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无脑回畸形的谱系:经磁共振成像分析的10例患者报告

The spectrum of lissencephaly: report of ten patients analyzed by magnetic resonance imaging.

作者信息

Barkovich A J, Koch T K, Carrol C L

机构信息

Department of Radiology, University of California, San Francisco, CA 94143.

出版信息

Ann Neurol. 1991 Aug;30(2):139-46. doi: 10.1002/ana.410300204.

Abstract

Clinical data and magnetic resonance imaging scans from 10 patients with lissencephaly were reviewed. Although 6 of the 10 patients were less than 1 year old at the time of their most recent examination, those who were old enough for developmental assessment were, with a single exception, severely developmentally delayed. All patients had anatomical features compatible with impaired neuronal migration but normal myelination and, consequently, adequate glial migration. The severity of the lissencephaly did not seem to correlate with the severity of the clinical manifestations. One patient showed less delayed development than anticipated, perhaps because the most severely involved region of the brain was the prefrontal area.

摘要

回顾了10例无脑回畸形患者的临床资料和磁共振成像扫描结果。尽管10例患者中有6例在最近一次检查时年龄小于1岁,但那些年龄足以进行发育评估的患者,除1例例外,均存在严重的发育迟缓。所有患者均具有与神经元迁移受损相符的解剖学特征,但髓鞘形成正常,因此神经胶质细胞迁移充分。无脑回畸形的严重程度似乎与临床表现的严重程度无关。1例患者的发育延迟程度低于预期,可能是因为大脑受累最严重的区域是前额叶区域。

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