[Congenital muscular dystrophy associated with micropolygyria of the cerebrum and cerebellum].
The characteristic features of micropolygyria were demonstrated in the cerebral and cerebellar gray matter of two cases, 5 and 16 years old males, of congenital muscular dystrophy. (Fokuyama type). The micropolygyria of the cerebral cortex showed an undifferentiated cytoarchitecture, e. g. status verrucosus deformis. On the surface of cortical molecular layer, subpial mesenchymal fibres, tangential myelin fibres and marginal glial fibres were observed to be still proliferated even at the stages examined and all the fibres were caved into the cortical cell layer associated with the molecular layer, without secondary sulcus formation. It is proposed that micropolygyria without secondary sulcus formation described in the present paper is named as "pachygyric micropolygyria", while four-layered type of micropolygyria with normal sulcus formation is "eugyric micropolygyria". On the other hand, micropolygyria of the cerebellar cortex was found to be consisted of various sized fragments in which basic structures of the cerebellum were preserved. On a part of surface of the cerebellar cortex the cytoarchitecture of cell layers was shown in inverse order. The Purkinje cell were irregularly migrated and the external granular layer remained in the molecular layer showing ectopic features. Tangential myelin fibres, connecting with fibres of the folial white matter, were observed to exist on the surface of the internal granular layer.
D000293:Adolescent; D001921:Brain / Q000002:abnormalities*; D002531:Cerebellum / Q000002:abnormalities*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000151:congenital* / Q000473:pathology; D009186:Myelin Sheath / Q000473:pathology