| Huntington
Disease This is an autosomally-inherited, dominant disorder in which the patient begins to exhibit symptoms in the third to fourth decades. Patients with Huntington Disease (HD) initially have a tendency to fidget which over months or years develops into jerky, choreiform movements. HD usually progresses over a 10 to 25 year period. As the disease progresses it leads to dementia and usually death from incurrent infection. There is a high incidence of suicide among patients with HD. Pathologically, there is atrophy of certain forebrain structures including the entire cerebral cortex and even more notably of the caudate nucleus and putamen The head of the caudate is reduced to a narrow brownish band of tissue that is flattened or concave. In normal brain the ratio of small neurons to large neurons in the corpus striatum is approximately 160:1 in Huntingtons patients the ratio is reduced to 40:1 with a marked decrease in the number of astrocytes. The gene for this disease has been isolated to the short arm of chromosome 4. For more information concerning Huntington |
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 Huntington,George 1850-1916 |