Creutzfeldt-Jakob disease (CJD) generally presents as a cognitive disease characterized by severe neuronal loss, spongiform degeneration (spongiosis) and amorphous PrP aggregates, and primarily targets the cortex (Fig. 1) (Gambetti et al., 2003; Gambetti et al., 1995). This evidence concerns the gene PRNP and Creutzfeldt Jacob disease.