以孤立性癫癎发作为表型的儿童抗髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床特征分析

Clinical Features of Anti-myelin Oligodendrocyte Glycoprotein Antibody-associated Diseases in Children with Isolated Seizures Onset as Phenotype

目的探讨抗髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的孤立性癫癎发作患儿的临床特点。方法回顾性分析2019至2021年于河北省儿童医院神经内科住院,血清抗MOG抗体检测阳性,病程中以孤立性癫癎发作为表型患儿的临床特点。使用基于转染细胞(CBA)的间接免疫荧光法检测抗MOG抗体。结果共3例患儿病程中存在1次及以上的癫癎发作,同时未达到中枢神经系统脱髓鞘综合征的临床或放射学标准。共6次癫癎发作中,局灶性发作5次,局灶继发双侧强直阵挛性发作1次。脑脊液白细胞存在不同程度升高(15~69×10^(6)/L),视频脑电图正常或局灶性慢波。所有患儿均予以丙种球蛋白及糖皮质激素治疗,未加用抗癫癎发作药物,随访期间无复发病例。结论抗MOG抗体可能介导癫癎发作,对于孤立性局灶性癫癎发作的学龄期患儿,应考虑检测MOG抗体,免疫治疗有效,预后好。

Aim To explore the clinical features of anti-myelin oligodendrocyte glycoprotein(MOG)antibody-positive isolated seisures in children.Methods Retrospective analysis was performed on the clinical characteristics of the children admitted to the department of neurology of Hebei Children’s Hospital from 2019 to 2021 with positive serum anti-mog antibody and isolated seizures as the phenotype during the course of the disease.Cell-based assays(CBA)were used to test MOG antibodies.Resluts Three children had the phenotype of isolated seizures during the course of the disease and did not meet the clinical or radiological criteria for central nervous system demyelination syndrome.Of the 6 seizures,5 were focal seizures and 1 was focal secondary to bilateral tonic-clonic seizure.White blood cell(WBC)counts of cerebrospinal fluid increased,ranging from 15×10^(6)/L to 69×10^(6)/L,and the video EEGs were normal or had focal slow waves.All patients recovered well after the treatment with intravenous immunogloblin and glucocorticoid.None had recurrent seizures during follow-up.Conclusion AntiMOG antibodies may mediate seizures.For school-age children with isolated focal seizures,MOG antibody detection should be considered.Immunotherapy is effective and the prognosis is good.