Epilepsy Monitoring Department, St. Luke's Hospital, Thessaloniki, Greece.
School of Electrical and Computer Engineering, National Technical University of Athens, Athens, Greece.
Epileptic Disord. 2023 Jun;25(3):343-359. doi: 10.1002/epd2.20062. Epub 2023 May 2.
Mild malformation with oligodendroglial hyperplasia (MOGHE) is a recently described clinicopathologic entity, associated with drug-resistant epilepsy and extensive epileptogenic networks. Knowledge is accumulating about particular electroclinical phenotypes, correlations with imaging, and potential prognostic significance for surgical outcomes. The study adds relevant information by documenting the presence of a hyperkinetic frontal lobe seizure phenotype in adolescents and an epileptic encephalopathy phenotype in young children.
Five cases were subjected to a structured presurgical evaluation protocol, including EEG-FMRI, chronic and acute invasive EEG, subjected to frontal lobe surgery with postoperative follow-up between 15 months and 7 years.
In the two adult cases, surface EEG demonstrated lateralized widespread frontal lobe epileptogenicity and hyperkinetic semiological features. MRI demonstrated cortical white matter blurring and deeper white matter abnormalities. EEG-FMRI suggested concordant frontal lobe involvement. iEEG demonstrated a widespread frontal lobe epilepsy network. The three young children demonstrated a diffuse epileptic encephalopathy phenotype, with nonlocalizing, nonlateralizing surface EEG, and "spasms" as the main seizure type. MRI demonstrated extensive frontal lobe subcortical gray and white matter abnormalities, consistent with MOGHE literature for this age, while EEG-FMRI, in 2/3, demonstrated concordant frontal lobe involvement. They did not undergo chronic iEEG, and the resection was assisted by acute intraoperative ECoG. All cases were subjected to extensive frontal lobectomies with Engel class IA (2/5), IB (1/5), and IIB (2/5) outcomes.
The study confirms the presence of frontal lobe epilepsy and epileptic encephalopathy phenotypes, in accordance with epilepsy phenotypes already described in MOGHE literature. Presurgical evaluation studies, including EEG-FMRI, can provide strong lateralizing and localizing evidence of the epileptogenic networks involved. All responded favorably to extensive frontal lobe resections, despite widespread epileptic activity recorded by surface and intracranial EEG pre- and postoperatively; an epileptic encephalopathy phenotype, in the first years of life, should not discourage such a resection.
轻度畸形伴少突胶质细胞增生（MOGHE）是一种最近描述的临床病理实体，与耐药性癫痫和广泛的致痫网络有关。关于特定的电临床表型、与影像学的相关性以及对手术结果的潜在预后意义，知识正在不断积累。该研究通过记录青少年的多动性额叶癫痫发作表型和幼儿的癫痫性脑病表型，提供了相关信息。
对 5 例患者进行了结构化术前评估方案，包括 EEG-FMRI、慢性和急性侵袭性 EEG，并进行了额叶手术，术后随访时间为 15 个月至 7 年。
在 2 例成年病例中，表面 EEG 显示局灶性广泛额叶致痫性和多动性症状。MRI 显示皮质白质模糊和深部白质异常。EEG-FMRI 提示额叶受累一致。iEEG 显示广泛的额叶癫痫网络。3 例幼儿表现为弥漫性癫痫性脑病表型，表面 EEG 无局灶性、无侧化，主要发作类型为“痉挛”。MRI 显示广泛的额叶皮质下灰质和白质异常，与该年龄组的 MOGHE 文献一致，而 2/3 的 EEG-FMRI 显示额叶受累一致。他们没有进行慢性 iEEG，切除由急性术中 ECoG 辅助。所有病例均行广泛额叶切除术，术后结果为 Engel Ⅰ A（2/5）、Ⅰ B（1/5）和Ⅱ B（2/5）。
该研究证实了额叶癫痫和癫痫性脑病表型的存在，与 MOGHE 文献中已经描述的癫痫表型一致。包括 EEG-FMRI 的术前评估研究可以提供强烈的致痫网络侧化和局灶性证据。尽管术前和术后表面和颅内 EEG 记录到广泛的癫痫活动，但所有病例均对广泛的额叶切除术反应良好；在生命的最初几年出现癫痫性脑病表型不应阻止这种切除。
