Progressive supranuclear palsy with fronto-temporal atrophy and various tau-positive abnormal structures.
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Abstract
An autopsy case of a 67-year-old man with typical clinical features of progressive supranuclear palsy (PSP) characterized by impairment of vertical ocular pursuit movement, pseudobulbar palsy, nuchal stiffness, parkinsonism, and dementia is described. In addition to typical pathological changes of PSP, the present case showed fronto-temporal cortical atrophy, accompanied with various Gallyas/tau-positive neuronal and glial structures such as neurofibrillary tangles, pretangle neurons, glial coiled bodies, astrocytic plaques and argyrophilic threads in the cerebral cortex and subcortical nuclei, and many senile plaques throughout the whole cerebral cortex. The present report suggests that PSP and corticobasal degeneration share a common background in neuronal and glial pathologies, that pathological changes of PSP and Alzheimer's disease are mixed in the entorhinal cortex, amygdala. Meynert nucleus, and hypothalamus, and that dementia with frontal lobe-like syndrome in PSP is related to the frontal and temporal cortical pathologies, and is cortical dementia as well as subcortical dementia.