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  • Characterized by an acute onset of choreiform movements, variable degrees of psychological disturbance, rheumatic endocarditis, and arthritis

  • Although the disorder follows infections with group A β-hemolytic streptococci, the interval between infection and chorea may be greatly prolonged

  • Disease is self-limited; may last from a few weeks up to 18 months

  • Relapse may occur with nonspecific stress or illness—or with breakthrough streptococcal infections (if penicillin prophylaxis is not done)

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Clinical Findings

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  • Rapid involuntary discoordinated movements of the limbs and face are hallmarks

  • Other symptoms and signs include

    • Emotional lability

    • Waxing and waning ("milkmaid's") grip

    • Darting tongue

    • Spooning" of the extended hands and their tendency to pronate

    • Knee jerks slow to return to their prestimulus position ("hung up" knee jerk)

  • Hemichorea occurs in 20% of patients

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Diagnosis

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  • Anemia, leukocytosis, and an increased erythrocyte sedimentation rate and C-reactive protein may be present

  • Antistreptolysin O or anti-DNase titer (or both) are usually elevated

  • Throat culture is sometimes positive for group A β-hemolytic streptococci

  • ECG and echocardiography are often essential to detect cardiac involvement

  • If antinuclear antibodies (ANA) are present, chorea may be secondary to lupus

  • Similarly, antiphospholipid antibody may be elevated in autoimmune-related chorea

  • Testing to exclude other causes may include

    • Thyroid screening tests

    • Serum calcium for hypocalcemia

    • Immunologic and virologic tests for (rare) HIV, parvovirus B19, and Epstein-Barr virus infection

  • Brain MRI should be obtained in all children to rule out basal ganglia structural abnormalities, inflammation, and tumor

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Treatment

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  • No specific treatment

  • Prednisone (high-dose intravenously or orally 0.5–2 mg/kg/d in divided doses); in severe cases, IVIg has been successful

  • Anticonvulsant sodium valproate (50–60 mg/kg/d in divided doses) and levetiracetam (20–80 mg/kg/d divided into twice a day dosing) is effective in reducing chorea symptoms

  • Dopaminergic blockers such as haloperidol (0.5 mg/d to 3–6 mg/d) and pimozide (2–10 mg/d) are rarely used because of other effective medications and possible parkinsonian side effects such as rigidity and masked facies, and tardive dyskinesia

  • Emotional lability and depression sometimes warrant administration of antidepressants

  • All patients should be given antistreptococcal rheumatic fever prophylaxis with either monthly benzylpenicillin injections or oral penicillin VK 250 mg twice a day

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