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[一例因单侧脑裂畸形导致症状性癫痫和镜像运动的病例]

[A case with symptomatic epilepsy and mirror movement due to unilateral schizencephaly].

作者信息

Takajo I, Ohi T, Shiomi K, Sugimoto S, Matsukura S

机构信息

Department of Internal Medicine, Miyazaki Medical College, Japan.

出版信息

No To Shinkei. 2000 Jul;52(7):617-20.

Abstract

Schizencephaly is an extremely rare congenital disease caused by abnormal neuronal migration. The etiology of schizencephaly is not established but vascular disturbance in early childhood could cause this condition. We have cared of a patient with schizencephaly. The patient was 47 year old male. He had focal motor seizure with secondary generalization. Neurological examination revealed, mild left hemiparesis, left pyramidal signs with no sensory impairment, left hemiatrophy, and mirror movement. MRI findings showed schizencephaly, open lip type(type II) in right cerebral hemisphere. His epileptic seizure was controlled by administration of sodium valproate. The possible mechanism of this mirror movement in his left hand and leg could be reorganization of non-affected brain and disinhibition on homolateral pyramidal tract in non-affected left cerebral hemisphere by the transcallosal inhibitory pathyway from affected right cerebral hemisphere. Sodium valpronate can not suppress this mirror movement.

摘要

脑裂畸形是一种由神经元迁移异常引起的极为罕见的先天性疾病。脑裂畸形的病因尚未明确,但儿童早期的血管紊乱可能导致这种病症。我们照料过一名患有脑裂畸形的患者。该患者为47岁男性。他有局灶性运动性癫痫发作并继发全身性发作。神经系统检查发现,轻度左侧偏瘫、左侧锥体束征且无感觉障碍、左侧半身萎缩以及镜像运动。磁共振成像(MRI)结果显示脑裂畸形,右侧大脑半球为开放性唇型(II型)。他的癫痫发作通过服用丙戊酸钠得到控制。其左手和左腿出现这种镜像运动的可能机制可能是未受影响的大脑进行了重组,以及受影响的右侧大脑半球通过胼胝体抑制通路对未受影响的左侧大脑半球同侧锥体束的去抑制作用。丙戊酸钠无法抑制这种镜像运动。

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