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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 6J3 PSP (Case 6) N9 Dentate NSE 7.jpg
Staining for NSE stains dentate neurons strongly but stains grumose terminals more modestly. (NSE IHE) ---- Not shown: Tau immunostain is positive in many of the deep cerebellar neurons and the cerebellar white matter contains numerous neuritic threads. ---- Neuro Final Diagnosis
Progressive supranuclear palsy (PSP), with grumose degeneration of the cerebellar dentate nucleus. ---- Neuro Diagnosis Comment:
The complete set of histopathologic hallmarks of progressive supranuclear palsy was present in this case. Neurodegeneration with abundant tau-positive neuronal inclusions, neuritic threads and tufted astrocytes were widespread in the brainstem and cerebellum. There was no evidence of synucleinopathy by immunohistochemistry. The finding of grumose degeneration (accumulation of dystrophic Purkinje axon terminals immediately adjacent to cerebellar dentate neurons) is an incompletely understood but highly specific finding in PSP.
