Creutzfeldt-Jakob disease (CJD) is a rare, but fatal and transmissible
brain disease. The clinical diagnosis is based upon progressing demen
tia, myoclonic jerks and characteristic EEG changes, but it is difficu
lt to diagnose and not only in the early phase of the disease. Cerebra
l biopsy is reserved for individual selected cases and contested becau
se of the darger of contamination from instruments and potential trans
mission. We report three patients with histologically confirmed MRI is
a valuable tool for the diagnosis of this disease.