αB-Crystallin negative astrocytic inclusions

We report on an unusual pathological finding of astrocytes, observed in the brain of a 16-year-old African American male with severe intellectual disability and spastic quadriplegia. The brain showed bilateral pericentral, perisylvian polymicrogyria and pachygyria, in conjunction with a large number of hypertrophic astrocytes with eosinophilic granular cytoplasmic inclusions. The astrocytic abnormality was more severe in the dysgenetic area but present throughout the cerebral cortex. Astrocytic inclusions stained with acid fuchsin, azocarmine and Holzer's stain, and were immunoreactive for GEAP, S-100, and ubiquitin, but not for alpha B-crystallin, filamin, vimentin, nestin, tau or alpha-synuclein. Based on the case and a review of the literature, the authors postulate that these astrocytic inclusions in the cerebral cortex reflect abnormalities in radial glial developmental processes, such as migration, differentiation, or glial neuronal interaction function during neuronal migration. (C) 2010 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.