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双皮质综合征:癫痫发作的一种罕见病因。

Double Cortex Syndrome: An Unusual Cause of Seizures.

作者信息

Nedday Soumia, Naji Yahya, Assardoun Mariam, Laadami Sara, Adali Nawal

机构信息

Department of Neurology, Agadir University Hospital, Agadir, MAR.

Department of Neurology, Neurosciences Innovation Cognition Ethique (NICE) Research Team, Rein Endocrinologie Gastroentérologie Neurosciences Ethique (REGNE) Research Laboratory, Faculty of Medicine and Pharmacy, Ibn Zohr University, Agadir, MAR.

出版信息

Cureus. 2024 Jun 30;16(6):e63507. doi: 10.7759/cureus.63507. eCollection 2024 Jun.

Abstract

Gray matter heterotopia (GMH) is caused by abnormal neuronal migration during brain development. Subcortical band heterotopia (SBH), or double cortex, is a rare variant of GMH that mainly affects female patients with epilepsy (PWE) with different degrees of mental retardation. We present the case of a 25-year-old woman who was admitted to the neurology department of our tertiary hospital with generalized tonic-clonic seizures. Her mother had a normal antenatal period and a history of labor. There was a history of immediate crying and normal appearance, pulse, grimace, activity, and respiration (APGAR) scores. She had delayed milestones, which affected various categories of child development. Physical examination revealed a global developmental delay. Laboratory values, including complete blood count, serum calcium, and arterial blood gas tests, were all within normal limits. An EEG showed significant abnormalities suggestive of epilepsy. An MRI of the brain showed a continuous band of gray matter located deep and parallel to the cortex in both cerebral hemispheres, suggesting double cortex syndrome (DCS).

摘要

灰质异位症(GMH)是由大脑发育过程中神经元迁移异常引起的。皮质下带状异位症(SBH),即双皮质,是GMH的一种罕见变体,主要影响患有不同程度智力障碍的女性癫痫患者(PWE)。我们报告一例25岁女性患者,因全身强直阵挛发作入住我院三级医院神经内科。她的母亲孕期正常,有分娩史。出生时有立即啼哭,外观、脉搏、面部表情、活动和呼吸(阿氏评分)正常。她有发育迟缓,影响了儿童发育的各个方面。体格检查显示全面发育迟缓。实验室检查值,包括全血细胞计数、血清钙和动脉血气检查,均在正常范围内。脑电图显示有明显异常,提示癫痫。脑部磁共振成像显示双侧大脑半球深部有一条与皮质平行的连续灰质带,提示双皮质综合征(DCS)。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00d6/11288217/9e3f8e855f2f/cureus-0016-00000063507-i01.jpg

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