儿童MOG抗体相关疾病9例临床病例分析

Analysis of 9 Clinical Cases of Children with MOG Antibody-Associated Diseases

目的:探讨儿童髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)的临床特征。方法:收集青岛大学附属医院2018年1月至2020年12月收集的9例MOGAD儿童患者,对他们的临床症状、临床表型、实验室检查、MRI表现、治疗措施及疗效、复发及预后情况进行总结分析。结果:9例患儿平均发病年龄5.78 ±2.73岁,男女比例5:4。临床表型主要有ADEM (5/9)、NMOSD (2/9)、ON (1/9)和脑炎(1/9)。ADEM患儿症状主要有头晕(3/5)、嗜睡(3/5)、头痛(2/5)、癫痫发作(2/5),ON的患儿表现为视物模糊、眼痛。所有患儿均未出现抗NMDAR抗体重叠。9例患儿颅脑MRI中额叶、顶叶、颞叶等所有脑叶均可受累,以皮层下白质、深部灰质为主,4例患儿皮层受累,ON、NMOSD患儿中2例出现视神经(视交叉前)信号异常,另1例则为楔回、视辐射受累。1例患儿颈髓受累,1例MRI检查正常。8例患儿均采用大剂量静脉内输注丙种球蛋白(IVIG)和甲强龙冲击治疗,1例ON患儿予小剂量糖皮质激素治疗,所有患儿症状均在1周内消失。9例患儿随访时间24.17 ±10.75个月,2例出现复发,复发表型为ADEM,复发后再次用免疫冲击治疗,症状快速消失,且无后遗症状。复查2例复发患儿的血清及脑脊液MOG抗体仍为阳性。结论:MOGAD患儿无明显女性优势,以ADEM为主要表型,MRI检查以皮层下白质最多见。皮层病变与癫痫症状相关。MRI正常不能排除MOGAD。MOGAD对免疫治疗敏感。MOG抗体持续阳性对预测复发病程有提示作用。

Objective: To explore the clinical features of childhood myelin oligodendrocyte glycoprotein (MOG) antibody-related diseases. Methods: Collect 9 children with MOGAD admitted from the Affiliated Hospital of Qingdao University from January 2018 to December 2020, summarize and analyze their clinical symptoms, clinical phenotypes, laboratory tests, MRI manifestations, treatment measures and efficacy, recurrence and prognosis. Results: The average age of onset was 5.78 years, and the male to female ratio was 5:4. The main clinical phenotypes are ADEM (5/9), NMOSD (2/9), ON (1/9) and encephalitis (1/9). The symptoms of ADEM children mainly include dizziness (3/5), drowsiness (3/5), headache (2/5), and seizures (2/5). Children with ON show blurred vision and eye pain. There was no overlap of anti-NMDAR antibodies in all children. All brain lobes including frontal lobe, parietal lobe and temporal lobe can be affected in 9 children with brain MRI, mainly affected subcortical white matter and deep gray matter. Cortex is affected in four children. Two children with ON and NMOSD had abnormal signals of the optic nerve (pre-optic chiasma), and the other had wedge gyrus and optic radiation involvement. Cervical spinal cord was involved in 1 case, and MRI examination was normal in 1 case. 8 children were treated with high-dose IVIG and methylprednisolone. One child with ON was treated with low-dose glucocorticoids. All the children’s symptoms disappeared within 1 week. The 9 children were followed up for 24.17 ±10.75 months, and 2 cases had recurrence. The recurrence phenotype was ADEM. After the recurrence, immune shock therapy was used again, and the symptoms disappeared quickly without any sequelae. The serum and cerebrospinal fluid MOG antibodies of the 2 relapsed children were still positive. Conclusion: There is no obvious female advantage in children with MOGAD, ADEM is the main phenotype, and subcortical white matter is the most common in MRI examination. Cortical lesions are associated with epilepsy symptoms. A normal MRI cannot exclude MOGAD. MOGAD is sensitive to immunotherapy. Continued positive MOG antibody has a suggestive effect on predicting the course of recurrence.