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单侧额顶叶脑回发育不良伴同侧脑桥发育不良和对侧代偿性增生。

Unilateral perirolandic polymicrogyria with ipsilateral brainstem hypoplasia and compensatory contralateral hyperplasia.

机构信息

Department of Neuroradiology, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal.

Department of Neurology, Centro Hospitalar Lisboa Ocidental, Lisbon, Portugal.

出版信息

Neurol Sci. 2023 Jul;44(7):2617-2619. doi: 10.1007/s10072-023-06657-4. Epub 2023 Mar 2.

Abstract

Polymicrogyria (PMG) is a malformation of cortical development that occurs mostly in the perisylvian region bilaterally (60-70%), most often presenting with epilepsy. Unilateral cases are much rarer with hemiparesis being the predominant symptom. We report a case of a 71-year-old man with right perirolandic PMG with ipsilateral hypoplasia and contralateral hyperplasia of the brainstem, with only non-progressive left-sided mild spastic hemiparesis. This imaging pattern is thought to occur due to the normal process of withdrawal of the axons of the corticospinal tract (CST) connected to aberrant cortex, possibly with compensatory contralateral CST hyperplasia. However, the majority of cases is additionally present with epilepsy. We believe it is worthwhile to investigate imaging patterns of PMG with symptoms' correlation, particularly with the help of techniques such as advanced brain imaging to assist in the study of cortical development along with adaptive somatotopic organization of the cerebral cortex in MCD with possible clinical applications.

摘要

多微小脑回畸形(PMG)是一种皮质发育畸形,主要发生在双侧的岛叶周围区域(60-70%),多数伴有癫痫。单侧病例则更为罕见,以偏瘫为主要症状。我们报告了一例 71 岁男性,右侧岛盖旁 PMG 伴同侧脑桥发育不良和对侧脑桥增生,仅表现为左侧轻度痉挛性偏瘫且病情无进展。这种影像学模式被认为是由于与异常皮质相连的皮质脊髓束(CST)轴突正常退缩过程引起的,可能伴有对侧 CST 代偿性增生。然而,大多数病例还伴有癫痫。我们认为,值得对具有症状相关性的 PMG 影像学模式进行研究,特别是借助先进的脑成像技术,辅助研究皮质发育以及大脑皮质的适应性躯体组织的变化,这可能具有临床应用价值。

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