Sporadic Creutzfeldt-Jakob disease (CDJ) is a fatal, rapidly progressive neurodegenerative disease that was first described as a dementia syndrome associated with cortical, striatal, and spinal cord involvement.(1) Its pathogenesis is related to the alteration of a naturally existing prion protein (PrPc) to an abnormal folder protein termed scrapie prion protein (PrPSc), and its clinical presentation can vary.(2) Brain MRI findings, especially those derived from diffusion-weighted imaging, play a pivotal role in recognizing and distinguishing sCJD from alternative diagnoses. Here, PRNP is linked to neurodegenerative disease.