Neurology Book

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Sydenham's ChoreaAka: Saint Vitus Dance

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  1. See Also
    1. Group A Beta Hemolytic Streptococcus
    2. Rheumatic Fever
    3. PANDAS
  2. Pathophysiology
    1. Childhood Movement Disorder
    2. Occurs within 6 months of Streptococcal infection
      1. Specifically Group A Beta Hemolytic Streptococcus
    3. Late manifestation of Rheumatic Fever
  3. Epidemiology
    1. Incidence: <5% of Rheumatic Fever cases
    2. Ages: 5 to 15 years
    3. Girls affected more often than boys
  4. Symptoms: Gradual onset of neurological symptoms
    1. Atypical behavior
      1. Irritability and crying
      2. Anxiety and restlessness
      3. Transient Psychosis
    2. Poor memory retention
    3. Occasional involuntary movements
      1. Irregular and aimless
      2. Affects extremities, face and trunk
  5. 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)
  6. 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
  7. Prognosis
    1. Self limited
    2. Expect complete recovery within 3 to 6 weeks
  8. Management
    1. Benzodiazepines have been used in the past
  9. Reference
    1. Swedo (1993) Pediatrics 91(4):706

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