Accumulation of prion protein in the peripheral nervous system in human prion diseases.

After the finding that anti-prion antibodies stain sensory and sympathetic ganglia in variant Creutzfeldt-Jakob disease (vCJD), it was suggested that this localization supported the oral route of entry. However, prion accumulation subsequently also appeared in the peripheral nervous system (PNS) in sporadic cases. This study aims at evaluating the extent of prion protein accumulation in the PNS in all clinicopathologic subgroups of the disorder, with the exception of the familial and sporadic forms of fatal insomnia.