Huntington's disease(redirected from Huntingtons Chorea)
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A rare inherited disease of the central nervous system characterized by progressive dementia, abnormal posture, and involuntary movements. The typical age of onset is between 30 and 50 years. Also called Huntington's chorea.
Huntington's diseaseHuntington's chorea Neurology An AD degenerative disease of adult onset–ages 40-50 that leads inexorably to death Clinical Slowly progressive mood and personality changes, mental deterioration, loss of coordination, chorea, cognitive decline, chronic fatigue, apathy Treatment None. See 'Escapee. ', Trinucleotide repeat disease.
Huntington's diseaseA rare, dominant, genetic brain disorder caused by a defective gene on chromosome number 4 that has complete penetrance. The disease appears most often in middle-aged adults and leads to loss of nerve cells and a buildup of the neurotransmitter dopamine. This causes involuntary twitching or jerking movements of the face and body (chorea), alternating excitement and depression, and progressive DEMENTIA. The chorea can be controlled by drugs but there is no treatment for the central problem. When the disease starts in childhood the inheritance is four times as likely to be from the father than from the mother. The mechanism of the disease is unknown but it is thought that neural toxicity results from the accumulation of amino-terminal fragments containing an expanded polyglutamine region. (George Huntington, 1850–1916, American General Practitioner)
A rare hereditary condition that causes progressive chorea (jerky muscle movements) and mental deterioration that ends in dementia. Huntington's symptoms usually appear in patients in their 40s. There is no effective treatment.
Mentioned in: Movement Disorders