huntington's disease meaning

Pronunciation:
Noun: Huntington's disease
  1. Hereditary disease; develops in adulthood and ends in dementia
    - Huntington's chorea

Type of: autosomal dominant disease, autosomal dominant disorder, chorea, monogenic disease, monogenic disorder

Encyclopedia: Huntington's disease


[Medicine]
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)also Huntington disease n : a progressive chorea that is inherited as an autosomal dominant trait, that usu. begins in middle age, that is characterized by choreiform movements and mental deterioration leading to dementia, and that is accompanied by atrophy of the caudate nucleus and the loss of certain brain cells with a decrease in the level of several neurotransmitters —called also Huntington's

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