Ubiquitin-reactive neurites in cerebral cortex of subjects with Huntington's chorea: a pathological correlate of dementia?

ABSTRACT

We studied the prevalence of ubiquitin-reactive dystrophic neurites in neocortex of cases with Huntington's disease (HD), with a history of dementia lasting from 5 to 8 years before death, and in four age-matched controls. The ubiquitin-reactive neurites, identified as round structures localized outside neuronal and glial cells, were quantified in six microscopic fields of cingulate, 2nd temporal and 2nd parietal cortical gyri. The number of ubiquitin-reactive neurites in HD was 12 to 16 times that of controls in the three cortical areas examined. The finding indicates that in HD neocortex there is a severe degeneration of neuronal processes and suggests that it may represent a pathological correlate of dementia.