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。

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。

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 mmH₂O(1 mmH₂O＝0.098 1 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/(m²·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。

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。

To investigate clinical manifestations, imaging features, treatment and prognosis of children with myelin oligodendrocyte glycoprotein (MOG) antibody-associated inflammatory demyelination disease (IDD).

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.

①At 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 mmH₂O (1 mmH₂O=0.098 1 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/m², 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.

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.