SYDENHAM'S CHOREA
 
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SYDENHAM'S CHOREA

 

DEFINITION:

A movement disorder characterized by chorea,hypotonia, and emotional labililty.

EPIDEMIOLOGY:

  • incidence: most common acquired chorea of childhood
  • age of onset:
    • 5 to 15 years of age
  • risk factors:
    • Group A beta-hemolytic Streptococcal infection (a component of Rheumatic Fever)
    • F > M

CLINICAL FEATURES:

1. Choreic Manifestations

  • begins abruptly or insidiously about 4 months after infection
  • chorea usually generalized but can be unilateral
  • affects face, trunk, and distal extremities
  • typical signs:
    • milkmaid's grasp: relaxing & tightening hand shake
    • choreic hand: spooning of flexed hand & extended fingers
    • darting tongue: unable to maintain protruded tongue
    • pronator sign: arms/palms outward when held above head
  • usually worsens over several weeks then resolves spontaneously over months to 1-2 years
  • may be recurrent within 2 years in 25% of patients
  • associated with:
    • hypotonia
    • emotional lability: uncontrollable crying & mood swings
    • dysarthria
    • school difficulties

2. Other Manifestations

  • of Rheumatic Fever
    • carditis, migratory polyarthritis, erythema marginatum, subcutaneous nodules, arthralgia

INVESTIGATIONS:

1. Serum

  • ASOT, blood cultures and ESR usually normal

MANAGEMENT:

1. Chorea

  • pimozide
  • benzodiazepines (diazepam)
  • phenothiazines or haloperidol

2. Supportive

  • treat Rheumatic Fever and complications
    • Penicillin, valvular heart disease, etc

 

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