The presence of florid plaques is a well-documented signature of vCJD (BSE strain) [31], while kuru-type plaques are the hallmark of the CJD V2 strain, although only in subjects carrying MV or MM at PRNP codon 129, since they are virtually lacking in those carrying VV despite the widespread focal PrP plaque-like deposits [24, 28]. Here, PRNP is linked to Creutzfeldt Jacob disease.