抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病合并抗体重叠综合征临床特点分析  

Clinical characteristics of patients with anti-myelin oligodendrocyte glycoprotein-IgG associated disorders complicated with antibody overlap syndrome

作　　者：秦晶晶 王梦涵[2] 周晨光[1] 杜娟 李明月 王建平[1] QIN Jing-jing;WANG Meng-han;ZHOU Chen-guang;DU Juan;LI Ming-yue;WANG Jian-ping(Department of Neurology,The Fifth Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,He'nan,China;Department of Neurology,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,He'nan,China)

机构地区：[1]郑州大学第五附属医院神经内科,450052 [2]郑州大学第一附属医院神经内科,450052

出　　处：《中国现代神经疾病杂志》2022年第6期519-526,共8页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：国家自然科学基金资助项目(项目编号:81771247)。

摘　　要：目的 总结抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(MOGAD)合并抗体重叠综合征的临床特点。方法与结果 纳入2016年4月至2021年4月郑州大学第一附属医院和郑州大学第五附属医院收治的10例MOGAD合并抗体重叠综合征患者,其中7例合并N-甲基-D-天冬氨酸受体(NMDAR)-IgG阳性、2例合并接触蛋白相关蛋白-2(CASPR2)-IgG阳性、1例合并胶质纤维酸性蛋白(GFAP)-IgG;男女比例为1∶2.33,平均发病年龄20.70岁;5例有前驱感染史;首发症状以头痛、发热最常见;入院时平均扩展残疾状态量表(EDSS)评分为2.40分。5例颅内压升高,8例脑脊液白细胞计数增加,无一例合并肿瘤。9例头部MRI异常,可同时累及幕上和幕下,以皮质下白质、脑干、丘脑、小脑常见;增强扫描可见点片状、结节状强化,部分伴脑膜线样强化;6例脊柱MRI异常,尤以颈胸髓常见,表现为长节段脊髓损伤;2例累及视神经。经免疫调节治疗后部分或完全缓解,出院时EDSS评分下降1~3.50分,平均随访12.80个月,3例复发,再次经免疫调节治疗反应良好。结论 MOGAD合并抗体重叠综合征可同时或先后检出髓鞘少突胶质细胞糖蛋白(MOG)-IgG与其他类型自身免疫抗体,但可仅表现为其中一种疾病症状,对免疫调节治疗反应良好。Objective glycoprotein-IgG associated disorders(MOGAD) complicated with antibody overlap syndrome.Methods and Results Affiliated Hospital of Zhengzhou University and the Fifth Affiliated Hospital of Zhengzhou University from April 2016 to April 2021 were included, including 7 patients with N-methyl-D-aspartate receptor(NMDAR)-IgG positive, 2 patients with contactin-associated protein 2(CASPR2)-IgG positive and one patient with glial fibrillary acidic protein(GFAP)-IgG. The ratio of male to female was 1∶2.33, and the average age of onset was 20.70 years old. Five cases had a history of precursor infection. Headache and fever were the most common initial symptoms. The average Expanded Disability Status Scale(EDSS) score at admission was2.40. Intracranial pressure(ICP) increased in 5 cases, white blood cell count in cerebrospinal fluid(CSF)increased in 8 cases, and there was no tumor. Head MRI abnormalities in 9 cases, involving both supratentorial and infratentorial, were common in subcortical white matter, brainstem, thalamus and cerebellum. Enhancement scan showed patchy and nodular enhancement, with some linear enhancement of meninges. Spine MRI abnormalities were found in 6 cases, especially in cervical and thoracic spinal cord,which showed long segment spinal cord injury. Optic nerve was involved in 2 cases. Partial or complete remission was achieved after immunomodulatory treatment. The EDSS score decreased by 1-3.50 at discharge. The average follow-up was 12.80 months. Three cases recurred and responded well after immunomodulatory treatment again.Conclusions can detect MOG-IgG and other types of autoimmune antibodies at the same time or successively, but it can only show the symptoms of one of the diseases. It has a good response to immunomodulatory treatment, and immunomodulatory treatment is still effective after recurrence.

关 键 词：髓鞘少突胶质细胞糖蛋白 抗体 N-甲基天冬氨酸 神经胶质原纤维酸性蛋白质 接触蛋白类 

分 类 号：R744.5[医药卫生—神经病学与精神病学]