Chinese Journal of Contemporary Neurology and Neurosurgery ›› 2024, Vol. 24 ›› Issue (5): 340-345. doi: 10.3969/j.issn.1672-6731.2024.05.006

• Central Nervous System Immune Diseases • Previous Articles     Next Articles

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

Hong-ling QIN1, Qi-hui LI2, Yi LU3, Wei QIU2, Ya-qing SHU2,*()   

  1. 1. Encephalopathy Division, The First Affiliated Hospital of Guangxi University of Chinese Medicine, Nanning 530023, Guangxi, China
    2. Department of Neurology, The Third Affiliated Hospital, Sun Yat-Sen University, Guangzhou 510630, Guangdong, China
    3. Department of Emergency, The Eighth Affiliated Hospital, Sun Yat-Sen University, Shenzhen 518033, Guangdong, China
  • Received:2024-04-01 Online:2024-05-25 Published:2024-06-06
  • Contact: Ya-qing SHU
  • Supported by:
    National Natural Science Foundation of China(82071343); National Natural Science Foundation of China(82371354); National Natural Science Foundation of China(81701188); Guangxi Traditional Chinese Medicine Brain Disease Clinical Research Center Project(桂科AD20238028); Guangdong Basic and Applied Basic Research Fund(2021A1515010393); Guangzhou Science and Technology Key Research and Development Plan(2023B03J1347); Guangzhou Municipal School (Hospital) Joint Funding(202201020415)

抗接触蛋白相关蛋白-2抗体相关脑炎临床特征分析

秦红玲1, 李启慧2, 卢意3, 邱伟2, 舒崖清2,*()   

  1. 1. 530023 南宁, 广西中医药大学第一附属医院脑病科
    2. 510630 广州, 中山大学附属第三医院神经内科
    3. 518033 深圳, 中山大学附属第八医院急诊科
  • 通讯作者: 舒崖清
  • 作者简介:

    秦红玲与李启慧对本文有同等贡献

    QIN Hong-ling and LI Qi-hui contributed equally to the article

  • 基金资助:
    国家自然科学基金资助项目(82071343); 国家自然科学基金资助项目(82371354); 国家自然科学基金资助项目(81701188); 广西中医脑病临床研究中心项目(桂科AD20238028); 广东省基础与应用基础研究基金资助项目(2021A1515010393); 广东省广州市科技计划重点研发计划项目(2023B03J1347); 广东省广州市校(院)联合资助项目(202201020415)

Abstract:

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.

Key words: Encephalitis, Autoimmune diseases, Contactins, Autoantibodies, Immunotherapy

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。

关键词: 脑炎, 自身免疫疾病, 接触蛋白类, 自身抗体, 免疫疗法