A PATHOLOGICAL STUDY IN HUNTINGTON'S CHOREA AND DENTATORUBROPALLIDOLUYSIAN ATROPHY

Abstract

Changes of cerebral cortex (frontal and occipital) in six cases of Huntington's chorea (HC) and two cases of Dentatorubropallidoluysian atrophy (DRPLA) were studied histopathologically. A particular attention was paid to the changes in the number and size of nerve cells in the third, fifth and sixth layers. These changes were then correlated with the severity of mental symptoms in each case.Results were summerized as follows : 1) In HC, the cerebral cortex was markedly atrophic ; the atrophy was most remarkable in the fifth and sixth layers. In DRPLA, the cortex was also atrophic, but to a lesser extent as compared with HC;2) In HC, the number and average size of cortical neurons were markedly reduced. The reductions in DRPLA were less than in HC. However, changes in the neuron density of the cortex were slight and variable in both HC and DRPLA;3) Changes in the cell size distribution of cortical neurons were almost the same in HC and DRPLA. The population ratio for large neurons greater than 150 μm2 was reduced to less than one half, while that for small neurons less than 60 μm2 doubled ; and4) Apparent correlations were found between the severity of dementia and personality changes, and the reduction in the number and average size of neurons in the frontal cortex.