- Donald L Gilbert, MD, MS
Donald L Gilbert, MD, MS
- Professor of Pediatrics and Neurology; Director, Movement Disorders and Tourette Syndrome Clinics
- Cincinnati Children's Hospital Medical Center
- 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), 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 , 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 . These antibodies also recognize the intracellular protein tubulin . 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 . Thus, tubulin appears to be an important neuronal target in the pathogenesis of SC.
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- CLINICAL MANIFESTATIONS
- Neurologic symptoms and signs
- Psychiatric symptoms
- Association with other manifestations of rheumatic fever
- DIAGNOSTIC EVALUATION
- Testing for GAS infection
- Cardiac testing
- Inflammatory markers
- Cerebrospinal fluid analysis
- Other tests
- CLINICAL DIAGNOSIS
- DIFFERENTIAL DIAGNOSIS
- Antibiotic therapy
- Treatment of chorea
- Immune modulation
- Recurrent chorea
- SUMMARY AND RECOMMENDATIONS