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额叶癫痫伴少突胶质细胞增生的轻度皮质发育畸形(MOGHE):保加利亚首例病例报告

Mild malformation of cortical development with oligodendroglial hyperplasia in frontal lobe epilepsy (MOGHE): a report of the first case in Bulgaria.

作者信息

Metodiev Dimitar, Minkin Krassimir, Dimova Petia, Blumcke Ingmar, Coras Roland, Ruseva Margarita, Ganeva Rumiana, Parvanov Dimitar, Penkov Marin, Nachev Sevdalin

机构信息

Neuropathological Laboratory, "Saint Ivan Rilski" University Hospital, Sofia, Bulgaria.

Department of Clinical Pathology, Nadezhda Women's Health Hospital, Sofia, Bulgaria.

出版信息

Folia Neuropathol. 2025;63(2):209-216. doi: 10.5114/fn.2024.138751.

Abstract

Herein, we report the first case of mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) in Bulgaria. It is a newly recognised clinico-pathological entity with medically intractable focal epilepsy in paediatric patients. The patient of interest is a 9-year-old boy who has been suffering from refractory epilepsy since the age of three. Positron emission tomography revealed a consistent hypometabolism with maximum in the orbitofrontal and fronto-opercular cortex, as well as in the adjacent anterior insula and the anterior temporal regions. A left frontal corticotomy anterior from the precentral sulcus, left insulectomy and temporal disconnection were performed. Pathomorphological examination of the material from the resected brain tissues demonstrated oligodendroglial hyperplasia with blurring of grey-white-matter boundaries and presence of subcortical heterotopic neurones. Eighteen months post-surgically the patient is seizure-free and drug-free. The observed oligodendroglial hyperplasia with increased proliferative activity and heterotopic neurones in the white matter with blurring of grey-white-matter junctions are the histopathological hallmarks of MOGHE. More new cases are needed to establish further data about this distinct entity in frontal lobe epilepsy.

摘要

在此,我们报告保加利亚首例伴有少突胶质细胞增生和癫痫的轻度皮质发育畸形(MOGHE)病例。它是一种新认识的临床病理实体,见于患有药物难治性局灶性癫痫的儿科患者。感兴趣的患者是一名9岁男孩,自3岁起就患有难治性癫痫。正电子发射断层扫描显示一致的代谢减低,在眶额皮质和额眶皮质以及相邻的前岛叶和颞前区域最为明显。进行了中央前沟前方的左侧额叶皮质切开术、左侧岛叶切除术和颞叶离断术。对切除的脑组织材料进行的病理形态学检查显示少突胶质细胞增生,灰白质边界模糊,存在皮质下异位神经元。术后18个月,患者无癫痫发作且无需服药。观察到的少突胶质细胞增生、增殖活性增加、白质中存在异位神经元以及灰白质交界处模糊是MOGHE的组织病理学特征。需要更多新病例来建立关于额叶癫痫中这一独特实体的进一步数据。

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