What is the recommended treatment for Sydenham chorea in a school‑age female following a recent group A streptococcal pharyngitis?

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Treatment of Sydenham Chorea Following Group A Streptococcal Pharyngitis

Immediately initiate a full 10-day course of penicillin to eradicate residual Group A Streptococcus, followed by long-term secondary prophylaxis with benzathine penicillin G injections, combined with valproic acid for symptomatic control of choreiform movements. 1, 2

Immediate Antibiotic Eradication Therapy

Administer a complete therapeutic course of penicillin even if the throat culture is negative at the time of chorea diagnosis, as residual streptococcal organisms may persist despite negative cultures. 1, 2

Preferred Regimens:

  • Oral Penicillin V: 250 mg twice daily for 10 days in children, or 500 mg 2-3 times daily for adolescents/adults for 10 days 1
  • Intramuscular Benzathine Penicillin G: Single dose of 600,000 units for patients <27 kg or 1,200,000 units for patients ≥27 kg 2, 3

For Penicillin Allergy:

  • First-generation cephalosporins (if no immediate-type hypersensitivity) 1
  • Erythromycin as alternative 1
  • Azithromycin 500 mg once daily for 5 days or clarithromycin 250 mg twice daily for 10 days 2

Critical Pitfall: Do not withhold penicillin based on a negative throat culture—the latency period between streptococcal infection and chorea onset is typically 14-21 days, and at least one-third of cases result from asymptomatic infections. 1, 2

Long-Term Secondary Prophylaxis (Most Critical Component)

Initiate continuous antimicrobial prophylaxis immediately upon diagnosis to prevent recurrences, as at least 20% of Sydenham chorea patients experience recurrent attacks. 2

Gold Standard Regimen:

Intramuscular benzathine penicillin G 1,200,000 units every 4 weeks (600,000 units for patients <27 kg) is approximately 10 times more effective than oral antibiotics. 1, 2

Duration Based on Cardiac Involvement:

Perform echocardiography on all patients to detect valvular disease and carditis, as this determines prophylaxis duration. 1

  • With carditis AND residual heart disease: 10 years after last episode OR until age 40 (whichever is longer) 1
  • With carditis but NO residual heart disease: 10 years OR until age 21 (whichever is longer) 1
  • Without carditis: 5 years OR until age 21 (whichever is longer) 1

Critical Pitfall: Prophylaxis must not be discontinued prematurely even after symptoms resolve—continue for the full recommended duration based on cardiac status. 1 Risk factors for recurrence include irregular prophylaxis, failure to reach remission within 6 months, and symptom persistence longer than one year. 1, 4

Symptomatic Treatment for Choreiform Movements

Valproic acid is the preferred first-line agent for controlling choreiform movements, with dosing individualized based on response and side effects. 1, 4

Alternative Symptomatic Agents:

  • Haloperidol is effective but carries risk of extrapyramidal side effects 1

Do not confuse Sydenham chorea with PANDAS—they should not be managed with the same immunomodulatory approaches. 1

Immunomodulatory Therapy (Reserved for Severe Cases)

Corticosteroids should be reserved only for severe or refractory cases, with prednisone 1-2 mg/kg/day for 1-2 weeks for severe inflammation or cardiac involvement. 1

IVIG and plasmapheresis should be reserved for severe, treatment-refractory cases only. 1

Family and Contact Management

Obtain throat cultures from ALL household contacts and treat those with positive results regardless of symptoms, as this prevents transmission and potential acute rheumatic fever development in genetically susceptible family members. 2

Cardiac Evaluation (Essential and Often Overlooked)

All patients require cardiovascular assessment including echocardiography to detect valvular disease, as most patients with Sydenham chorea have associated carditis requiring long-term management. 1 If valvular disease is detected, classify as acute rheumatic fever and continue secondary prophylaxis accordingly. 1

Special Considerations for High-Risk Patients

Consider more frequent benzathine penicillin G injections (every 3 weeks instead of 4 weeks) in patients with recurrent chorea or irregular compliance. 4 Chorea may recur during pregnancy in women with prior Sydenham chorea, and prophylaxis should be maintained throughout pregnancy with appropriate penicillin regimens. 1

References

1
Guideline

Management of Sydenham's Chorea

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2025

2
Guideline

Sydenham Chorea Management and Prevention

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2026

3
Guideline

Treatment of Strep Pharyngitis in Children

Praxis Medical Insights: Practical Summaries of Clinical Guidelines, 2026

4
Research

Rare Recurrence of Sydenham Chorea in an Adult: A Case Report.

International medical case reports journal, 2023

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Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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