, Volume 19, Issue 4, pp 347-352

Progressive supranuclear palsy

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Two female cases of progressive supranuclear palsy (“heterogeneous system degeneration”) with histological findings are reported. Clinically, vertical gaze palsy, dystonia, akinesia, pseudobulbar palsy and mental impairment were prominent. Pathologically, widespread appearance of neurofibrillary tangles of the subcortical type in the brain stem and basal ganglia was associated with neuronal loss and gliosis in the substantia nigra, subthalamic nuclei and pallidum. There was less severe affection of the dentate nuclei and minimal cortical involvement. The distribution of changes in “subcortical argyrophilic dystrophy” is critically compared with and separated from brain stem affection in (pre)senile Alzheimer's disease.