Focal transmantle dysplasia: A specific malformation of cortical development

Focal transmantle dysplasia: A specific malformation of cortical development
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DOI:
10.1212/wnl.49.4.1148
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发表时间:
1997-10-01
期刊:
影响因子:
9.9
通讯作者:
Grant, PE
Grant, PE
中科院分区:
医学1区
文献类型:
--
作者:
Barkovich, AJ;Kuzniecky, RI;Grant, PE

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我们报告了18例20岁之前出现癫痫或固定神经功能缺损的患者。MRI显示异常信号从皮质延伸至侧脑室上外侧壁。组织学检查显示皮质紊乱、神经元细胞肥大、气球样细胞、不清楚的皮质灰质-白质连接和可变伴随星形胶质细胞增生。我们认为,这种transmantle发育不良是一种特殊的异常导致异常干细胞发育。
We report 18 patients who presented prior to age 20 years with epilepsy or fixed neurologic deficits. MRI showed signal abnormality extending from the cortex to the superolateral wall of the lateral ventricle. Histology showed cortical disorganization, neuronal cytomegaly, balloon cells, indistinct cortical gray matter-white matter junctions, and variable accompanying astrogliosis. We propose that this transmantle dysplasia is a specific anomaly resulting from abnormal stem cell development.