抗N-甲基-D-天冬氨酸受体脑炎与髓鞘少突胶质细胞糖蛋白抗体相关疾病并存的临床特征  

Clinical Characteristics of Cases with Anti-N-methyl-D-aspartate Receptor Encephalitis and Myelin Oligodendrocyte Glycoprotein Antibody Associated Diseases

作　　者：曹丽平 马晓宇 吴远华 王婧琪[3] 舒崖清[3] 孙晓渤[3] 李小晶 邱伟[3] CAO Li-ping;MA Xiao-yu;WU Yuan-hua;WANG Jing-qi;SHU Ya-qing;SUN Xiao-bo;LI Xiao-jing;QIU Wei(The Fifth Clinical Medical College of Guangzhou University of Chinese Medicine,Guangzhou 510405,China//The First Affiliated Hospital of Guizhou University of Chinese Medicine,Guiyang 550001,China;Department of Neurology,the Third Affiliated Hospital of Sun Yat-sen University,Guangzhou 510630,China;Department of Neurology,Guangzhou Women and Children Medical Center,Guangzhou 510000,China)

机构地区：[1]广州中医药大学第五临床医学院,广东广州510405 [2]贵州中医药大学第一附属医院脑病内科,贵州贵阳550001 [3]中山大学附属第三医院神经内科,广东广州510630 [4]广州市妇女儿童医疗中心神经内科,广东广州510000

出　　处：《中山大学学报（医学科学版）》2020年第6期858-866,共9页Journal of Sun Yat-Sen University:Medical Sciences

基　　金：国家自然科学基金(81971140,81771300);广东省自然科学基金(2020A1515010053);白求恩基金会“共享阳光-重大疾病临床科研合作项目”(2020009)。

摘　　要：【目的】探讨抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎与髓鞘少突胶质细胞糖蛋白(MOG)抗体相关疾病并存患者的临床特征。【方法】回顾性分析2015年2月至2019年8月广东省中山大学附属第三医院神经内科和广州市妇女儿童医疗中心神经内科共同收治的36例抗NMDAR脑炎患者,其中17例为与MOG抗体相关疾病共存的并存组,19例为单纯抗NMDAR脑炎的对照组。分析总结两组患者的首发症状、影像学特征、实验室检查、治疗及预后情况。【结果】与对照组比较,并存组患者在发病性别方面男性多于女性,少合并卵巢畸胎瘤,部分患者首发症状表现为抗NMDAR脑炎不常见的脱髓鞘症状,脑脊液抗NMDAR抗体滴度波动在(1∶1~1∶100),血清MOG抗体滴度波动在(1∶25~1∶1280),病灶影像学不仅表现为累及皮质及皮质下,还表现为皮质下白质病灶或累及脊髓。从治疗及预后分析,两组患者经免疫治疗后症状均有改善,经3~50个月随访,并存组3例有复发,对照组1例有复发,均再次对免疫治疗反应良好。【结论】临床上抗NMDAR脑炎与MOG抗体相关疾病并存的患者在发病性别上以男性居多,成人多于儿童,合并肿瘤的情况少见,对免疫治疗反应良好,推测此类患者在免疫致病机制上具有相对的特异性。【Objective】To investigate the clinical characteristics of Anti-N-methyl-D-aspartate receptor encephalitis coexisting with myelin oligodendroprotein antibody(MOG).【Methods】Retrospective analysis was performed on 36 patients with anti-NMDAR encephalitis who were admitted to the Department of Neurology of the Third Affiliated Hospital of Sun Yat-sen University and the Department of Neurology of Guangzhou Women and Children Medical Center from February 2015 to August 2019.Among them,17 patients were in the coexisting group with MOG antibody related diseases and 19 patients were in the control group with simple anti-NMDAR encephalitis.The first symptoms,imaging features,laboratory examination,treatment and prognosis of the two groups of patients were analyzed and summarized.【Results】Compared with the control group,there were more male than female patients in the coexisting group,so fewer of them were complicated with ovarian teratoma.In some patients,the first symptoms were atypical demyelination of anti-NMDAR encephalitis.The titer of cerebrospinal fluid anti-NMDAR antibody(1∶1~1∶100)and serum MOG antibody(1∶25~1∶1280)fluctuated.The imaging findings showed not only cortical and subcortical involvement but also subcortical white matter involvement or spinal cord involvement.From the analysis of treatment and prognosis,the symptoms of patients in both groups were improved after immunotherapy.After 3-50 months of follow-up,3 patients in the coexisting group and 1 patient in the control group had recurrence,and all of them responded well to immunotherapy again.【Conclusions】Clinically,the incidence of Anti-NMDAR encephalitis and MOG antibody related diseases mostly occur in male patients,with more adults than children.Combined tumor is rare,and the patients have a good response to immunotherapy.It is speculated that such patients have a relatively specific immune pathogenesis.

关 键 词：抗NMDAR脑炎 MOG抗体相关疾病 临床特征分析 

分 类 号：R74[医药卫生—神经病学与精神病学]