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KMID : 0377220020270010212
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Medical Journal of Chosun Univercity
2002 Volume.27 No. 1 p.212 ~ p.217
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Two Cases of Schilder’s Myelinoclastic Diffuse Sclerosis
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Cho Kyung-Won
Jung Won-Young
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Abstract
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The myelinoclastic diffuse sclerosis of true Schilder’s disease(MDS), characterized clinically by subacute or chronic progressive dementia, visual disturbance, hemi- or quadri-plegia, and mental detrioration, and pathologically by bilateral demyelinating plaque involving centrum semiovale of the cerebral hemispheres, is a rare variant of multiple sclerosis. For the differential diagnosis with other leucoencephalopathy, it is essential to check very long chain fatty acids.
We report two suspected adult cases of MDS with different steroid responsiveness. One presented with bipolar disease and cortical blindness and the other presented with stuporous mentation and generalized rigidity. Brain MR imagings revealed diffuse, homogenous lesions in the bilateral subcortical white matter. EEGs showed marked generalized slow waves without normal background activity. Stereotaxic biopsy of the brain was performed in one patient and it showed non-specific gliosis pathologically. One responded to steroid well, but the other did not. CONCLUS10N AND COMMENT : Two different suspected Schilder’s MDS were presented with a review of the literature.
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KEYWORD
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Schilder’s myelinoclastic diffuse sclerosis, MDS
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