Kadhim H J, Lammens M, Gosseye S, Gadisseux J F, Evrard P
Pediatric Neurology Service, University of Louvain Medical School, Brussels, Belgium.
Pediatr Pathol. 1993 Jul-Aug;13(4):519-36. doi: 10.3109/15513819309048240.
Defects of neuronal migration were detected in the brains of five unrelated infants with Potter syndrome (oligohydramnios sequence). These consisted of abnormal lamination of cerebral cortex, white matter heterotopias, and meningeal and molecular zone neuronal-glial ectopias. Besides, various other brain anomalies were sometimes found. They comprised one or more of the followings: abnormal gyration patterns (gyral fusion, cerebellar microgyria), cerebellar granule and Purkinje cell heterotopias, brain stem heterotopias, adysplasia of basal ganglia, gliosis, mineralization, and hydrocephalus. Detailed investigations, using standard neuropathologic stains, immunohistochemical and Golgi methods, and a new electron microscopic histochemical technique that we applied to study the developing human brain, suggest that migration defects of neurons are caused by an abnormality in their glial guides, the radial glial fibers, during the period of cortical histogenesis. We hypothesize that abnormally and precociously induced radial glial transformation into astrocytes is the pathogenic mechanism for the defects in neuronal migration. The etiological factor(s) that precipitates such abnormal glial transformation seems to be heterogeneous. Its relation to the Potter anomaly is discussed.
在五名患有波特综合征（羊水过少序列）的无血缘关系婴儿的大脑中检测到神经元迁移缺陷。这些缺陷包括大脑皮质分层异常、白质异位、脑膜和分子层神经元 - 胶质细胞异位。此外，有时还会发现各种其他脑异常。它们包括以下一种或多种：异常脑回模式（脑回融合、小脑微小脑回）、小脑颗粒细胞和浦肯野细胞异位、脑干异位、基底神经节发育异常、胶质增生、矿化和脑积水。使用标准神经病理染色、免疫组织化学和高尔基方法以及我们应用于研究发育中的人类大脑的一种新的电子显微镜组织化学技术进行的详细研究表明，神经元迁移缺陷是由皮质组织发生期间其胶质引导物——放射状胶质纤维的异常所引起的。我们推测，放射状胶质细胞异常且过早地转化为星形胶质细胞是神经元迁移缺陷的致病机制。引发这种异常胶质细胞转化的病因似乎是异质性的。本文讨论了其与波特异常的关系。
