| Abstract: | The fundamental neuropathological findings of progressive supranuclear palsy (PSP) are presented, based on 14 autopsied cases of PSP. The blunt pathologies of PSP are degeneration in the substantia nigra, globus pallidus, subthalamic nuclei, dentate nucleus of the cerebellum and red nucleus, where there are neuronal loss, gliosis and neurofibrillary changes, to varying degree. In addition, the atrophy of the midbrain, especially the characteristic pattern of tegmental atrophy of the pons exhibiting a hand-bag profile, is an important finding. Hypertrophy of inferior olivary nucleus is frequently observed, suggesting the existence of lesions in the dentato-olivary system. Hypoxemic affects at the agonal stage should be noted because death of Purkinje cells of the cerebellum leads to degeneration of their axons and subsequently to the decrease of grumose degeneration in the dentate nucleus. Familial PSP have been reported by several authors but all case in the present study were sporadic. |
