Abstract
MRI including diffusion-weighted imaging (DWI) and fluid-attenuated inversion recovery (FLAIR) is useful for the diagnosis of prion disease, particularly Creutzfeldt-Jakob disease (CJD). Hyperintensity lesions are predominant on DWI, and are often seen in the cerebral cortex (“cortical ribboning”) or both in the cerebral cortex and striatum (anterior dominant). However, clinical and MRI findings of CJD can be mimicked by those of many other dementing conditions, including autoimmune encephalitis. Non-prion diagnosis should be considered when hyperintensity is predominant on FLAIR, apparent diffusion coefficient (ADC) is increased early, the lesion is symmetric, the limbic region is most affected, or ADC is decreased in the white matter.
