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一例因双侧脑裂畸形导致的先天性双侧外侧裂周综合征。

A case of congenital bilateral perisylvian syndrome due to bilateral schizencephaly.

作者信息

Vinayan K Puthenveettil, Terada Kiyohito

机构信息

National Epilepsy Center, Shizuoka Institute of Epilepsy and Neurological Disorders, Aoi-ku, Shizuoka, Japan.

出版信息

Epileptic Disord. 2007 Jun;9(2):190-3. doi: 10.1684/epd.2007.0108.

DOI:10.1684/epd.2007.0108
PMID:17525033
Abstract

A case of congenital bilateral perisylvian syndrome (CBPS) associated with bilateral perisylvian schizencephaly in a 24-year-old woman is reported. She presented the classical clinical triad of CBPS, which included congenital facio-masticatory diplegia, epilepsy and only mild mental retardation, despite the presence of bilateral, open-lip clefts in the perisylvian region. We hypothesize that the minimal loss of cortical tissue, along with the possible sparing of vital white matter association fibers and neuronal plasticity might have contributed to the better functional outcome in this patient.

摘要

报告了一例24岁女性先天性双侧外侧裂周综合征(CBPS)合并双侧外侧裂周脑裂畸形的病例。她表现出CBPS的典型临床三联征,包括先天性面咀嚼肌双侧瘫痪、癫痫发作,尽管外侧裂周区域存在双侧开放性唇裂,但仅有轻度智力障碍。我们推测,皮质组织的最小程度损失,以及重要的白质联合纤维可能得以保留和神经元可塑性,可能促成了该患者较好的功能预后。

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