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局灶性穿通性发育异常:一种特定的皮质发育畸形。

Focal transmantle dysplasia: a specific malformation of cortical development.

作者信息

Barkovich A J, Kuzniecky R I, Bollen A W, Grant P E

机构信息

Department of Radiology, University of California, San Francisco 94143-0628, USA.

出版信息

Neurology. 1997 Oct;49(4):1148-52. doi: 10.1212/wnl.49.4.1148.

DOI:10.1212/wnl.49.4.1148
PMID:9339707
Abstract

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.

摘要

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。磁共振成像(MRI)显示信号异常从皮质延伸至侧脑室的上外侧壁。组织学检查显示皮质结构紊乱、神经元巨细胞、气球样细胞、皮质灰质 - 白质交界不清以及不同程度的伴随星形胶质细胞增生。我们认为这种跨壁发育异常是干细胞发育异常导致的一种特殊异常情况。

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