Huntington Disease
Synonyms
Akinetic Rigid Variant of Huntington Disease
Akinetic-Rigid Variant of Huntington Disease
Chorea, Chronic Progressive Hereditary (Huntington)
Chorea, Huntington
Chorea, Huntington's
Chronic Progressive Hereditary Chorea (Huntington)
Huntington Chorea
Huntington Chronic Progressive Hereditary Chorea
Huntington Disease, Akinetic Rigid Variant
Huntington Disease, Akinetic-Rigid Variant
Huntington Disease, Juvenile
Huntington Disease, Juvenile Onset
Huntington Disease, Juvenile-Onset
Huntington Disease, Late Onset
Huntington Disease, Late-Onset
Huntington's Chorea
Huntington's Disease
Juvenile Huntington Disease
Juvenile Onset Huntington Disease
Juvenile-Onset Huntington Disease
Late Onset Huntington Disease
Late-Onset Huntington Disease
Progressive Chorea, Chronic Hereditary (Huntington)
Progressive Chorea, Hereditary, Chronic (Huntington)
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)