Sydenham Chorea

Aka: Sydenham Chorea, Sydenham's Chorea, Rheumatic Chorea, Saint Vitus Dance

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II. Pathophysiology

  1. Childhood Movement Disorder
  2. Occurs within 8 months of Streptococcal infection
    1. Specifically Group A Beta Hemolytic Streptococcus
  3. Late manifestation of Rheumatic Fever

III. Epidemiology

  1. Incidence: <5% of Rheumatic Fever cases
  2. Ages: 5 to 15 years
  3. Girls affected more often than boys

IV. Symptoms: Gradual onset of neurological symptoms

  1. Atypical behavior
    1. Irritability and crying
    2. Anxiety and restlessness
    3. Transient Acute Psychosis
  2. Poor memory retention
  3. Motor weakness
  4. Abrupt onset of choreoform movements (purposeless, nonrhythmic, involuntary) only present while awake
    1. Irregular and aimless
    2. Affects extremities, face and trunk

V. Signs

  1. Choreiform movements
    1. Abrupt, purposeless, nonrhythmic involuntary movement
  2. Hyperextended joints
  3. Motor weakness
  4. Inaccurate voluntary motions
  5. Clumsiness
  6. Hypotonia
  7. Diminished Deep Tendon Reflexes
  8. Speech Impairment (Dysarthria)
  9. Asymmetric (one side usually more affected than other)

VI. Labs

  1. Strep Test or Throat Culture
    1. Negative in >50% of cases
  2. Cerebrospinal fluid
    1. Intracerebral pressure (opening pressure) increased
    2. CSF Glucose increased
    3. CSF Leukocyte count increased
  3. Late manifestation results in negative markers
    1. ASO Titer negative
    2. Acute phase reactants negative

VII. Management

  1. Evaluate for other manifestations of Rheumatic Heart Disease
  2. Initiate Penicillin
    1. Initial Treatment (often given regardless of Throat Culture result)
      1. See Streptococcal Pharyngitis
    2. Secondary Prophylaxis
      1. Medications
        1. Penicillin G 1.2 million units IM every 21 days
      2. Duration
        1. No Carditis: Administer for at least 5 years AND until age >18 years
        2. Mild Carditis: Administer for at least 10 years AND until age >21 years
        3. Moderate to severe carditis: Lifelong Penicillin prophylaxis
  3. Mild to no functional Impairment
    1. No Chorea treatment needed
  4. Moderate to severe Impairment
    1. Step 1a: Anticonvulsant (choose one)
      1. Valproate 20 mg/kg/day OR
      2. Carbamazepine 15 mg/kg/day
    2. Step 1b: Adjunctive Corticosteroids (Indicated in severe or refractory cases, Chorea paralytica)
      1. Prednisone 2 mg/kg/day for 2-4 weeks, followed by taper OR
      2. Methylprednisolone 25 mg/kg/day IV for 5 days, followed by taper
    3. Step 2: Reevaluate response to anticonvulsants
      1. Improved: Wean as tolerated after 4 weeks symptom free
      2. Refractory: Go to Step 3
    4. Step 3: Refractory Management
      1. Add Antipsychotic (choose 1)
        1. Risperidone 1-2 mg orally twice daily (teen 0.5 mg twice daily in age 13-17 years) OR
        2. Olanzapine 5-10 mg/day orally (teen 2.5 to 5 mg/day in age 13 to 17 years) OR
        3. Haloperidol 5-10 mg orally every 6-8 hours (child 0.05 to 0.15 mg/kg up to 2-5 mg for teen) OR
        4. Pimozide 1-2 mg at bedtime (child 0.05 mg/kg up to 1 to 2 mg)
      2. Persistent refractory symptoms despite Antipsychotic and anticonvulsant
        1. Consider Corticosteroids (see Step 1b above)
        2. Consider experimental treatments
          1. IVIG 2 g/kg IV for 5 days
          2. Plasmapheresis for 5 days

VIII. Prognosis

  1. Self limited
  2. Expect improvement within 3 to 6 weeks (complete resolution by 2-3 years)

IX. Reference

  1. Dean (2017) Tremor Other Hyperkinet Mov 7:456 +PMID: 28589057 [PubMed]
  2. Swedo (1993) Pediatrics 91(4): 706-13 [PubMed]

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