Motor

Sydenham Chorea

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

  • See Also
  1. Group A Beta Hemolytic Streptococcus
  2. Rheumatic Fever
  3. PANDAS
  • 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
  • Epidemiology
  1. Incidence: <5% of Rheumatic Fever cases
  2. Ages: 5 to 15 years
  3. Girls affected more often than boys
  • 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
  • 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)
  • Labs
  • Cerebrospinal fluid
  1. Intracerebral pressure (opening pressure) increased
  2. CSF Glucose increased
  3. CSF Leukocyte count increased
  4. Late manifestation results in negative markers
    1. ASO Titer negative
    2. Acute phase reactants negative
  • Prognosis
  1. Self limited
  2. Expect improvement within 3 to 6 weeks (complete resolution by 2-3 years)
  • Management
  1. Benzodiazepines have been used in the past
  • Reference
  1. Swedo (1993) Pediatrics 91(4): 706-13 [PubMed]