NR AFAK
AU Hainfellner,J.A.; Budka,H.
TI Disease associated prion protein may deposit in the peripheral nervous system in human transmissible spongiform encephalopathies
QU Acta Neuropathologica 1999 Nov; 98(5): 458-60
PT journal article
AB There is increasing evidence indicating involvement of the peripheral nervous system (PNS) in the pathogenesis of transmissible spongiform encephalopathies (TSEs). Immunocytochemically detectable deposits of TSE-specific abnormal prion protein (PrPsc) are considered as a surrogate marker for infectivity. We used anti-PrP immunocytochemistry to trace PrPsc deposition in spinal and enteric ganglia, and peripheral nerve in Creutzfeldt-Jakob disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS), and fatal familial insomnia. Discrete PrPsc deposits were detectable only in a few posterior root nerve fibers in an adaxonal location in one of nine CJD and the one GSS patients examined. Follicular dendritic cells of the gut and enteric nervous system were not labeled. Thus, PrPsc may spread to the PNS in different forms of human prion disease. In contrast to our observations in experimental scrapie (Groschup et al., Acta Neuropathol, this issue), the deposits were scant. Possible explanations for this discrepancy comprise strain difference, or centripetal (experimental scrapie) versus centrifugal (sporadic and genetic human prion diseases) spread of PrPsc, resulting in different patterns and amounts of PrPsc accumulation in the PNS.
MH Adult; Aged; Creutzfeldt-Jakob Syndrome/pathology; Encephalopathy, Bovine Spongiform/*pathology; Gerstmann-Sträussler-Scheinker Disease/pathology; Human; Immunohistochemistry; Middle Age; Multiple Sclerosis/pathology; Peripheral Nervous System/*pathology; PrPsc Proteins/*analysis
AD Institute of Neurology, University of Vienna, AKH, POB 48, A-1097 Wien, Austria.
SP englisch
PO Deutschland