Summary
A case of Schilder's diffuse sclerosis with a four-year history of progressive neurologic deterioration in a boy dying at ten years of age is reported. The asymmetry of the process was indicated during life by the clinical findings. A unicentric focus of demyelination was found with the initial stages in the occipital and temporal white matter and a subacute reaction in the rostral cerebral white matter, brain stem, and cerebellum. Electron microscopic study confirmed the extensive axonal loss. No particles compatible with a virus structure were identified.
Analysis of the regional composition of cerebral lipids demonstrated large deficits of cerebroside, sulfatide, cholesterol, total phospholipid, sphingomyelin, and ganglioside in the occipital lesion. A significant but less complete deficit of lipids was found at the lesion margin in frontal white matter. An increase of cholesterol esters was demonstrated at this site as well. The normal-appearing white matter of rostral frontal lobe showed a decreased level of ganglioside and normal concentrations of the other structural lipids. This decreased ganglioside concentration of grossly normal white matter suggest the advisability of considering Wallerian degeneration in interpreting the analytical data obtained in the study of the demyelinating disorders.
The morphological and biochemical findings indicate the progressive spread of the axonolytic and myelinolytic process in the pathogenesis of Schilder's diffuse sclerosis.
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Supported by U. S. Public Health grants NS 06926 and HE 11078. K.C.J. is a research trainee supported by U. S. Public Health grant GM 00404 and J. P. H. is a Special Fellow of USPHS in Neuropathology.
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Hogan, E.L., Joseph, K.C., Hurt, J.P. et al. Schilder's diffuse sclerosis: A biochemical and ultrastructural study of myelinoclastic demyelination. Acta Neuropathol 20, 85–95 (1972). https://doi.org/10.1007/BF00691126
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DOI: https://doi.org/10.1007/BF00691126