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Sydenham chorea

Donald L Gilbert, MD, MS
Section Editors
Marc C Patterson, MD, FRACP
Sheldon L Kaplan, MD
Thomas JA Lehman, MD
Deputy Editor
Carrie Armsby, MD, MPH


Sydenham chorea (SC), or rheumatic chorea, is one of the major clinical manifestations of acute rheumatic fever (ARF) and is the most common form of acquired chorea in childhood. It is a movement disorder characterized by chorea (involuntary brief, random and irregular movements of the limbs and face), emotional lability, and hypotonia.

This topic reviews the clinical manifestations, diagnosis, and treatment of SC. Other clinical manifestations of ARF and the approach to diagnosis and treatment of rheumatic fever are presented separately. (See "Acute rheumatic fever: Clinical manifestations and diagnosis" and "Acute rheumatic fever: Treatment and prevention".)


Although SC is clearly related to group A streptococcal infection [1], its pathogenesis is not completely understood. Molecular mimicry, in which antibodies directed against part of the group A streptococcus bacterium crossreact with host antigens in susceptible subjects, is thought to play an important role.

In acute rheumatic fever (ARF), antibodies are mounted against N-acetyl-beta-D-glucosamine (NABG or GlcNAc), the immunodominant carbohydrate antigen of group A streptococci. These antibodies likely play a role in valvular injury in rheumatic carditis, and in other manifestations of ARF (see "Acute rheumatic fever: Epidemiology and pathogenesis", section on 'Molecular mimicry').

Different subsets of NABG antibodies appear to correlate with distinct clinical manifestations of ARF. In SC, the antibodies bind to lysoganglioside on the neuronal cell surface [2,3], where they are capable of triggering a signaling cascade [4]. These antibodies also recognize the intracellular protein tubulin [5]. Tubulin-specific antibodies are not found in patients with ARF without SC, or in patients who have recovered from SC. The genes encoding these antibodies are similar to the genes encoding antibodies implicated in the pathogenesis of motor neuropathies [6]. Thus, tubulin appears to be an important neuronal target in the pathogenesis of SC.

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Literature review current through: Nov 2017. | This topic last updated: Sep 02, 2016.
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