title: Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob disease.
abstract: We report here on the exuberant glial reaction in the optic nerves affected by prion diseases. Optic nerves from CJD- and GSS-, and scrapie-infected mice and hamsters showed severe pathology. These lesions were qualitatively indistinguishable from each other but were more intense in the Fujisaki model than in the hamsters inoculated with Echigo-1. Exuberant cellular reaction comprised of macrophages containing numerous mitochondria, abundant rough endoplasmic reticulum, and secondary lysosomes filled with digested myelin debris, electron-dense material and occasionally, entire myelin-bound vacuoles were readily observed in both models. Macrophages actively digesting myelin fragments and containing lyre-like bodies and paracrystalline inclusions were frequently noted. Some macrophages extended long filopodia to form labyrinth-like structures, and within a few macrophages, concentric arrays of cisterns and channels sequestrated part of the cytoplasm. An analogous network of narrow cisterns was seen to surround whole segments of the myelinated fibers.
authors.affiliation: Department of Molecular Pathology and Neuropathology, Medical University of Lódź, Poland. ppliber@csk.am.lodz.pl.
authors.forename: Paweł P
authors.initials: PP
authors.lastname: Liberski
authors.forename: Beata
authors.initials: B
authors.lastname: Sikorska
authors.forename: Jolanta
authors.initials: J
authors.lastname: Bratosiewicz-Wasik
authors.forename: Anna
authors.initials: A
authors.lastname: Waliś
authors.forename: Paul
authors.initials: P
authors.lastname: Brown
authors.forename: David
authors.initials: D
authors.lastname: Brown
mesh_terms: D000818:Animals; D007562:Creutzfeldt-Jakob Syndrome / Q000473:pathology*; D006224:Cricetinae; D006801:Humans; D008297:Male; D051379:Mice; D008854:Microscopy, Electron; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology* / Q000648:ultrastructure; D009900:Optic Nerve / Q000473:pathology* / Q000648:ultrastructure; D017096:Prion Diseases / Q000473:pathology; D012608:Scrapie / Q000473:pathology