以脑白质营养不良样影像表现的儿童复发型MOGAD四例

Four cases of recurrent MOGAD in children with leukodystroph-like imaging

目的抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(MOGAD)在儿童中的比例明显高于成人。随着MOGAD表型的不断更新,一些少见表型的临床及影像特征、用药策略仍未明确。方法分析4例头颅磁共振(MRI)表现为脑白质营养不良样表现的复发型MOGAD儿童患者的临床特征。结果患儿年龄均为4岁以下,头颅MRI示脑白质受损,病变对称及部位广泛,形状大小不一,呈脑白质营养不良样改变;脑电图(EEG)示正常或连续/间断的慢波、棘波/尖慢波;脑脊液(CSF)提示白细胞正常或增多,蛋白正常或轻度升高;采用CBA法检测血清抗MOG-IgG阳性,其他自身免疫抗体及基因遗传检查均为阴性。尽管进行了2次的一线免疫治疗,患儿仍呈复发病程,或应用二线免疫治疗后病情趋于稳定,3例遗留不同程度的神经系统功能障碍。结论对于发病年龄小、头颅MRI呈脑白质营养不良样改变的患儿,临床医师应尽早进行MOG-IgG检测,确诊后避免激素快速减量或尽早使用二线药物免疫治疗,尽可能减少复发及后遗表现。

Objective The prevalence of anti-myelin oligodendrocyte glycoprotein(MOG)-IgG associated disorders(MOGAD)is significantly higher in children than in adults.As the continuous updating of phenotypes with MOGAD,the clinical and radiological characteristics of the rare phenotypes and drug strategies remain unclear.Methods We analyzed and described the clinical characteristics of the four patients who showed leukodystrophy-like imagings and relapse courses with MOGAD in children.Results The ages of the children were all less than 4 years old.Brain MRI showed white matter lesions involvement,which were symmetrical and extensive,with different shapes and sizes,presenting with leukodystrophy-like lesions.Electroencephalogram(EEG)showed normal or continuous/intermittent slow waves,spikes/sharp slow waves.Cerebrospinal fluid(CSF)analysis showed pleocytosis with normal or slightly increased protein levels.The MOG-IgG of the serum were positive detected by cell-based assay(CBA)method.Despite first-line immunotherapyn for two times,the patients still had recurrent courses until second-line immunotherapy,and 3 of them remained functional impairments to some extent.Conclusion Clinicians should detect MOG-IgG as soon as possible for children with young ages and leukodystrophy-like images in brain MRI.Rapid hormone reduction should be avoided and use second-line drug immunotherapy after diagnosis early for these patients to prevent recurrence and neurologic sequelae.