Presentation
4 months history of progressive severe dementia.
Patient Data
Extensive, albeit subtle, cortical restricted diffusion involves large areas of the cerebral cortex, predominantly in the left cerebral hemisphere involving all lobes, while cortical restricted diffusion is also seen in the medial right occipital, right parietal lobe, and right inferior temporal lobe. There is unequivocal mild restricted diffusion involving the caudate head and lentiform nucleus bilaterally. The involved regions also demonstrate extremely subtle FLAIR hyperintensity. No further abnormality is identified, with no intracranial mass or abnormal region of contrast enhancement and no T2/FLAIR signal abnormality is identified. No hydrocephalus and no extra-axial collections. The intracranial arterial vasculature is unremarkable.
Case 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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