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Proliferation of mesaxons in the optic nerve of hamsters infected with the Echigo-1 strain of Creutzfeldt-Jakob disease

100%

Walis A., Liberski P. P.

Acta Neurobiologiae Experimentalis

1999

vol. 59

issue 3

177-180

The Echigo-1 strain of CJD was isolated by Mori and colleagues from a case of 33-years-old female with a panencephalopathic type of CJD. An incubation period following intracerebral inoculation of hamsters with 10 % cleared suspension of the Echigo -1-affected brain was approximately six months. We report here ultrastructural changes in the optic nerves. Vacuoles developed within myelinated axons: within axoplasm or within the myelin sheath and these were accompanied by exuberant reaction of macrophages and hypertrophied astrocytes. Axons underwent Wallerian degeneration and dystrophic neurites were also seen. Most important, we observed proliferation of inner mesaxons. Cross-sectional profiles of innumerable myelinated fibers contained membranous organelles which were continuous with the inner lamellae of the oligodendroglial cells. These unusual proliferations of inner mesaxons formed whorls and elaborated loops. In some axons, proliferation was so severe that loops of mesaxon filled the whole cross-section of the axon. Occasionally, we observed intrusion of the membranous tongue of the inner mesaxon into axoplasm.

Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob disease

61%

Liberski P. P., Sikorska B., Bratosiewicz-Wasik J., Walis A., Brown P., Brown D.

Acta Neurobiologiae Experimentalis

2003

vol. 63

issue 4

309-318

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.

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2 Acta Neurobiologiae Experimentalis

1 2003

1 1999

2 Liberski P. P.

2 Walis A.

1 Bratosiewicz-Wasik J.

1 Brown D.

1 Brown P.

1 Sikorska B.

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Proliferation of mesaxons in the optic nerve of hamsters infected with the Echigo-1 strain of Creutzfeldt-Jakob disease

Exuberant cellular reaction of the optic nerves in experimental Creutzfeldt-Jakob disease