摘要
目的 探讨我国南方地区儿童髓鞘少突胶质细胞糖蛋白抗体(MOG抗体)相关复发性脱髓鞘病(RDD)的临床特点、治疗及预后.方法 对2015年1月至2018年12月在广州市妇女儿童医疗中心神经内科就诊的10例MOG抗体相关RDD患儿进行回顾性分析.采用年化复发率(ARRs)评估复发频率,扩展的残疾评分(EDSS)评估神经功能障碍.结果 患儿起病年龄为(6.4±3.6)岁,男女比例为4∶6.(1)临床表型:随访过程中共24次发作,首次发作脱髓鞘类型为急性播散性脑脊髓炎(ADEM)(7/10例)、视神经脊髓炎谱系障碍(NMOSD)(3/10例);14次复发依次为ADEM(9/14次)、视神经炎(ON)(3/14次)及脑干脑炎(2/14次).末次随访最终诊断为多相性播散性脑脊髓炎(MDEM)(6/10例),NMOSD(3/10例),ADEM伴ON(ADEM-ON)(1/10例).(2)实验室检查:所有患儿首发及复发时血MOG抗体阳性,首次发病时血MOG抗体滴度低组(1∶320)(4例)与滴度高组(≥1∶640)(6例)相比较,后者伴发ON(3例)、长节段脊髓炎(3例)多于前者.(3)影像学改变:急性期共行25次核磁共振(MRI)检查,头颅MRI主要累及皮质、皮质下白质.4例患儿脊髓MRI异常.(4)治疗及预后:急性期予静脉输注甲泼尼龙冲击(IVMP)联合静脉输注人免疫球蛋白(IVIG)治疗.维持期分别予利妥昔单抗(2/10例)、霉酚酸酯(4/10例)、每月1次IVIG(2/10例)及小剂量泼尼松口服维持(2/10例).加用上述治疗后ARRs由1.4降至0,EDSS明显改善.3例NMOSD、3例MDEM、1例ADEM-ON遗留神经功能障碍.结论 儿童MOG抗体相关的RDD中ADEM最常见,首次发病血MOG抗体滴度高者易伴发ON或长节段脊髓炎.复发患儿予免疫修饰治疗效果好,遗留的神经功能障碍与反复脱髓鞘的类型有关.
Objective To investigate the clinical characteristics,treatment and prognosis of relapsed demyeli-nating disease(RDD)associated with myelin oligodendrocyte glycoprotein antibodies(MOG abs)children in southern China.Methods Children with RDD associated with MOG abs at Department of Neurology in Guangzhou Women and Children′s Medical Center from January 2015 to December 2018 were retrospectively analyzed.The annualized relapse rates(ARRs)and expand disability status scale(EDSS)were used to assess the recurrence frequency and neurological dysfunction respectively.Results Ten children were included with the age of(6.4±3.6)years old,and male to female ratio was 4∶6.(1)Clinical phenotype:all children had 24 episodes during follow-up,with acute disseminated encephalomyelitis(ADEM)(7/10 cases)and neuromyelitis optica spectrum disorders(NMOSD)(3/10 cases)on the first episode.Among 14 recurrent episodes,ADEM(9/14 times)was the most common,followed by optic neuritis(ON)(3/14 times)and brainstem encephalitis(2/14 times).By the final follow-up,the final diagnosis was multiphasic disseminated encephalomyelitis(MDEM)(6/10 cases),NMOSD(3/10 cases),ADEM-ON(1/10 case),respectively.(2)Laboratory examination:all the children had positive serum MOG abs in the acute stage.The serum MOG abs titer high group(≥1∶640)(6 cases)on the first episode complicated ON(3 cases)and long segment myelitis(3 cases)more common than those of low group(1∶320)(4 cases).(3)Imaging changes:25 times of bain magnetic resonance imaging(MRI)were performed in the acute stage,MRI changes mostly involved the cortex and subcortical white matter.Four cases had abnormal spinal cord MRI.(4)Treatment and prognosis:intravenous methylprednone(IVMP)combined with intravenous immunoglobulin(IVIG)were administrated in acute stage.Rituximab(2/10 cases),mycophenolate mofetil(4/10 cases),IVIG(2/10 cases)monthly and low dose prednisone orally(2/10 cases)were given respectively in maintains stage.ARRs decreased from 1.4 to 0 and EDSS score improved significantly after
作者
侯池
李小晶
张雅妮
郑可鲁
曾意茹
梁惠慈
陈连凤
朱海霞
田杨
陈文雄
Hou Chi;Li Xiaojing;Zhang Ya′ni;Zheng Kelu;Zeng Yiru;Liang Huici;Chen Lianfeng;Zhu Haixia;Tian Yang;Chen Wenxiong(Department of Neurology,Guangzhou Women and Children′s Medical Center,Guangzhou 510120,China)
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2019年第23期1807-1811,共5页
Chinese Journal of Applied Clinical Pediatrics
