Diamandis Phedias, Chitayat David, Toi Ants, Blaser S, Shannon Patrick
Department of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, Canada; Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Department of Medical Genetics, Mount Sinai Hospital, Toronto, Canada; Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada.
Brain Dev. 2017 Jan;39(1):23-39. doi: 10.1016/j.braindev.2016.06.005. Epub 2016 Jul 9.
To characterise the early tissue changes of post encephaloclastic polymicrogyria in the human fetus.
We identified and reviewed the clinical histories and autopsy pathology of post ischemic fetal cerebral cortical injury at less than 30weeks gestational age (GA). The histology of local cortical abnormalities was examined with neuronal, glial, microglial and vascular immunohistochemical markers.
We identified eight cases ranging from 18 to 29weeks GA: 5 cases show full thickness cortical infarcts and 3 show periSylvian post-ischemic necrosis of the cerebral cortex. The maximal age is less than 10weeks after injury. There are abnormalities in gross fissuration as early as one month after injury. Disruption of the pia limitans was associated with a microglial and glial response and full thickness cortical injury. Macrophages were often seen accumulating deep to abnormal cortex. Hyperplasia of the subpial granular cell layer was universal in perilesional cortex. Cajal Retzius neuron hyperplasia, aggregation, and both superficial and deep displacement were noted. Where there was loss and dispersal of early cortical pyramidal neurons there was usually no pseudolaminar necrosis. Radial glia by 18weeks GA showed altered growth patterns and lateral branching. Altered migration of primitive elements was often prominent. Particularly prior to 20weeks GA subadjacent subplate neurons showed striking hypertrophy.
The array of histological changes encompasses all tissue elements of the affected brains, early in the evolution polymicrogyria. Although subpial alterations were ubiquitous, not all changes are referable to alterations in the pia limitans. The role of the necroinflammatory response in the genesis of abnormal cytoarchitecture deserves further study.
描述人类胎儿脑破坏后多小脑回畸形的早期组织学变化。
我们识别并回顾了妊娠龄小于30周（GA）的缺血性胎儿脑皮质损伤的临床病史及尸检病理。采用神经元、胶质细胞、小胶质细胞和血管免疫组化标记物检查局部皮质异常的组织学情况。
我们识别出8例GA为18至29周的病例：5例显示全层皮质梗死，3例显示大脑皮质外侧裂周围缺血后坏死。最大发病年龄在损伤后小于10周。损伤后1个月时即出现明显的沟回异常。软脑膜破坏与小胶质细胞和胶质细胞反应及全层皮质损伤相关。巨噬细胞常聚集于异常皮质深部。软膜下颗粒细胞层增生在病灶周围皮质普遍存在。观察到Cajal-Retzius神经元增生、聚集以及浅层和深层移位。在早期皮质锥体细胞丢失和分散的部位，通常无假层状坏死。18周GA时放射状胶质细胞显示生长模式改变和侧向分支。原始成分迁移改变通常较为显著。特别是在20周GA之前，相邻的皮质下板层神经元显示出明显肥大。
组织学变化涉及受累脑的所有组织成分，在多小脑回畸形演变早期即出现。虽然软膜下改变普遍存在，但并非所有变化都归因于软脑膜破坏。坏死性炎症反应在异常细胞结构形成中的作用值得进一步研究。
