抗α-氨基-3-羟基-5-甲基-4-异 唑丙酸受体抗体脑炎3例分析  

Clinical analysis of 3 cases of autoimmune encephalitis with antibodies against theα-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor

作　　者：李欢欢 李春红 孙超 阮哲 郭荣静 高婷 唐永兰 常婷 Li Huanhuan;Li Chunhong;Sun Chao;Ruan Zhe;Guo Rongjing;Gao Ting;Tang Yonglan;Chang Ting(Department of Neurology,Tangdu Hospital,the Air Force Military Medical University,Xi′an 710038,China)

机构地区：[1]空军军医大学唐都医院神经内科,西安710038

出　　处：《中华神经科杂志》2022年第4期319-328,共10页Chinese Journal of Neurology

基　　金：陕西省重点研发计划(2021ZDLSF02-01)。

摘　　要：目的:分析抗α-氨基-3-羟基-5-甲基-4-异 唑丙酸受体(AMPAR)抗体脑炎的临床特点。 方法:回顾性总结2020年1月至2021年5月空军军医大学唐都医院诊治的3例抗AMPAR抗体脑炎患者的临床资料,对其临床表现、辅助检查、治疗及预后进行分析。结果:3例抗AMPAR抗体脑炎(年龄12~70岁)均急性起病,首发症状包括认知减退、性格改变及少见的头痛、运动障碍,其中例1肺部占位性病变经病理证实为小细胞肺癌,血清及脑脊液抗AMPAR抗体、抗Y染色体性别决定区相关高迁移率组盒蛋白1抗体阳性,激素联合肿瘤化学治疗后症状仍持续存在,无临床复发;例2责任病灶位于双侧额颞叶、半卵圆中心及侧脑室旁,同时合并低颅压综合征典型影像学特征,血清抗AMPAR抗体阳性,激素治疗后症状部分改善;例3颅内病灶位于脑桥及右侧小脑中脚,伴显著小脑萎缩,脊髓磁共振成像提示颈 2~胸 10水平脊髓病变,血清及脑脊液抗AMPAR抗体阳性,激素联合静脉滴注人免疫球蛋白治疗后症状显著改善。 结论:抗AMPAR抗体脑炎临床表现异质性高,除边缘系统外,尚可波及脑干、脊髓,出现脑萎缩;存在多重抗神经元抗体尤其是抗神经元细胞内抗原抗体时需密切监测肿瘤;免疫治疗有效,肿瘤叠加多重抗体时预后差。Objective To analyse the clinical features of encephalitis patients with antibodies against theα-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor(AMPAR).Methods Three anti-AMPAR encephalitis patients diagnosed in Tangdu Hospital,the Air Force Military Medical University between January 2020 and May 2021 were retrospectively reviewed.The clinical symptoms,supplementary examination,treatment options and outcomes with knowledge from literature were summarized in this study.Results Three patients aging from 12 to 70 years presented with symptoms ranging from cognitive impairment,personality change to headache and paralysis.The lung occupying lesion was pathologically proved to be small cell lung cancer in case 1.Antibody to AMPAR(AMPAR-ab)was positive in both blood and cerebrospinal fluid of case 1,with coexisting antibodies against sex-determining region of Y chromosome-related high mobility group box 1 in blood,and the symptoms persisted but did not recur following therapy with corticosteroids.AMPAR-ab was detected only in serum in case 2,with the lesion located in both frontal and temporal lobes,centrum semiovale and lateral ventricle,combined with classic imaging features of intracranial hypotension,and the syndrome was partially improved following treatment with corticosteroids.The lesions were located in the pons and middle cerebellar peduncle,accompanied by cerebellar atrophy in case 3.Spinal cord magnetic resonance imaging showed long hyperintense lesions involving the cervical and thoracic cord,extending from C2 to Th10 level on T2-weighted images.AMPAR-ab was positive in both serum and cerebrospinal fluid.And the symptoms improved significantly following treatment with corticosteroids and intravenous immunoglobulin.Conclusions The clinical manifestations of anti-AMPAR encephalitis are highly heterogeneous,and brainstem and spinal cord can also be involved in addition to the limbic system,accompanied by brain atrophy.Combining with concurrent antibodies,especially the intracellular antibodies,mal

关 键 词：α-氨基-3-羟基-5-甲基-4-异唑丙酸 癌 小细胞 边缘叶脑炎 

分 类 号：R742.9[医药卫生—神经病学与精神病学]