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Sydenham’s Chorea

  • Roongroj Bhidayasiri
  • Daniel Tarsy
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Sydenham’s chorea (SC) is a delayed complication of certain Aβ-hemolytic streptococcal infections and serves as a major criterion for the diagnosis of acute rheumatic fever. SC is characterized by chorea, muscle weakness, and a number of neuropsychiatric symptoms. It is an antibody-mediated disorder in which patients produce antibodies that cross-react with caudate and subthalamic nucleus neurons. However, documented evidence of a preceding streptococcal infection is identified in only 20–30% of cases.

Age of presentation is usually 5–15 years with a female preponderance. Chorea is usually generalized and produces smaller amplitude and more distal involuntary movements than those which occur in Huntington’s disease. Chorea occurs at rest or with activity and remits during sleep.

Keywords

Muscle Weakness Rheumatic Fever Major Criterion Neuropsychiatric Symptom Streptococcal Infection 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Sydenham’s Chorea.mp4 (MP4 20,779KB)

The patient exhibits generalized, asymmetrical chorea which is more severe on the right side. It is more severe while extending her arms. She has difficulty maintaining continuous hand grip with her right hand. The movements are less evident while reaching for a pen. (Video contribution from Dr. Kongkiat Kulkantrakorn, Thammasat University Hospital, Thailand.)

References

  1. 1.
    Swedo SE. Sydenham’s chorea: a model for childhood autoimmune neuropsychiatric disorder. JAMA. 1994;272:1788–91.PubMedCrossRefGoogle Scholar
  2. 2.
    Church AJ, Cardoso F, Dale RC, et al. Anti-basal ganglia antibodies in acute and persistent Sydenham’s chorea. Neurology. 2002;59:227–31.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  • Roongroj Bhidayasiri
    • 1
    • 2
  • Daniel Tarsy
    • 3
  1. 1.Chulalongkorn Center of Excellence on Parkinson’s Disease and Related DisordersChulalongkorn University HospitalBangkokThailand
  2. 2.Department of NeurologyDavid Geffen School of Medicine at UCLALos AngelesUSA
  3. 3.Department of NeurologyHarvard Medical School Beth Israel Deaconess Medical CenterBostonUSA

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