syndrome was ascribed to psychogenic causes. Observations were made in the 1960s that neuroleptic drugs were effective in treating Tourette syndrome; this refocused attention from a psychological etiology to an organic central nervous system etiology. Although Gilles de la Tourette himself believed this condition was hereditary, it was not until the late 1970s that a familial, genetic etiology for Tourette syndrome was fully recognized (Albin and Mink 2006; Leckman et al 2006; Jankovic and Kurlan 2011). Once considered a rare psychiatric curiosity, Tourette syndrome is now recognized as a relatively common and complex neurobehavioral disorder. There has been speculation that many notable historical figures, including Dr. Samuel Johnson and possibly Wolfgang Amadeus Mozart (Ashoori and Jankovic 2007), were afflicted with Tourette syndrome.

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