Creutzfeldt-Jakob disease (CJD)

Creutzfeldt-Jakob disease (CJD) is a spongiform encephalopathy that results in a rapidly progressive dementia and other non-specific neurological features and death usually within a year or less from onset. The vast majority are sporadic, but familial and acquired forms are also occasionally encountered.

On imaging, it classically manifests as T2/FLAIR hyperintensities within the basal ganglia, thalamus, and cortex. These lesions show diffusion restriction on DWI/ADC sequences.

Reference:
https://radiopaedia.org/articles/creutzfeldt-jakob-disease?lang=us