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)