Sydenham's Chorea
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Clinical Features
- Acute onset (w/in hours) asymmetric chorea.
- The chorea is of the entire body, including the face and tongue (called serpent tongue).
- It typically arises in childhood/adolescence (5 to 15 years old).
- Occurs in females more commonly than males.
- Additional manifestations:
- Motor impairment (eg, incoordination, gait abnormality, impairment of activities of daily living)
- Flaccid muscle weakness
- Psychological and behavioral issues (eg, anxiety, depression, obsessive compulsive behaviors (along with tics)) accompany the chorea.
Pathological Findings/Diagnosis
- It arises anywhere from weeks to months (7 to 180 days) following group A beta-hemolytic streptococcal infection (typically manifesting with pharyngitis).
- The pathology involves an autoimmune attack of the basal ganglia, most likely.
- High blood titers of streptococcal antibodies (ASO and antiDNAseB) and rheumatic heart disease (see JONES criteria below) can support the diagnosis.
Prognosis/Treatment
- The symptoms resolve within weeks to months (21 – 180 days) but can return in adulthood, especially during the 1st trimester of pregnancy (called: chorea gravidarum).
- Treatment typically involves: chorea suppressing medication (eg, haloperidol or tetrabenazine); antibiotic therapy to prevent rheumatic heart disease; short-term immune therapy.
Rheumatic Fever & Sydenham's Chorea
- Sydenham's chorea may occur independently or as part of acute rheumatic fever.
- JONES criteria:
- J (Joints): Polyarthritis
- O (looks like a heart): Cardiomyopathy (also, antistreptolysin O titre)
- N: Nodules
- E: Erythema marginatum
- S: Sydenham chorea
References
- See the Atypical Parkinson's Disease tutorial