Generalized cortical dysplasia manifested by diffusely thick cerebral cortex.
Generalized cortical dysplasia manifested by diffusely thick cerebral cortex.
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广泛性皮质发育不良表现为大脑皮质弥漫性增厚。
DOI：
10.1001/archneur.1989.00520400090025
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发表时间：
1989
影响因子：
--
通讯作者：
Pierre Langevin
中科院分区：
文献类型：
--
作者：
Gilles Marchal;Frederick Andermann;D. Tampieri;Yves Robitaille;Denis Melanson;Barry Sinclair;André Olivier;Kenneth Silver;Pierre Langevin
单侧或双侧大脑回被描述为癫痫的原因之一，在某些情况下，还会导致发育迟缓。病变周边组织显示局灶性皮质发育不良改变。本文报道的证据表明，皮质发育不良也可能是全身性的。2例顽固性癫痫和智力低下患者表现为弥漫性异常，皮质较厚，回较浅，灰白质分界较差。一位患者接受了前膝盖骨切开术，使癫痫症状有了显著改善。活检材料上不能区分皮质层5和6。白质髓鞘较少，内含大量异位神经元。这种综合征是一种先天性神经元迁移障碍，存活时间较长，代表了一种轻度的无脑畸形。它可以在生活中通过计算机断层扫描或磁共振扫描来诊断。
Unilateral or bilateral rolandic macrogyria has been described as a cause of epilepsy and, in some cases, retardation. Tissue from the periphery of these lesions shows the changes of focal cortical dysplasia. Evidence reported herein suggests that cortical dysplasia may also be generalized. Two patients with intractable epilepsy and mental retardation had diffusely abnormal, thick cortex, shallow gyri, and poor demarcation of gray and white matter. One patient had an anterior callosotomy that led to considerable improvement of the epilepsy. Cortical layers 5 and 6 could not be differentiated on biopsy material. The white matter was poorly myelinated and contained clusters of heterotopic neurons. This syndrome, a congenital disorder of neuronal migration, with prolonged survival, represents a mild form of lissencephaly. It can be diagnosed during life by computed tomography or magnetic resonance scanning.