抗接触蛋白相关蛋白-2抗体相关脑炎临床特征分析  

Clinical analysis and literature review of anti-contactin-associated protein 2 antibody-associated encephalitis

作　　者：秦红玲[1] 李启慧 卢意 邱伟[2] 舒崖清[2] Hong-ling QIN;Qi-hui LI;Yi LU;Wei QIU;Ya-qing SHU(Encephalopathy Division,The First Affiliated Hospital of Guangxi University of Chinese Medicine,Nanning 530023,Guangxi,China;Department of Neurology,The Third Affiliated Hospital,Sun Yat-Sen University,Guangzhou 510630,Guangdong,China;Department of Emergency,The Eighth Affiliated Hospital,Sun Yat-Sen University,Shenzhen 518033,Guangdong,China)

机构地区：[1]广西中医药大学第一附属医院脑病科,南宁530023 [2]中山大学附属第三医院神经内科,广州510630 [3]中山大学附属第八医院急诊科,深圳518033

出　　处：《中国现代神经疾病杂志》2024年第5期340-345,共6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:82071343);国家自然科学基金资助项目(项目编号:82371354);国家自然科学基金资助项目(项目编号:81701188);广东省基础与应用基础研究基金资助项目(项目编号:2021A1515010393);广西中医脑病临床研究中心项目(项目编号:桂科AD20238028);广东省广州市科技计划重点研发计划项目(项目编号:2023B03J1347);广东省广州市校(院)联合资助项目(项目编号:202201020415)。

摘　　要：目的 总结抗接触蛋白相关蛋白-2(CASPR2)抗体相关脑炎的临床特征。方法与结果收集2017年8月至2022年8月中山大学附属第三医院诊断与治疗的13例抗CASPR2抗体相关脑炎患者。首发表现:9例表现为边缘系统症状,2例表现为周围神经症状(均为肢体疼痛),1例为小脑性共济失调,1例肢体乏力。抗CASPR2抗体检测:13例患者均进行血清抗CASPR2抗体检测,均呈阳性;10例患者接受脑脊液抗CASPR2抗体检测,5例呈阳性;有3例患者合并其他自身抗体阳性。头部MRI检查:13例患者中2例表现为单侧或双侧颞叶内侧T2-FLAIR成像高信号;2例存在脑微出血灶。治疗:2例仅单纯采用激素治疗,4例仅静脉注射免疫球蛋白,5例则先后接受激素+静脉注射免疫球蛋白治疗,1例仅接受利妥昔单抗治疗,1例先后接受静脉注射免疫球蛋白+激素+蛋白A免疫吸附+利妥昔单抗。随访:进行1~5年的随访,2例失访;1例患者自行停药后复发,经治疗后改良Rankin量表(mRS)评分为零;余10例患者中1例症状未见改善,1例部分症状存在不能正常生活,6例正常生活,2例死亡。结论 抗CASPR2抗体相关脑炎临床特征复杂,血清抗体检测对于明确诊断至关重要。Objective:To summarize the clinical characteristics of patients with anti-contactin-associated protein 2(CASPR2)antibody-associated encephalitis.Methods and Results:Total 13 patients diagnosed and treated for anti-CASPR2 antibody-associated encephalitis at The Third Affiliated Hospital,Sun Yat-Sen University from August 2017 to August 2022 were collected.Initial manifestations:9 patients exhibited limbic system symptoms,2 patients presented with peripheral nerve symptoms(both with limb pain),one patient had cerebellar ataxia,and one patient had limb weakness.Anti-CASPR2 antibody detection:all the 13 patients underwent serum anti-CASPR2 antibody testing,which was positive in all cases;10 patients underwent cerebrospinal fluid(CSF)anti-CASPR2 antibody testing,with 5 cases testing positive;3 patients had other concomitant positive autoimmune antibodies.Head MRI examination:among the 13 patients,2 patients head T2-FLAIR showed hyperintensity in the medial temporal lobes either unilaterally or bilaterally;2 patients had cerebral microbleeds.Treatment:2 patients were treated only with steroids,4 patients received only intravenous immunoglobulin(IVIg),5 patients were treated with steroids and IVIg,one patient received rituximab only,and one patient received a combination of IVIg,steroids,protein A immunoadsorption and rituximab.Follow-up:follow-up ranged from 1-5 years,with 2 patients lost to follow-up;one patient relapsed after self-discontinuation of medication and achieved a modified Rankin Scale(mRS)score of 0 after treatment;of the remaining 10 patients,one patient showed no symptom improvement,one patient had some symptoms preventing normal life,6 patients had normal life,2 patients died.Conclusions:Anti-CASPR2 antibody-associated encephalitis has complex clinical characteristics,and serum antibody testing is crucial for diagnosis.

关 键 词：脑炎 自身免疫疾病 接触蛋白类 自身抗体 免疫疗法 

分 类 号：R742.9[医药卫生—神经病学与精神病学]