Sydenham's chorea is a movement disorder that is the most common form of autoimmune chorea. It typically develops in children between ages 5-15 after a Group A beta-hemolytic streptococcal infection and is part of rheumatic fever. Symptoms include generalized chorea and other neurological symptoms like motor impersistence. Diagnosis involves looking for elevated antistreptococcal titers and imaging may show basal ganglia enlargement. Treatment considers severity, requirements, availability, cost, evidence and side effects. It is thought to be caused by molecular mimicry between streptococcal and human proteins that leads to an autoimmune response.

1. A D E W I J A Y A , M D
N O V E M B E R 2 0 1 9
Sydenham’s Chorea

2. Bridenbaugh S.A., Kressig R.W. (2018) Movement Disorders. In: Roller-Wirnsberger R., Singler K., Polidori M. (eds) Learning Geriatric Medicine. Practical Issues in Geriatrics.
Springer, Cham

3. Outline:
 Introduction
 Clinical presentation
 Diagnosis
 Pathophysiology
 Treatment
 Summary

4. Introduction
Most common form of autoimmune chorea
Onset: age 5-15
Female > Male
Usually develops 4–8 weeks after a group A beta-
hemolytic streptococcal (GABHS) pharyngitis
Part of Rheumatic Fever
Resolve in 1-6 months
Punukollu M, Mushet N, Linney M, Hennessy C, Morton M. Neuropsychiatric manifestations of Sydenham’s chorea: a systematic review. Dev Med Child Neurol. 2016;58:16–28.

5. Clinical Presentation
Generalized chorea
Hemichorea (25 %)
Other neurological symptoms:
motor impersistence, hypometric saccades, reduced muscle tone,
tics, clumsiness, dysarthria, and weakness
Profound hypotonia  chorea paralytica or chorea mollis
Neuropsychiatric symptoms, including obsessive compulsive
behaviors, personality changes, emotional lability, distractibility,
irritability, anxiety, age-regressed behaviors, and anorexia, are
common and frequently predate the appearance of chorea
Punukollu M, Mushet N, Linney M, Hennessy C, Morton M. Neuropsychiatric manifestations of Sydenham’s chorea: a systematic review. Dev Med Child Neurol. 2016;58:16–28.

6. Diagnosis
Elevated antistreptococcal titers are present in about 15–30%
imaging is usually normal, except for possible acute phase
enlargement of the basal ganglia and increased T2 intensity
in rare cases
Giedd JN, Rapoport JL, Kruesi MJ, et al. Sydenham’s chorea: magnetic resonance imaging of the basal ganglia. Neurology. 1995;45:2199–2202.

7. Pathophysiology
Cunningham, M. W., & Cox, C. J. (2016). Autoimmunity against dopamine receptors in neuropsychiatric and movement disorders: a review of Sydenham chorea and beyond. Acta
Physiologica, 216(1), 90-100.

8. Treatment
Dean, S. L., & Singer, H. S. (2017). Treatment of Sydenham’s chorea: a review of the current evidence. Tremor and Other Hyperkinetic Movements, 7.

9. Dean, S. L., & Singer, H. S. (2017). Treatment of Sydenham’s chorea: a review of the current evidence.
Tremor and Other Hyperkinetic Movements, 7.

10. Treatment
Consideration:
- Severity of the problem
- The requirement for a therapeutic agent
- Availability
- Cost
- Supporting evidence
- Side effects

11. Summary
 Is a movement disorder
 Most common form of autoimmune chorea
 Part of rheumatic fever
 Group A beta-hemolytic streptococcal (GABHS)
 Molecular mimicry
 Role for immunomodulatory therapy

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