Huntington Disease

An autosomal dominant inherited disease, when the patient begins to exhibit symptoms at 20s and 30s.Initially the patients 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 Huntington Disease (HD).

Pathology : 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.