| 摘要: |
| 目的 探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病（MOGAD）的临床特点、影像学特征、治疗及转归，加深对该病的认识。方法 回顾性纳入2018年1月至2021年12月我科收治的14例MOGAD患儿，收集其一般资料、临床表现、实验室检查、影像学特征、治疗及转归等相关数据并进行总结分析。结果 14例MOGAD患儿中，男6例、女8例，起病年龄为2～12岁。10例表现为急性播散性脑炎，2例表现为视神经脊髓谱系疾病，2例表现为视神经炎；3例合并意识障碍，5例合并行为异常，6例合并惊厥发作。MRI检查提示异常信号累及广泛，主要累及脑白质、小脑、视神经、脊髓。所有患儿外周血髓鞘少突胶质细胞糖蛋白IgG均为阳性（滴度为1∶10～ 1∶1 000），3例同时合并脑脊液抗N-甲基-D-天冬氨酸受体抗体阳性。所有患儿急性期均给予大剂量甲泼尼龙联合丙种球蛋白冲击治疗，1例予血浆置换治疗。随访0.5～2.0年，9例呈单相病程，恢复较好；5例病情反复发作，3例予利妥昔单抗治疗、2例予环磷酰胺治疗后病情缓解。结论 儿童MOGAD临床表现复杂，影像学检查缺乏特异性。急性期大剂量糖皮质激素联合丙种球蛋白冲击治疗有效，部分患儿呈多相病程，应动态监测髓鞘少突胶质细胞糖蛋白抗体水平以早期发现可能复发的患儿，及时启动利妥昔单抗和环磷酰胺等二线药物治疗方案有助于改善预后。 |
| 关键词: 髓鞘少突胶质细胞糖蛋白抗体相关疾病 儿童 临床特征 影像学特征 治疗 |
| DOI：10.16781/j.CN31-2187/R.20220668 |
| 投稿时间：2022-08-15修订日期：2022-11-14 |
| 基金项目: |
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| Clinical analysis of myelin oligodendrocyte glycoprotein antibody-associated disorders in children |
| ZHANG Hui-qiong,ZHANG Yu-xin,ZENG Xiao-lu,ZHAI Qiong-xiang* |
(Department of Pediatrics, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou 510080, Guangdong, China
*Corresponding author) |
| Abstract: |
| Objective To investigate the clinical manifestations, imaging characteristics, treatment and outcome of children with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGADs), so as to improve the understanding of the disease. Methods Fourteen MOGAD children who were admitted to our department from Jan. 2018 to Dec. 2021 were retrospectively enrolled, and the general data, clinical manifestations, laboratory tests, imaging characteristics, as well as the treatment and outcome were collected and summarized for analysis. Results Of the 14 children with MOGAD, 6 were male and 8 were female, and the age of onset ranged from 2 to 12 years. Ten cases presented with acute disseminated encephalitis, 2 with neuromyelitis optica spectrum disorder, and 2 with optic neuritis; 3 cases combined with consciousness disorder, 5 with abnormal behaviors, and 6 with convulsive seizures. Magnetic resonance imaging suggested extensive involvement of abnormal signals, mainly involving the white matter, cerebellum, optic nerve and spinal cord. All children had positive peripheral blood myelin oligodendrocyte glycoprotein immunoglobulin G (titers of 1∶10 to 1∶1 000), and 3 cases were combined with positive cerebrospinal fluid anti-N-methyl-D-aspartate receptor antibodies. All patients were treated with high-dose methylprednisolone combined with gamma globulin in the acute phase, and 1 patient was treated with plasma exchange. At 0.5 to 2.0 years of follow-up, 9 cases had a monophasic course and recovered well; 5 cases had recurrent episodes and remitted after treatment with rituximab in 3 cases and cyclophosphamide in 2 cases. Conclusion The clinical manifestations of MOGAD in children are complex, and the imaging features are lack of specificity. In the acute phase, high-dose methylprednisolone combined with gamma globulin is effective, while some children have a multiphasic course of disease. Dynamic monitoring of myelin oligodendrocyte glycoprotein antibody level is helpful to early detection of patients with possible recurrence. Timely starting second-line drug treatment such as rituximab and cyclophosphamide can improve the prognosis. |
| Key words: myelin oligodendrocyte glycoprotein antibody-associated disorders child clinical features imaging features treatment |
