A Golgi study of the cerebral cortex in Fukuyama-type congenital muscular dystrophy, Walker-type "lissencephaly," and classical lissencephaly

Abstract

The cortical neuronal organization of the brain was studied and compared in three conditions. The neurons in the polymicrogyric cortex from a subject with Fukuyama-type congenital muscular dystrophy and from a child with ocular dysplasia (Walker-type lissencephaly) were large, irregularly aligned, and not completely mature. In the former condition, the abnormal neuronal arrangement was predominantly in the superficial cortex; in the latter both the superficial and deep cortex were involved. These two diseases may have a similar pathogenetic mechanism. In classical lissencephaly without evidence of other disease manifestations, the neurons were arranged in a radial pattern with heterotopia but no large neurons. The pathogenesis of these conditions is discussed.