抗接触蛋白相关蛋白-2抗体相关脑炎临床特征分析

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中文摘要：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.

外文标题：Clinical analysis and literature review of anti-contactin-associated protein 2 antibody-associated encephalitis

外文摘要：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.

外文关键词：

EncephalitisAutoimmune diseasesContactinsAutoantibodiesImmunotherapy

作者：

秦红玲、李启慧、卢意、邱伟、舒崖清

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作者单位：

530023 南宁,广西中医药大学第一附属医院脑病科

510630 广州,中山大学附属第三医院神经内科

518033 深圳,中山大学附属第八医院急诊科

关键词：

脑炎自身免疫疾病接触蛋白类自身抗体免疫疗法

基金：

国家自然科学基金资助项目国家自然科学基金资助项目国家自然科学基金资助项目广西中医脑病临床研究中心项目广东省基础与应用基础研究基金资助项目广东省广州市科技计划重点研发计划项目广东省广州市校(院)联合资助项目

项目编号：

820713438237135481701188桂科AD202380282021A15150103932023B03J1347202201020415

出版年：

2024

DOI：

10.3969/j.issn.1672-6731.2024.05.006

中国现代神经疾病杂志

中国医师协会,天津市科学技术协会,天津市神经科学学会

中国现代神经疾病杂志

CSTPCD北大核心

影响因子：0.968

ISSN：1672-6731

年,卷(期)：2024.24(5)

参考文献量8