The evaluation of the histopathological phenotype revealed some characteristics matching those described by Dermaut et al. (2000), including: (i) a generalized brain atrophy; (ii) mild spongiosis with preferential distribution in the cortical layer II; (iii) absence of SD in the hippocampus (CA1–CA4); (iv) loss of Purkinje cells; and (v) presence of elongated PrP deposits in the cerebellar molecular layer. This evidence concerns the gene PRNP and Brain atrophy.