福山型先天性筋ジストロフィーにおける皮質病変(cortical dysplasia)の形成に関する神経病理学的研究 : 胎児脳における基底膜欠損の病因論的意義

概要

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Fukuyama type congenital muscular dystrophy (FCMD) is a progressive myopathy associated with malformation of the central nervous system. Cytogenetically, it is an autosomal recessive disease. The brain shows cortical dysplasia including mixed polymicrogyria and lissencephaly in the cerebrum and cerebellum. The lesions represent abnormal neuronal cell migration at the cortical surface covered with glio-mesenchymal tissue. We investigated three fetal brains; 17, 18, and 20 weeks. We found small irregular defects of basement membrane structure by periodic acid-Schiff staining. Electronmicroscopic examination revealed an abnormal configuration of the basement membrane. These findings suggest that the cerebral lesion is caused by a defective glia limitans-basal lamina complex.
東京女子医科大学の論文
1998-03-25