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 and training funding from Allergan, F Hoffmann-La Roche, Medtronic Neuromodulation, Merz, Neurocrine  Biosciences, Nuvelution, Revance, and Teva and consulting/advisory board honorariums from Abide, Lundbeck, Retrophin, Parexel, Teva, and Allergan.

)
Originally released April 19, 1993; last updated April 23, 2019; expires April 23, 2022

Overview

Tourette syndrome is a chronic neurodevelopmental disorder consisting of motor and phonic 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. Although majority of patients have at least a partial remission in their tics after age 18 years, for most patients, Tourette syndrome is a lifelong condition, often associated with numerous behavioral comorbidities, including obsessive-compulsive disorder and attention deficit hyperactivity disorder. In this article, the author provides a succinct but thorough review of current knowledge regarding the phenomenology, pathogenesis, and treatment 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 Hospital in Paris (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 repeating other people s gestures (echopraxia). In addition, 5 of the 9 individuals were 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 that act by blocking dopamine receptors were effective in treating Tourette syndrome, and 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 (Jankovic and Kurlan 2011; Robertson et al 2017). Once considered a rare psychiatric curiosity, Tourette syndrome is now recognized as a relatively common 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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