Chorea is a manifestation of a number of neurologic disorders in childhood: Sydenham chorea is one of the most representative. Over the past decade, chorea has been increasingly seen in a number of conditions that affects children. In this article, the author presents the most common neurologic disorders for which chorea is or may be one of the most prominent manifestations in individuals younger than 18 years of age. New information has been added that should be considered with presentation of chorea.
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• Sydenham chorea should be suspected in children between 5 and 15 years of age presenting with chorea, although there is no single diagnostic test.
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• Autoimmune-related chorea such as anti-NMDA receptor encephalitis, systemic lupus erythematosus, and the antiphospholipid syndrome are important causes of chorea in children and are frequently present, along with encephalitis.
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• In case of paroxysmal episodic chorea in children, gene mutations in ADCY5, PRRT2 (paroxysmal kinesigenic dyskinesia), MR-1 (paroxysmal nonkinesigenic dyskinesia), and GLUT1 (paroxysmal exercise-induced dyskinesia) should be suspected.
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• Huntington disease and dentatorubral-pallidoluysian atrophy are neurodegenerative disorders classically associated with chorea in adults; however, in children, they usually present with seizures, parkinsonism, and psychomotor regression rather than chorea.
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• A review of recent drug administration is also recommended in children with chorea, due to the possibility of withdrawal emergent syndrome.
Historical note and terminology
Chorea consists of brief, involuntary, irregular, quasi-purposive, rapid movements that flow from one body part to another without a rhythmic pattern.
It is defined as an ongoing random-appearing sequence of one or more discrete involuntary movements or movement fragments. As opposed to dystonia, choreiform movements are random, unpredictable, more rapid, and continuously ongoing and are not triggered by voluntary attempts. As a result, children with chorea will appear to be in constant motion or fidgety. Comparing chorea to athetosis, chorea presents as a sequence of brief and discrete movements, giving the appearance of jerking, whereas athetosis results in a more flowing, sinuous, and continuous movement. Myoclonus, on the other hand, is differentiated from chorea by the quickness of all of its movements and the consistency of muscular involvement (155).
The term chorea derives from the Greek word for dancing and was initially applied to epidemics of “dancing mania” in the middle ages. Many such dances were described; chorea Sancti Viti was the most renowned one. Sydenham used this term to describe rheumatic chorea in his Schedula Monitoria in 1686.
In 1686 Thomas Sydenham first described the clinical syndrome that now bears his name. Originally, Sydenham described rheumatic fever by its articular manifestations, but he failed to connect it with the chorea. It was not until 1831 that Richard Bright identified the link between rheumatic fever and the dancing movements of “St. Vitus’ dance,” now known as rheumatic chorea (62). In 1889 Cheadle described the full rheumatic syndrome of carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum. Several decades later, epidemiologic and microbiological studies led to the modern understanding of the etiologic role of streptococcal infection in rheumatic fever.