EEG and brain mapping (on basal conditions and after i.v. administration of diazepam) and visual, somatosensory and auditory evoked potentials have been performed on a patient at terminal evolution of CJD. These combined neurophysiologic studies indicated that very important functional impairments of either thalamic-frontal circuits and frontal, parietal and occipital cortex were associated with absent or mild dysfunction of the visual, somatosensory and auditory subcortical pathways. Combined neurophysiologic studies are important to better understand the pathophysiologic mechanisms of neurodegenerative diseases.