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.
KeywordsMuscle Weakness Rheumatic Fever Major Criterion Neuropsychiatric Symptom Streptococcal Infection
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.)