Summary
The ultrastructure of the optic nerve is described and compared in:
a)Schilder's Disease,
b)Devic's Disease,
c)Disseminated Sclerosis.
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a)
Schilder's Disease. Parts of the nerve were demyelinated, sometimes extensively so and from these regions the oligodendrocytes had disappeared and there was proliferation of astrocytes. Parallel bundles of fibres with a “railway line” formation, occurred in the cytoplasm of the astrocytes. Phagocytes infiltrated the damaged nerve bundles and the fibrous septa between them. Osmiophilic particles occurred in the astrocytes, capillary endothelial cells and in the phagocytes. The collagen fibres of some septa were widely separated presumably by fluid.
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b)
Devic's Disease. This condition occurred in a patient with active pulmonary tuberculosis. The nerve was extensively demyelinated and showed absence of oligodendrocytes, proliferation of astrocytes and infiltration by macrophages. Some astrocytes possessed Rosenthal fibres. Intranuclear inclusions occurred in the astrocytes and electron dense cytoplasmic inclusions in the capillary endothelial cells and the macrophages.
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c)
Disseminated Sclerosis. Parts of the nerve were partially and other parts completely demyelinated. Oligodendrocytes were absent from the completely demyelinated zones but were present in the partially demyelinated zones. In and around the demyelinated regions, there was proliferation of astrocytes and they frequently exhibited the “railway line” patterns in their cytoplasm. Phagocytes were frequent amongst the degenerating myelin and the proliferating astrocytes and also in the fibrous septa. Some macrophages presented intranuclear inclusions having a “corn on the cob” appearance.
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de Preux, J., Mair, W.G.P. Ultrastructure of the optic nerve in Schilder's disease, Devic's Disease and Disseminated Sclerosis. Acta Neuropathol 30, 225–242 (1974). https://doi.org/10.1007/BF00688923
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DOI: https://doi.org/10.1007/BF00688923