Accumulation of prion protein in the vagus nerve in creutzfeldt–jakob disease

Abstract

Disease-associated proteins are thought to propagate along neuronal processes in neurodegenerative diseases. To detect disease-associated prion protein (PrP Sc ) in the vagus nerve in different forms and molecular subtypes of Creutzfeldt–Jakob disease (CJD), we applied 3 different anti-PrP antibodies. We screened the vagus nerve in 162 sporadic and 30 genetic CJD cases. Four of 31 VV-2 type sporadic CJD and 7 of 30 genetic CJD cases showed vagal PrP Sc immunodeposits with distinct morphology. Thus, PrP Sc in CJD affects the vagus nerve analogously to α-synuclein in Parkinson disease. The morphologically diverse deposition of PrP Sc in genetic and sporadic CJD argues against uniform mechanisms of propagation of PrP Sc . Ann Neurol 2019;85:782–787.