Ultrastructural alterations in the optic nerve in transmissible spongiform encephalopathies or prion diseases--a review.

The involvement of the visual system is well recognised in TSEs. The present review summarises the ultrastructural changes in the optic nerves in experimental infections of laboratory rodents with the agents of two human TSEs (CJD and GSS) and with two isolates of the scrapie agent. Vacuoles of myelinated fibres were found within myelin sheaths and themselves contained secondary vacuoles (vacuoles within other vacuoles) and curled membrane fragments not unlike the vacuoles in cerebral grey matter (see also: Spongiform change--an electron microscopic view; this issue).

Fate of myelinated fibres in the optic nerves in experimental Creutzfeldt-Jakob disease in rodents: an ultrastructural study.

We report the ultrastructural appearances of myelinated fibres of the optic nerves from mice infected with the Fujisaki strain of Gerstmann-Sträussler-Scheinker (GSS) disease and from Echigo-1 strain of Creutzfeldt-Jakob disease (CJD). Optic nerves from CJD- and GSS-infected rodents showed severe pathological changes. Theses changes were qualitatively indistinguishable from each other but were more robust in the Fujisaki GSS model than in the hamsters inoculated with Echigo-1.

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.

Ultrastructural changes in the optic nerves of rodents with experimental Creutzfeldt-Jakob Disease (CJD), Gerstmann-Sträussler-Scheinker disease (GSS) or scrapie.

This report describes the ultrastructural changes in the optic nerves of (1) hamsters infected with the Echigo-1 strain of Creutzfeldt-Jakob disease (CJD), (2) hamsters infected with the 263K or 22C-H strain of scrapie, and (3) mice infected with the Fujisaki strain of Gerstmann-Sträussler-Scheinker disease (GSS). Vacuolation of myelinated fibres was present in the myelin sheaths, with splitting of myelin lamellae. These vacuoles contained typical secondary vacuoles and curled membrane fragments.

A special report I. Prion protein (PrP)--amyloid plaques in the transmissible spongiform encephalopathies, or prion diseases revisited.

We present a retrospective analysis of PrP-amyloid plaques encountered in CJD and GSS. In human TSEs (kuru, CJD and GSS) several PrP-immunopositive plaques and plaque-like deposits were detected. In kuru, plaques were typical "kuru" plaques--stellate structures deposited mostly in the granular- and Purkinje-cell layer of the cerebellum.