Two cases of Creutzfeldt-Jakob Disease (CJD) are reported. In both cases, pathologically confirmed, magnetic resonance imaging (MRI) showed bilateral altered signal areas in the basal ganglia, early in the course of the disease. The CT scan, although performed later than MRI, confirmed these findings. Only in one patient did electroencephalography (EEG) show typical triphasic sharp-waves, but this support to clinical diagnosis was obtained 1 month after the appearance of MRI alterations.