What is the management of Sydenham's (Saint Vitus Dance) chorea?

Management of Sydenham's Chorea

All patients with Sydenham's chorea should receive immediate penicillin prophylaxis to prevent recurrent rheumatic fever and cardiac complications, combined with symptomatic treatment using valproic acid or haloperidol for moderate-to-severe chorea, with corticosteroids reserved for severe or refractory cases. 1, 2, 3

Immediate Antibiotic Treatment

Primary Eradication Therapy

• Administer a full therapeutic course of penicillin to eradicate residual Group A Streptococcus, even if throat culture is negative at diagnosis 2
• Oral penicillin V: 250 mg twice daily for children, 500 mg 2-3 times daily for adolescents/adults for 10 days 2
• For penicillin-allergic patients: use erythromycin or first-generation cephalosporins (if no immediate-type hypersensitivity) 2

Secondary Prophylaxis (Critical for All Patients)

• Intramuscular benzathine penicillin G: 1,200,000 units every 4 weeks is the preferred regimen 2
• In high-risk populations or patients with recurrences despite adherence, consider administration every 3 weeks 2, 4
• This prophylaxis reduces both cardiac complications and chorea recurrence rates 3

Duration of Prophylaxis

The duration depends on cardiac involvement 1, 2:

• With carditis and residual heart disease: 10 years after last episode OR until age 40 (whichever is longer), sometimes lifelong 2
• With carditis but no residual heart disease: 10 years OR until age 21 (whichever is longer) 2
• Without carditis: 5 years OR until age 21 (whichever is longer) 2
• Prophylaxis should continue even after valve surgery 1, 2

Symptomatic Treatment for Chorea

First-Line Symptomatic Agents

Valproic acid is the preferred first-line symptomatic treatment based on clinical experience and case reports 3, 4:

• Effective for controlling choreiform movements
• Generally well-tolerated in children
• Dosing should be individualized based on response and side effects 3

Alternative Symptomatic Agents

If valproic acid is ineffective or contraindicated 3, 5:

• Haloperidol: effective antipsychotic option, though carries risk of extrapyramidal side effects 3, 5
• Other antipsychotics and anticonvulsants have been used in case reports 3

Important caveat: Data on symptomatic therapy are limited to case reports and small series, with no large controlled trials comparing agents 3

Immunomodulatory Therapy

Corticosteroids

Corticosteroids should be reserved for severe or refractory cases 2, 3:

• Prednisone 1-2 mg/kg/day for 1-2 weeks for severe inflammation or cardiac involvement 2
• Efficacy is supported by a single placebo-controlled study and several case series 3
• In severe cases with significant cardiac involvement, consider intravenous methylprednisolone (1000 mg/day initially), followed by oral prednisone 2

IVIG and Plasmapheresis

• Evidence is extremely limited to small case reports and a single comparison study 3
• Should be reserved for severe, treatment-refractory cases only 3
• Not recommended as first-line therapy 1, 6

Cardiac Evaluation and Management

All patients require cardiovascular assessment 1:

• Evaluate for carditis, which occurs in the majority of Sydenham's chorea cases 1
• Perform echocardiography to detect valvular disease 1, 4
• If valvular disease is detected, classify as acute rheumatic fever and continue secondary prophylaxis accordingly 1
• Monitor carefully for several months for clinical evidence of carditis development 1

Special Considerations

Recurrence Risk Factors

Patients at higher risk for chorea recurrence include those with 7:

• Irregular antibiotic prophylaxis or poor compliance
• Failure to reach remission within 6 months
• Symptom persistence longer than one year

Pregnancy

• Chorea may recur during pregnancy in women with prior Sydenham's chorea 7
• Maintain prophylaxis throughout pregnancy with appropriate penicillin regimens 1

Long Latent Period

• Evidence of preceding streptococcal infection may be difficult to document due to the long latent period between infection and chorea onset 1
• Absence of supporting streptococcal evidence should not exclude diagnosis when chorea is present after a long latent period 1

Common Pitfalls to Avoid

• Do not discontinue prophylaxis prematurely: Even after symptoms resolve, continue prophylaxis for the full recommended duration based on cardiac status 1, 2
• Do not withhold penicillin if throat culture is negative: Residual GAS may still be present 2
• Do not confuse with PANDAS: While Sydenham's chorea is a proven manifestation of rheumatic fever, PANDAS remains an unproven hypothesis and should not be managed with the same immunomodulatory approaches 1, 6
• Do not overlook cardiac evaluation: Most patients with Sydenham's chorea have associated carditis requiring long-term management 1