复发的髓鞘少突胶质细胞糖蛋白抗体和N-甲基-D-天冬氨酸受体抗体双阳性自身免疫性脑炎的诊疗分析(附1例报告)  

Diagnosis and treatment analysis of recurrent autoimmune encephalitis with anti-myelin oligodendrocyte glycoprotein antibody and anti-N-methyl-D-aspartate receptor double-positive(report of one case)

作　　者：赵瑞 高赛[2] 武雷[2] 黄德晖[2] ZHAO Rui;GAO Sai;WU Lei(Department of Neurology,the First Hospital of Yulin,Yulin 719000,China)

机构地区：[1]榆林市第一医院神经内科,719000 [2]解放军总医院第一医学中心神经内科医学部

出　　处：《临床神经病学杂志》2025年第2期92-95,共4页Journal of Clinical Neurology

基　　金：中国研究型医院学会科研课题项目(Y2023FH-SJKX09)。

摘　　要：目的探讨复发的髓鞘少突胶质细胞糖蛋白(MOG)抗体和N-甲基-D-天冬氨酸受体(NMDAR)抗体双阳性自身免疫性脑炎的诊疗。方法回顾性分析1例MOG抗体和NMDAR抗体双阳性的自身免疫性脑炎患者的临床资料。结果患者男性,18岁。2017年10月出现间断头痛,发作性意识丧失肢体抽搐1次。2018年4月右眼视力下降,MRI示右侧颞枕交界区、视神经异常信号,血MOG-IgG抗体阳性,经甲基泼尼松龙治疗后好转,未加用免疫抑制剂。2018年10月患者再次出现癫痫发作,血和CSF MOG-IgG抗体阳性,CSF NMDAR-IgG抗体阳性,头颅MRI示双侧顶叶、枕叶、颞叶及胼胝体异常信号及边缘强化。诊断考虑MOG抗体相关疾病(MOGAD),给予甲基泼尼松龙及对症治疗后症状控制,复查MRI病灶信号变淡、强化消失。而后分别在2018年10月、2019年4月、2020年8月、2021年7月输注利妥昔单抗。2021年7月、2022年7月2次血MOG抗体和AQP4抗体阴性。2023年3月再次出现左侧肢体力弱、发作性意识不清伴肢体抽搐,复查血、CSF MOG抗体阳性,CSF NMDAR-IgG抗体阳性;MRI示右侧额顶叶皮质异常信号伴脑回样强化。考虑MOGAD复发,使用甲基泼尼松龙治疗后症状改善,复查头颅MRI病灶消失。序贯注射利妥昔单抗治疗后出院。2023年6月20日随访,患者未再发。结论本例自身免疫性脑炎患者MOG抗体和NMDAR抗体双阳性,临床表型考虑MOGAD。经过免疫治疗后抗体可以转阴,但停药后仍可复阳并带来相关复发。启动和停用免疫抑制治疗的时机、自身抗体的监测频率及对再次启用免疫治疗的提示和指导意义,都值得更进一步研究。To explore the experience for diagnosis and treatment of recurrent autoimmune encephalitis with antimyelin oligodendrocyte glycoprotein(MOG)antibody and anti-N-methyl-D-aspartate receptor(NMDAR)doublepositive.Methods Clinical data of a autoimmune encephalitis patient with double-positive for MOG antibody and anti-NMDAR antibody was obtained through retrospective analysis.Results Male patient,18 years old.He presented with intermittent headaches,a single episode of loss of consciousness and a convulsion in October 2017.He experienced decreased vision in the right eye in April 2018.Brain MRI showed abnormal signals in the right temporal-occipital junction and optic nerve,with the MOG-Ig G antibody positive in the serum.The symptoms improved after treatment with methylprednisolone without immunosuppressants.In October 2018,the patient had another seizure.The MOG-Ig G antibodies,both in serum and CSF,were positive,and the NMDAR-Ig G antibody was positive in the CSF.Brain MRI exhibited abnormal signals and peripheral enhancement in the bilateral parietal,occipital,temporal lobes,and the corpus callosum.A diagnosis of MOG antibody-associated disease(MOGAD)was confirmed.The Symptoms controlled after administration of methylprednisolone and symptomatic treatment.Follow-up MRI showed a reduction in lesion signal and disappearance of enhancement.Subsequently,Rituximab was administered sequentially in October 2018,April 2019,August 2020 and July 2021.MOG and AQP4 antibodies in serum were negative when tested in July 2021 and July 2022.Unfortunately,the patient again experienced weakness in the left limb and episodic confusion with limb convulsions in March 2023.The re-test of MOG antibodies in serum and CSF were positive again,and CSF NMDAR-Ig G antibody was also positive.MRI showed abnormal signals in the right frontal-parietal cortex with gyral-like enhancement.MOGAD relapse was considered,and symptoms improved after treatment with methylprednisolone.The re-examination of brain MRI showed the disappearance of the lesions.

关 键 词：髓鞘少突胶质细胞糖蛋白 利妥昔单抗 复发 

分 类 号：R744.5[医药卫生—神经病学与精神病学]