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脑裂畸形患者中年轻成人的神经心理学功能。

Neuropsychological functioning in a young adult case of schizencephaly.

机构信息

Health Psychology, University of Missouri, Columbia, MO 65212, USA.

出版信息

Clin Neuropsychol. 2010;24(5):827-40. doi: 10.1080/13854041003627787. Epub 2010 Mar 23.

DOI:10.1080/13854041003627787
PMID:20336580
Abstract

Schizencephaly is a rare neuromigrational/organizational disorder characterized by the development of cerebral clefts, which are typically associated with neurological sequelae including seizures, motor disturbances, and cognitive dysfunction. Although there are multiple case reports of schizencephaly and associated neurological sequelae, primarily in children, the literature regarding neuropsychological manifestations of schizencephaly is limited. This article reviews the case of a woman diagnosed with bilateral schizencephaly at age 29. Neuropsychological testing revealed intact intelligence and memory functioning. However, impairments were noted in attention, executive functioning, expressive language skills, visual-spatial abilities, and bilateral manual motor skills, all of which were adversely impacting her functional abilities (e.g., ability to be gainfully employed). Given the potential variability in deficits associated with schizencephaly, this case demonstrates the utility of neuropsychological evaluation for understanding cognitive and functional consequences of bilateral schizencephaly.

摘要

脑裂畸形是一种罕见的神经迁移/组织障碍,其特征是大脑裂隙的发育,通常与包括癫痫发作、运动障碍和认知功能障碍在内的神经后遗症相关。尽管有多个脑裂畸形和相关神经后遗症的病例报告,主要是在儿童中,但关于脑裂畸形的神经心理学表现的文献有限。本文回顾了一位 29 岁女性被诊断为双侧脑裂畸形的病例。神经心理学测试显示智力和记忆力正常。然而,注意力、执行功能、表达性语言技能、视觉空间能力和双侧手动运动技能受损,这些都对她的功能能力(例如,就业能力)产生了不利影响。鉴于脑裂畸形相关缺陷的潜在可变性,该病例证明了神经心理学评估对于理解双侧脑裂畸形的认知和功能后果的效用。

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