Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob disease.
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.
Liberski
Paweł P
PP
Department of Molecular Pathology and Neuropathology, Medical University of Lódź, Poland. ppliber@csk.am.lodz.pl.
Sikorska
Beata
B
Bratosiewicz-Wasik
Jolanta
J
Waliś
Anna
A
Brown
Paul
P
Brown
David
D
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