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弥漫性神经元迁移障碍的离心性停滞阶段及模式

Stages and patterns of centrifugal arrest of diffuse neuronal migration disorders.

作者信息

Palmini A, Andermann F, de Grissac H, Tampieri D, Robitaille Y, Langevin P, Desbiens R, Andermann E

机构信息

Department of Neurology and Neurosurgery, McGill University, Montreal, PQ, Canada.

出版信息

Dev Med Child Neurol. 1993 Apr;35(4):331-9. doi: 10.1111/j.1469-8749.1993.tb11645.x.

Abstract

Centrifugal migration of newly generated neuroblasts toward the cortical surface can be arrested at different levels, resulting in anatomically different disorders. This is illustrated by three patients with diffuse or generalized neuronal migration disorders (NMDs): pachygyria, subcortical laminar heterotopia ('double-cortex' syndrome), and periventricular laminar heterotopia. All had medically intractable partial and generalized epileptic seizures, but there was no close correlation between the type of dysplasia and intelligence or clinical pattern. The low intelligence found in these patients may relate to the epileptic syndrome, rather than to the NMDs alone. MRI suggested that in this spectrum of disorders the migration process was arrested at different stages, depending on the extent, timing and site of damage to radial glial fibres.

摘要

新生成的神经母细胞向皮质表面的离心迁移可在不同水平受阻,从而导致解剖结构上不同的紊乱。三名患有弥漫性或全身性神经元迁移障碍(NMDs)的患者说明了这一点:巨脑回、皮质下板层异位(“双皮质”综合征)和脑室周围板层异位。他们都患有药物难治性部分性和全身性癫痫发作,但发育异常的类型与智力或临床模式之间没有密切关联。这些患者智力低下可能与癫痫综合征有关,而不仅仅是与NMDs有关。MRI显示,在这一系列疾病中,迁移过程根据放射状胶质纤维受损的程度、时间和部位在不同阶段受阻。

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