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Washington University Experience | NEURODEGENERATION | Polyglucosan Body Disease (PGBD) | 9A1 Polyglucosan Body Dz (AANP 1995, Case 5) LFB-PAS X1.5 1
At autopsy the unfixed brain weighed 975 g. The published findings (PMID: 9048988) include diffuse cerebral cortical atrophy and cerebellar atrophy. Degeneration of the lateral corticospinal tracts and dorsal columns was present. The posterior roots showed a mild loss of myelinated fibers while the anterior roots appeared intact. Sections of the sciatic nerve and brachial plexus showed a marked loss of myelinated fibers. ---- 9A1 This LFB-PAS stained whole mount of the spinal cord in this case shows areas of pallor in the lateral corticospinal tracts and dorsal columns. (LFB-PAS)