Molecular Genetics of Huntington's Disease
This extract was created in the absence of an abstract.
Excerpt
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by premature localized nerve-cell death (Hayden 1981; Martin 1984). In most cases, signs of the disease are not apparent until middle age. The onset is insidious, involving minor adventitious movements, but it is followed by inexorable progression to severe chorea. The movement disorder, which is eventually totally debilitating, is paralleled by progressive dementia. In some cases, psychiatric symptoms such as impulsive or aggressive behavior or chronic depression accompany or even precede the onset of motor symptoms. The risk of suicide is also increased in this disease. There is no effective treatment for preventing or delaying the onset and progression of HD. Affected individuals, most of whom have already had children before their symptoms appear, become increasingly debilitated over a 15-20-year span until death ensues, usually as a result of heart disease due to the incessant choreic movements or from pneumonia...