CORTICAL CYTOARCHITECTURAL AND IMMUNOHISTOCHEMICAL STUDIES ON ZELLWEGER SYNDROME

In two cases of Zellweger syndrome, Golgi studies revealed an irregular neuronal arrangement, the presence of immature neurons, poor dendritic aborization and poor spine development, all of which suggest abnormal morphogenesis and delayed maturation. In immunohistochemical studies with antisera against human catalase, negative staining of neurons suggested a decrease of catalase due to defects of microperoxisomes, and positive staining of myelination glia only in the internal capsule may have been related to delayed myelination. Abnormal peroxisomal membrane or its related metabolites may cause a migration disorder in intrauterine development and myelination disturbance in perinatal maturation.