Department of Pediatrics, Peking University First Hospital, Beijing, China.
Department of Radiology, Peking University First Hospital, Beijing, China.
Epilepsia Open. 2023 Sep;8(3):898-911. doi: 10.1002/epi4.12755. Epub 2023 May 16.
Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) is a new and rare histopathological entity of cortical developmental malformations. The clinical characteristics of MOGHE remain challenging.
Children with histologically confirmed MOGHE were retrospectively studied. The clinical findings, electroclinical and imaging features, and postoperative outcomes were analyzed, and previously published studies were reviewed up to June 2022.
Thirty-seven children were included in our cohort. Clinical characteristics included early onset in infancy (94.6% before 3 years), multiple seizure types, and moderate or severe delay. Epileptic spasm is the most common seizure type and initial manifestation. The lesions were mainly multilobar (59.5% multiple lobes and 8.1% hemispheres), and predominance in the frontal lobe was observed. The interictal EEG pattern was circumscribed or widespread. The prominent MRI characteristics were cortical thickening, cortical/subcortical hyperintense T2/FLAIR signal, and blurring at the GM and WM transition. Among the 21 children followed up for more than 1 year after surgery, 76.2% were seizure-free. Preoperative interictal circumscribed discharges and larger resections were significantly associated with a good postoperative outcome. The clinical features of 113 patients in the reviewed studies were similar to those we reported, but the lesions were mainly unilobar (73.5%) and Engel I was achieved in only 54.2% after surgery.
Distinct clinical characteristics in MOGHE, especially age at onset, epileptic spasm, and age-related MRI characteristics, can help in early diagnosis. Preoperative interictal discharge and surgical strategy may be predictors of postoperative outcomes.
伴少突胶质细胞增生的轻度皮质发育不良伴癫痫（MOGHE）是一种新的罕见皮质发育畸形的组织病理学实体。MOGHE 的临床特征仍然具有挑战性。
对经组织学证实的 MOGHE 患儿进行回顾性研究。分析临床发现、电临床和影像学特征以及术后结果，并对截至 2022 年 6 月发表的研究进行综述。
我们的队列纳入了 37 名儿童。临床特征包括婴儿期（94.6%在 3 岁前）发病早、多种癫痫发作类型以及中重度发育迟缓。癫痫性痉挛是最常见的发作类型和初始表现。病变主要为多脑叶（59.5%多个脑叶和 8.1%半球），以额叶为主。发作间期脑电图模式为局限性或广泛性。突出的 MRI 特征是皮质增厚、皮质/皮质下 T2/FLAIR 信号高信号和 GM 与 WM 交界处模糊。在术后随访超过 1 年的 21 名儿童中，76.2%无癫痫发作。术前发作间期局限性放电和更大的切除范围与术后良好结局显著相关。我们报道的病例与回顾性研究中 113 例患者的临床特征相似，但病变主要为单侧（73.5%），术后仅 54.2%达到 Engel I 级。
MOGHE 的独特临床特征，尤其是发病年龄、癫痫性痉挛和年龄相关的 MRI 特征，有助于早期诊断。术前发作间期放电和手术策略可能是术后结局的预测因素。
