NR ASZY
AU Lee,C.C.; Kuo,L.T.; Wang,C.H.; Scaravilli,F.; An,S.F.
TI Accumulation of prion protein in the peripheral nervous system in human prion diseases
QU Journal of Neuropathology and Experimental Neurology 2005 Aug; 64(8): 716-21
PT journal article
AB 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. Patients included 2 vCJD cases, 2 Gerstmann-Sträussler-Scheinker (GSS), 2 iatrogenic (iCJD), and 16 sporadic CJD (sCJD) cases. Gasserian (17) and spinal (9), celiac (2) and thoracic sympathetic (one) ganglia, spinal cord and medulla of one vCJD, 2 GSS, one iCJD, and 5 sCJD cases were examined. Immunostained sensory ganglia were seen in both vCJD, both iCJD, one GSS, and 10 sCJD cases; the celiac ganglion was positive in one of two sCJD cases, and the spinal dorsal horn and the medullary sensory nuclei were positive in one patient with vCJD, one with iCJD, and 3 with sCJD. Western blot demonstrated presence of PrP in the gasserian ganglion of one patient with sCJD. Accumulation of prion in ganglia (including autonomic) of the PNS, shared by all subgroups of spongiform encephalopathy, and in the dorsal horns and medullary sensory nuclei, shows that the sensory route is involved in the trafficking of this protein.
IN Die Autoren untersuchten die Verbreitung von PrPsc im peripheren Nervensystem von 2 nvCJK-Fällen, 2 Gerstmann-Sträussler-Scheinker-Patienten, 2 iatrogenen CJK-Fällen und 16 sporadischen CJK-Fällen. Sie konnten es immunhistopathologisch nachweisen in sensorischen Ganglien von beiden nvCJK-Fällen, beiden iCJK-Fällen, 1 Gerstmann-Sträussler-Scheinker-Patienten und 10 sporadischen CJK-Fällen. Das Bauchhöhlenganglion (Ganglion celiacum oder Ganglion coeliacum) war positiv in einem von 2 getesteten sCJK-Fällen. Die dorsalen Spinalganglien und in den sensorischen Kernen in der Medulla oblongata (medullary sensory nuclei) waren positiv in 1 vCJD-Fall, 1 iCJD-Fall und 3 sCJD-Fällen. Ein Western blot wies PrPsc auch im Ganglion trigeminale (Gasser'sches Ganglion als sensibler Teil des V. Hirnnervs) eines sCJK-Patienten nach.
MH Adult; Aged; Aged, 80 and over; Blotting, Western/methods; Comparative Study; Female; Ganglia/metabolism; Humans; Immunohistochemistry/methods; Male; Middle Aged; Peripheral Nervous System/*metabolism/pathology; PrPsc Proteins/metabolism; Prion Diseases/classification/*metabolism/pathology; Prions/*metabolism
AD Department of Pathology and Laboratory Medicine, Shin-Kong, Wu Ho-Su Memorial Hospital, Taipei Medical University, Taipei, Taiwan.
SP englisch
PO USA