-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