-IBIS-1.5.0-
tx
nervous system
chorea
diagnoses

definition and etiology

definition:
Irregular, spasmodic, involuntary movements of the limbs and facial muscles, usually referring to:
• Huntington's chorea: "Chronic progressive, Hereditary, or Degenerative chorea"
» inherited as an autosomal dominant trait
» characterized by gross atrophy of corpus striatum with neuronal degeneration in the caudate and other deep nuclei and frontal cerebral cortex
• Sydenham's chorea (chorea minor, rheumatic chorea, St. Vitus' dance)
» CNS disease, often of insidious onset but of finite duration
» characterized by involuntary, purposeless, nonrepetitive movements, and subsiding without neurologic residua

Sydenham's chorea is generally regarded as an inflammatory complication of Group A streptococcal infections. Onset of chorea is often up to 6 months after the infection, therefore may seem unrelated. More common in girls than in boys; in childhood; in summer and early fall after the rheumatic season.

signs and symptoms

signs and symptoms:

Huntington's:
• obstinacy
• moodiness
• lack of interest
These may antedate choreiform movements (irregular, spasmodic, involuntary movements of the limbs and facial muscles); these usually begin in the upper extremities neck, and face, progressing from mild fidgeting to facial grimaces, hesitant speech, torticollis, and irregular trunk movements
• gait is wide based and prancing
• euphoria is common
• later stages include: dysphagia, dementia, and inability to walk

Sydenham's:
• development of rapid, purposeless, nonrepetitive movements which involve all muscles except the eyes
• impaired coordination
• facial grimacing
• slight clumsiness in some cases
• arm flailing in severe cases
• neurologic exam shows no defect in strength or sensation except occasional pendulous knee jerk

course and prognosis

Huntington's: insidious onset; symptoms begin between 30 and 50 years; an important note is that signs and symptoms do not occur until well into childbearing years
Sydenham's: 3-8 month course; insidious onset and gradual cessation make this illness difficult to diagnose and treat

differential diagnosis

• Huntington's chorea
• Sydenham's chorea
• Chorea gravidarum (develops first trimester of pregnancy) may recur with subsequent pregnancies; is not necessarily progressive
• habit spasms seen in hyperkinetic children
• paresis


footnotes