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额叶离断术治疗额叶伴有少突胶质细胞增生的轻度皮质发育不良性癫痫(MOGHE)

Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe.

机构信息

Pediatric Epilepsy Center, Peking University First Hospital.

Department of Pediatrics, Peking University First Hospital.

出版信息

J Vis Exp. 2024 Aug 16(210). doi: 10.3791/66970.

Abstract

Malformation of cortical development is an important cause of drug-resistant epilepsy in young children. Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) has been added to the last focal cortical dysplasia (FCD) classification and commonly involves the frontal lobe. The semiology at the onset of epilepsy is dominated by non-lateralizing infantile spasm; the boundaries of the malformation are usually difficult to determine by magnetic resonance imaging (MRI) and positron emission tomography (PET), and electroencephalography (EEG) findings are often widespread. Therefore, the traditional concept and strategy of preoperative evaluation to determine the extent of the epileptogenic zone by comprehensive anatomo-electro-clinical methods are difficult to implement. Frontal disconnection is an effective surgical method for the treatment of epilepsy, but there are few related reports. A total of 8 children with histo-pathologically confirmed MOGHE were retrospectively studied. MOGHE was located in the frontal lobe in all patients, and frontal disconnection was performed. The periinsular approach was used in the disconnective procedures, divided into several surgical steps: the partial inferior frontal gyrus resection, the frontobasal and intrafrontal disconnection, and the anterior corpus callosotomy. One patient presented with a short-term postoperative speech disorder, while another patient exhibited transient postoperative limb weakness. No long-term postoperative complications were observed. At 2 years after surgery, 75% of patients were seizure-free, with cognitive improvement in half of them. This finding suggested that frontal disconnection is an effective and safe surgical procedure for the treatment of MOGHE instead of extensive resection in the frontal lobe.

摘要

皮质发育畸形是小儿耐药性癫痫的重要原因。癫痫伴少突胶质细胞增生性皮质发育不良(MOGHE)已被纳入最后一次局灶性皮质发育不良(FCD)分类,通常涉及额叶。癫痫发作的起始症状主要为非局灶性婴儿痉挛;MRI 和正电子发射断层扫描(PET)通常难以确定畸形的边界,脑电图(EEG)结果通常广泛存在。因此,传统的术前评估概念和策略,即通过综合解剖-电-临床方法来确定致痫灶的范围,难以实施。额叶离断是治疗癫痫的有效手术方法,但相关报道较少。本研究回顾性分析了 8 例经组织病理学证实的 MOGHE 患儿。所有患者的 MOGHE 均位于额叶,并进行了额叶离断术。离断术采用岛叶周围入路,分为几个手术步骤:部分额下回切除术、额基底和额内离断术、胼胝体前部切开术。1 例患者术后出现短期言语障碍,1 例患者出现短暂性术后肢体无力。无长期术后并发症。术后 2 年,75%的患者无癫痫发作,其中一半患者认知功能改善。这一发现表明,额叶离断术是治疗 MOGHE 的有效且安全的手术方法,而非广泛切除额叶。

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