摘要
目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体相关炎性脱髓鞘疾病(IDD)患儿的临床表现、影像学特征、治疗方案及临床预后。方法选择2014年6月1日至2020年9月30日,在中山大学孙逸仙纪念医院儿科确诊的7例MOG抗体相关IDD患儿为研究对象。其中,女性患儿为2例,男性为5例;平均年龄为8.3岁。回顾性分析该7例MOG抗体相关IDD患儿的临床特征、影像学检查结果、治疗方案及临床预后等。本研究遵循的程序符合2013年修订的《世界医学协会赫尔辛基宣言》要求。结果①初诊时,这7例患儿分别被误诊为急性播散性脑脊髓炎(ADEM)(2例)、视神经炎(ON)(2例)、多发性硬化症(MS)(1例)、脑膜炎(1例)及自身免疫性脑炎(AE)(1例)。其中年龄较小患儿临床表现为ON症状,而年龄较大者为ADEM症状。②7例患儿中,2例脑脊液检查结果显示白细胞计数(WBC)增高、蛋白水平增高;而脑脊液MOG抗体呈阳性为6例,外周血抗CASPR-2抗体呈阳性为1例;6例患儿颅内压>200 mmH 2O(1 mmH 2O=0.0981 kPa)。③7例患儿中,外周血WBC增高为2例,C反应蛋白(CRP)呈阳性为1例、红细胞沉降率(ESR)增高为1例。所有患儿外周血MOG抗体检测结果均呈阳性,而外周血抗CASPR-2抗体呈阳性为1例。④影像学检查结果显示,病变累及侧脑室周围为5例、基底节为3例,丘脑、脊髓和视神经各为2例,脑干和延髓各为1例,伴脑膜强化为1例;而且这7例患儿的病灶直径均>2 cm;年龄>5岁的3例患儿的病灶边界光滑、清晰。⑤治疗方案:1例患儿于急性期静脉输注甲泼尼龙10~30 mg/(kg·d)(每3天剂量递减1/2)+静脉注射免疫球蛋白(IVIG)2 g/(kg·d)×1 d,6例静脉输注地塞米松[起始剂量为0.5 mg/(kg·d),序贯减量至泼尼松1 mg/(kg·d)口服维持治疗]+IVIG 0.4 g/(kg·d)×5 d。治疗维持期内,对这7例患儿均进行泼尼松口服治疗,其中5例每个月静脉输注1剂IVIG,剂量为2 g/kg。对2例MOG抗体相关IDD复发患儿,均再次进行静脉输注甲泼尼龙+IVIG治疗,其中1例接受利妥昔单抗治疗的方案为375 mg/(m^(2)·次)×1次/周×4周。治疗后,5例患儿获得完全恢复,2例部分恢复,平均年复发率(ARR)为0.40次/年。结论MOG抗体相关IDD患儿的临床表现多样,早期易被临床误诊。MOG抗体可与AE相关抗体同时存在,影像学诊断可见多灶性病变。MOG抗体相关IDD虽为MOG抗体所介导,但是MOG抗原的免疫致病性,则与T、B淋巴细胞息息相关。MOG抗体对激素治疗敏感,即使在该病患儿多次复发的情况下,预后亦良好。
Objective To investigate clinical manifestations,imaging features,treatment and prognosis of children with myelin oligodendrocyte glycoprotein(MOG)antibody-associated inflammatory demyelination disease(IDD).Methods Seven children with MOG autoantibody-associated IDD diagnosed at the Department of Pediatrics,Sun Yat-Sen Memorial Hospital of Sun Yat-sen University from June 1,2014 to September 30,2020 were selected as research subjects,including 2 girls and 5 boys with an average age of 8.3 years old.The clinical features,imaging findings,treatment and prognosis of 7 children with MOG antibody-related IDD were analyzed retrospectively.The procedures followed in this study were in line with the requirements of the World Medical Association Declaration of Helsinki revised in 2013.Results①At initial diagnosis,the 7 cases were misdiagnosed as acute disseminated encephalomyelitis(ADEM,2 cases),optic neuritis(ON,2 cases),multiple sclerosis(MS,1 case),meningitis(1 case)and autoimmune encephalitis(AE,1 case).Among them,the younger children presented with ON symptoms,while the older children presented with ADEM symptoms.②Among the 7 cases,2 cases had elevated white blood cell count(WBC)and protein level in cerebrospinal fluid,6 cases with intracranial pressure>200 mmH 2O(1 mmH 2O=0.0981 kPa).All the children were positive for MOG antibody in peripheral blood,6 cases were positive for MOG antibody in cerebrospinal fluid,and 1 case was positive for anti-CASPR-2 antibody in peripheral blood.③Among the 7 children,the results of laboratory examination showed that WBC increased in 2 cases,and C-reaction protein(CRP)was positive and erythrocyte sedimentation rate(ESR)increased in 1 case in peripheral blood.All the children′s peripheral blood MOG antibody were positive,and 1 child′s peripheral blood anti-CASPR-2 antibody was positive.④The results of imaging examination showed that the lesions involved around the lateral ventricle in 5 cases,basal ganglia in 3 cases,thalamus,spinal cord and optic nerve in 2 cases,brainstem in 1 case and medulla oblongata in 1 case,meningeal enhancement in 1 case.The lesion diameters of 7 cases were larger than 2 cm.In addition,a clear boundary of lesions could be found in 3 children whose ages were more than 5 years old.⑤In the acute phase,1 child was treated with methylprednisolone[10-30 mg/(kg·d),dose halved every 3 days]and intravenous immunoglobulin(IVIG)[2 g/(kg·d)×1 d],and 6 children were treated with dexamethasone[initial dose of 0.5 mg/(kg·d)and IVIG 0.4 g/(kg·d)×5 d].During the maintenance period,all of them were treated with oral prednisone,and 5 of them were treated with IVIG(2 g/kg)every month.2 recurrent children were treated with methylprednisolone and IVIG again,and 1 of them was treated with rituximab(375 mg/m^(2),once a week for 4 times).After treatment,5 children recovered completely and 2 children partially recovered.The average annualized relapse rate(ARR)was 0.4 times per year.Conclusions MOG antibody-associated IDD has various clinical manifestations and is easy to be misdiagnosed in the early stage.MOG antibody and AE-related antibody can exist at the same time,and most of the imaging findings are multifocal lesions.Although MOG antibody-associated IDD is mediated by antibody,the immunopathogenicity of MOG antigen is closely related to T cells and B cells.MOG-antibody is sensitive to hormones and has a good prognosis even in the case of multiple relapses.
作者
廖雄宇
邱坤银
覃丽君
何展文
Liao Xiongyu;Qiu Kunyin;Qin Lijun;He Zhanwen(Department of Pediatric Neurology,Sun Yat-Sen Memorial Hospital,Sun Yat-Sen University,Guangzhou 510120,Guangdong Province,China)
出处
《中华妇幼临床医学杂志（电子版）》
CAS
2021年第3期311-320,共10页
Chinese Journal of Obstetrics & Gynecology and Pediatrics(Electronic Edition)
基金
广东省自然科学基金(2021A1515011809)。
关键词
脱髓鞘疾病
炎症
中枢神经系统
抗体
磁共振成像
复发
糖皮质激素类
儿童
Demyelinating diseases
Inflammation
Central nervous system
Antibodies
Magnetic resonance imaging
Recurrence
Glucocorticoids
Child
作者简介
通信作者:何展文,Email:hezhanw@mail.sysu.edu.cn。
