PRNP and cerebral amyloid angiopathy: Besides GSS, PRNP truncating mutations have been linked to cerebral amyloid angiopathy (Ghetti et al., 2018) and, more recently, even found in patients with chronic diarrhea, progressive autonomic failure, and peripheral polyneuropathy in association with widespread abnormal PrP deposition in both peripheral organs and CNS (Matsuzono et al., 2013; Mead et al., 2013; Capellari et al., 2018).