Brain, Grumose degeneration of the dentate nucleus [LMP27265]

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Diagnostic Modality: 
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Disease Category: 
Cellular Responses to Stress and Toxic Insults: Adaptation, Injury, and Death
Diagnosis: 
Grumose degeneration of the dentate nucleus
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Male
Age: 
65 years
Diagnosis Code: 
ICD-10: G23.1
Slide Contributor Information
Gao, A., Keith, J.

Keywords

PSP, tauopathy, neurodegenerative disease, dentate, cerebellum

Clinical History

65-year-old man with language impairment and gait instability. Brain autopsy confirms a diagnosis of progressive supranuclear palsy.

Clinical Discussion

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.

Related Content

Neurofilament immunohistochemistry