Chorea in childhood

Kimon Bekelis MD (Dr. Bekelis of Dartmouth-Hitchcock Medical Center has no relevant financial relationships to disclose.)
Robert J Singer MD (Dr. Singer of Dartmouth-Hitchcock Medical Center/Geisel School of Medicine at Dartmouth has no relevant financial relationships to disclose.)
Bernard Maria MD, editor. (Dr. Maria of Icahn School of Medicine at Mount Sinai and Director of Pediatric Neurology and Developmental Medicine at Goryeb Children)
Originally released January 28, 2000; last updated June 8, 2017; expires June 8, 2020

This article includes discussion of chorea in childhood and St. Vitus dance. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Chorea is a manifestation of a number of neurologic disorders in childhood: Sydenham chorea being the prototype. Over the past decade, chorea has been increasingly seen in a number of conditions that affects children. In this chapter, we present the conditions that chorea is a symptom. In this revision we add new information that should be considered with presentation of chorea.

Key points

 

• The youngest case of rheumatic chorea has been reported in a 3-year-old child.

 

• There is no single diagnostic test for Sydenham chorea.

 

• Oxidative stress has been reported to be associated with the pathogenesis of Huntington disease.

 

• Caution should be taken when administering intrathecal methotrexate as acute, reversible chorea has been implicated.

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 (Sanger et al 2010).

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.

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