Pachygyria and congenital nephrosis disorder of migration and neuronal orientation

A case of pachygyria with associated nephrosis has been studied. Several microscopic abnormalities have been identified: cytoarchitectonic disorders including neuronal ectopies in the molecular layer and in the meninges, improperly oriented neurons shown with Golgi stain, fetal aspect of inferior olives. The mechanism of the disorder of migration and neuronal and dendritic orientation are discussed. The significance of the association of microcephaly and nephrosis is also reviewed in light of recent literature.