The co-occurrence of prion disease and MSA, as reported earlier in a single individual (Shibao et al., 2008) and here in different members of a Sicilian family, might suggest the possibility of molecular interaction between PrP and α-synuclein in the process of protein accumulation and neurodegeneration, warranting investigation of the PRNP codon 129 variant in MSA. Here, SNCA is linked to multiple system atrophy.