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- W3021781002 abstract "We report a case of 32–year–old man with myoclonus, rapidly progressive dementia, apraxia, ataxic gait and subtle right hemiparesis. The clinical and first pathological diagnosis was Creutzfeldt Jakob disease. A brain biopsy was obtained from the left temporoparietal area. Autopsy was performed one hour after death. Formalin–fixed, paraffin embedded tissues were used for routine and immunohistochemical microscopic stainings. Part of the autopsy material was fixed in 4% glutaraldehyde in cacodylate buffer for electron microscopy. On microscopic examination apart from neuronal loss and minimal spongiform change the most striking feature was presence of abundant eosinophylic, round, plaques with distinct borders–cotton wool plaques. These plaques were observed in the cerebral cortex, more frequently in the occipital and temporal than frontal lobes. The classic amyloid plaques were also sporadically seen in the cerebral cortex. In cerebellar cortex the cotton wool plaques were also abundant, the classic plaques were sparse in the pyramidal layer. In Congo staining the cotton wool plaques didn't show green birefringence in polarized light. The immunohistochemistry with antibodies for Ab40 and Ab42/43 showed immunoreactivity for the Ab42/43 but not for Ab40 antibody within the cotton wool plaques. The classic plaques showed classical pattern of immunoreactivity by bounding Ab40 and Ab42 in the central core and Ab42/43 in the surrounding. There was also a robust Ab42 and Ab40 immunoreactivity within the cerebral vessel walls especially in cerebellum. There was also a robust Ab42/43 and Ab40 immunoreactivity within the cortical and meningeal vessel walls especially in cerebellum. The immunochemistry shows reactivity for the pathological form of PrP. The most striking ultrastructural feature was presence of dystrophic neuritis with robust autophagic vacuoles. The autophagic vacuoles were also observed in the cell bodies. The clinical history with myoclonus, rapidly progressive dementia in a young man suggested Creutzfeldt Jakob disease. After re–estimation of the whole case the CJD can be excluded and Alzheimer disease with cotton wool plaques must be diagnosed." @default.
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- W3021781002 date "2006-07-01" @default.
- W3021781002 modified "2023-09-27" @default.
- W3021781002 title "P3-198: A case of early onset Alzheimer's disease with cotton wool plaques but without spastic paraparesis" @default.
- W3021781002 doi "https://doi.org/10.1016/j.jalz.2006.05.1466" @default.
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