CJD is a rapidly progressive and fatal neurodegenerative disease caused by misfolding of the normal prion protein (PrP^C) into a pathological conformation (PrP^Sc). Classical presentations include rapidly progressive dementia with behavioral changes, cerebellar and extrapyramidal features, myoclonus, positive 14-3-3 on CSF, cortical ribboning on MRI, and periodic sharp wave complexes (PSWC) on EEG. However, EEG findings vary depending on the molecular classification and the stage of the disease.

Case: A 49 year-old gentlemen presented with left upper extremity jerking movements, numbness of the 4^th and 5^th digits, decreased coordination and difficulty walking. His symptoms later progressed to slowing of speech without cognitive impairment. Neuroimaging showed cortical ribboning in bilateral parietal lobes. EEG showed left hand rhythmic twitches time locked with central-parietal rhythmic discharges suggestive of focal motor seizures/status epilepticus.