[Some problems on neuropathology of Huntington disease]

The most striking neuronal loss in the brains of Huntington disease (HD) occurs in the striatum, predominant in the caudate nucleus. However, pathogenesis of this disease is supposed to be mainly based on the process of "simple atrophy" in the whole brain neurons and their axons. Therefore, although the autopsied brains of HD are atrophic and their weight are light, histopathology based on the classical method shows few abnormal findings in the cerebrum. Against this fact, "numeric atrophy" of the neurons occurs in the striatum of chorea acanthocytosis.