Creutzfeldt-Jakob disease

Discussion:

MRI Brain showing widespread cortical restricted diffusion in a young adult patient, the appearances are suggestive of Creutzfeldt-Jakob disease in this clinical context. 

Brain biopsy was performed.

Histology

MICROSCOPIC DESCRIPTION: Serial sections show fragments of brain parenchyma comprising cortex and white matter. There is established florid spongiform degeneration involving cortex, concentrated in laminae II, III, and IV. Prominent vacuolation is noted surrounding neurons as well as within neurophils separate from neuronal cell bodies. There is moderate reactive astrocytic gliosis. No florid plaques are identified. There is no inflammation. In particular, there is no evidence of vasculitis. No granulomas are seen. There is no evidence of a tumor. Immunohistochemistry shows diffuse staining for PrP (anti 12F10 antibody) in a granular/synaptosomal pattern. Staining is positive after both short and long incubations and appears stronger in the long incubation preparation. No plaques are seen. The features are of spongiform encephalopathy most consistent with Creutzfeld-Jakob Disease.

FINAL DIAGNOSIS: spongiform encephalopathy most consistent with Creutzfeld-Jakob Disease

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