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65-year-old man with language impairment and gait instability. Brain autopsy confirms a diagnosis of progressive supranuclear palsy.
Grumose degeneration is a pathological change of the dentate nucleus of the cerebellum. It consists of an accumulation of granular eosinophilic material, composed of swollen degenerating Purkinje cell axons, around neurons of the dentate nucleus. Generally, it develops in the context of dentate nucleus degeneration but with relative preservation of the cerebellar Purkinje cells and is most closely associated with progressive supranuclear palsy, dentatorubropallidoluysian atrophy, and spinocerebellar ataxia type 3. Neurofilament immunohistochemistry (see Related Content) or silver stains can be used to highlight the swollen Purkinje cell axons surrounding dentate neurons.