Three patients are described with pathologically verified Creutzfeldt-Jakob disease (CJD) who presented with localizing clinical signs accompanied by focal electroencephalographic abnormalities including periodic lateralized epileptiform discharges (PLEDS). With further progression of the disease, diffuse background slowing and continuous bisynchronous periodic discharges appeared in all three cases. There was good correlation between the initial focal clinical manifestations and the EEG findings.