Short Original Communications

Acta Neuropathologica

, Volume 19, Issue 4, pp 347-352

First online:

Progressive supranuclear palsy

Subcortical argyrophilic dystrophy
  • K. JellingerAffiliated withNeurological Institute of University of Vienna

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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.

Key words

Progressive Supranuclear Palsy Heterogenous System Degeneration Subcortical Argyrophilic Dystrophy Neurofibrillary Tangles Brain Stem