- Robert P Cruse, DO
Robert P Cruse, DO
- Assistant Professor
- University of Missouri College of Medicine
- Section Editors
- Marc C Patterson, MD, FRACP
Marc C Patterson, MD, FRACP
- Section Editor — Pediatric Neurology
- Professor of Neurology, Pediatrics, and Medical Genetics
- Chair, Division of Child and Adolescent Neurology
- Mayo Clinic College of Medicine
- Sheldon L Kaplan, MD
Sheldon L Kaplan, MD
- Editor-in-Chief — Pediatrics
- Section Editor — Pediatric Infectious Diseases
- Professor and Vice Chairman for Clinical Affairs
- Baylor College of Medicine
- Thomas JA Lehman, MD
Thomas JA Lehman, MD
- Section Editor — Pediatric Rheumatology
- Professor of Clinical Pediatrics
- Cornell University Medical College
Sydenham chorea (SC), also known as St. Vitus dance, St. Johannis' chorea, chorea minor, 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.
The incidence of ARF and SC has declined dramatically in the Western world . However, chorea is still a common manifestation of rheumatic fever, particularly in developing countries. In the United States, chorea was reported in 18 to 36 percent of cases of rheumatic fever [2-5]. Chorea also may occur as the initial manifestation of other illnesses, including post-infectious syndromes or primary immune disorders, which include N-methyl-D-aspartate (NMDA) receptor encephalitis and systemic lupus erythematosus . (See 'Differential diagnosis' below.)
Other clinical manifestations of ARF and the approach to diagnosis of rheumatic fever are presented separately. (See "Acute rheumatic fever: Clinical manifestations and diagnosis".)
Although SC clearly is related to group A streptococcal infection , 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, 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 Sydenham chorea, the antibodies bind to lysoganglioside on the neuronal cell surface [8,9], where they are capable of triggering a signaling cascade . These antibodies also recognize the intracellular protein tubulin . Tubulin-specific antibodies are not found in patients with acute rheumatic fever 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 . Thus, tubulin appears to be an important neuronal target in the pathogenesis of Sydenham chorea.
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- CLINICAL MANIFESTATIONS
- Neurologic symptoms and signs
- Psychiatric symptoms
- Association with other manifestations of rheumatic fever
- Streptococcal antibodies
- Differential diagnosis
- Imaging and echocardiography
- Pharmacologic therapy of chorea
- Antibiotic therapy
- RECURRENT CHOREA
- SUMMARY AND RECOMMENDATIONS