Huntington's disease
Hun·ting·ton's disease
H0329550 (hŭn′tĭng-tənz)Huntington's disease
(ˈhʌntɪŋtən)| Noun | 1. | Huntington's disease - hereditary disease; develops in adulthood and ends in dementia |
| 单词 | huntington's disease | |||
| 释义 | Huntington's diseaseHun·ting·ton's diseaseH0329550 (hŭn′tĭng-tənz)Huntington's disease(ˈhʌntɪŋtən)
Huntington's diseaseHuntington's disease,hereditary, acute disturbance of the central nervous system usually beginning in middle age and characterized by involuntary muscular movements and progressive intellectual deterioration; formerly called Huntington's chorea. The disease is sometimes confused with choreachoreaor St. Vitus's dance, acute disturbance of the central nervous system characterized by involuntary muscular movements of the face and extremities. The disease, known also as Sydenham's chorea (not to be confused with Huntington's disease, a hereditary disease of ..... Click the link for more information. or St. Vitus's dance, which is not hereditary. A faulty gene produces a defective protein attacks neurons in the basal ganglia, clusters of nerve tissue deep within the brain that govern coordination. The onset is insidious and inexorably progressive; no treatment is known. Psychiatric disturbances range from personality changes involving apathy and irritability to bipolar or schizophreniform illness. Motor manifestations include flicking movements of the extremities, a lilting gait, and motor impersistence (inability to sustain a motor act such as tongue protrusion). In 1993 the gene responsible for the disease was located; within that gene a small segment of code is, for some reason, copied over and over. Genetic counseling is extremely important, since 50% of the offspring of an affected parent inherit the gene, which inevitably leads to the disease. Huntington's diseaseHuntington's disease(hŭn′tĭng-tənz)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)Huntington's diseaseHuntington's disease
Synonyms for Huntington's disease
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