Gut, bladder and vascular dysautonomia, and length-dependent neuropathy appear to be commonly shared clinical features, reflecting abundant PrP amyloid deposition in the central nervous system, vascular system and in other affected peripheral tissues on autopsy.2 The neuropathy seen in this pedigree affects mostly autonomic pathways in contrast to the peripheral neuropathy reported in sporadic CJD cases,17 suggesting different pathogenetic mechanisms in PrP systemic amyloidosis. Here, PRNP is linked to primary systemic amyloidosis.