伴癫痫发作的儿童抗髓鞘少突胶质细胞糖蛋白抗体相关疾病的临床分析

Clinical analysis of children with anti-myelin oligodendrocyte glycoprotein antibody-associated disorders complicated with seizures

目的 探讨抗髓鞘少突胶质细胞糖蛋白(anti-myelin oligodendrocyte glycoprotein,MOG)抗体相关疾病(MOG associated disorders,MOGAD)中伴癫痫发作患儿的临床特征。方法 回顾性收集2017年1月至2021年6月于首都儿科研究所附属儿童医院神经内科住院的MOGAD患儿的临床资料,分析患儿的癫痫发作特征,并比较伴或非癫痫发作MOGAD患儿临床特征的差异。结果 入组MOGAD患儿共48例,包括伴癫痫发作组13例(27.1%),非癫痫发作组35例(72.9%),两组患儿起病年龄及性别比无明显差异(t=-1.76,P=0.09;χ^(2)=1.33,P=0.25)。伴癫痫发作组患儿在18例次急性发作期出现癫痫发作,其中10例次(55.6%)表现为癫痫持续状态,这18例次临床综合征符合急性播散性脑脊髓炎诊断者7例次,皮质脑炎4例次,不能分类的表型4例次,孤立性癫痫发作3例次。与非癫痫发作组相比,伴癫痫发作组皮质受累更常见(χ^(2)=7.27,P=0.01),脊髓、小脑受累少见(χ^(2)=4.85,P=0.03;χ^(2)=4.31,P=0.04)。8例(61.5%)伴癫痫发作组患儿呈复发病程,与非癫痫发作组(22.9%)相比差异具有统计学意义(χ^(2)=4.76,P=0.03),且两组患儿复发次数具有明显差异(Z=-2.43,P=0.02)。患儿对急性期免疫治疗敏感,至末次随访多数未遗留症状性癫痫。结论 癫痫发作是MOGAD患儿的临床表现之一,约半数出现癫痫持续状态,对免疫治疗敏感,癫痫发作多呈良性转归。伴癫痫发作患儿复发性病程多见。

Objective To explore the features of children with anti-myelin oligodendrocyte glycoprotein autibody-associated disorders(MOGAD)complicated with seizures. Methods Clinical data were obtained from 48 children with MOGAD treated in Capital Institute of Pediatrics from January 2017 to June 2021. The features of the seizures were analyzed. The different clinical characteristics between children with seizures and those without seizures were evaluated.Results In this case series,thirteen children(27.1%)with MOGAD suffered from seizures and 35 children(72.9%)didn’t. There were no significant differences in the age of onset or the ratio of sex between children with and without seizures(t=-1.76,P=0.09;χ^(2)=1.33,P=0.25). Thirteen patients had 18 episodes with seizures. A status epilepticus was found in 10/18 episodes(55.6%). Among the 18 episodes,7 were acute disseminated encephalomyelitis,4 were cortical encephalitis,4 were undefined phenotype and 3 were isolated epilepsy. There was a higher proportion of cortical lesions in children with seizures as compared to those without seizures(χ^(2)=7.27,P=0.01). Less involvement of the spinal cord and cerebellum was found in children suffering from seizures(χ^(2)=4.85,P=0.03;χ^(2)=4.31,P=0.04). Relapses of neurological symptoms occurred in 8 of 13(61.5%)children with seizures,and in 8 of 35(22.9%)children without seizures(χ^(2)=4.76,P=0.03),and the number of relapse had significant difference between the two groups(Z=-2.43,P=0.02). All patients were sensitive to immunotherapy,and most of them remained seizure free. Conclusion Seizures are the clinical manifestation of MOGAD children,and half of them have status epilepticus. They are sensitive to immunotherapy and most seizures have good prognosis. Relapses mostly occur in children with MOG associated seizures.