抗接触蛋白相关蛋白2抗体相关脑炎5例临床特点分析并文献复习  

Clinical analysis and literature review of 5 cases of anti contactin associated protein-related 2 antibody associated encephalitis

作　　者：潘合跃 王晓华[1] 徐建洋[1] 王守勇[1] 史向松 李洪亮[1] Pan Heyue;Wang Xiaohua;Xu Jianyang;Wang Shouyong;Shi Xiangsong;Li Hongliang(Department of Neurology,the Third People's Hospital of Huai'an,Huai'an Psychiatric Hospital,Huai'an 223001,China)

机构地区：[1]淮安市第三人民医院,淮安市精神病医院神经内科,223001

出　　处：《神经疾病与精神卫生》2020年第9期655-659,共5页Journal of Neuroscience and Mental Health

基　　金：淮安市自然科学研究计划(HAB202045)。

摘　　要：目的 探讨抗接触蛋白相关蛋白2(CASPR2)抗体相关脑炎患者的临床特点、辅助检查、治疗及预后情况.方法 对2018年3月至2020年3月淮安市第三人民医院神经内科连续收治的5例抗CASPR2抗体相关脑炎患者临床资料进行回顾性分析并文献复习.结果 临床表现为边缘系统症状5例(癫痫发作3例,精神行为异常1例,认知障碍伴精神行为异常1例),边缘系统症状合并莫旺综合征1例,周围神经兴奋性增高合并自主神经功能障碍者2例;MRI示海马信号明显异常者2例;1例发现恶性肿瘤;失眠5例.均采用免疫治疗,随访3~14个月,中位随访时间9个月,4例症状明显改善,合并肿瘤的患者效果欠佳.结论 抗CASPR2抗体相关脑炎主要表现为边缘系统症状、外周神经兴奋性增高、自主神经功能障碍、失眠等,当出现上述表现,需考虑该疾病.通过抗体检测等早期确诊,免疫治疗及抗癫痫等对症治疗,大多预后较好,合并肿瘤效果欠佳,部分患者可复发,并可能出现新的抗体.Objective To investigate the clinical characteristics,auxiliary examination,treatment and prognosis of patients with anti contactin associated protein-related 2(CASPR2)antibody associated encephalitis.Methods The clinical data of 5 consecutive patients with anti CASPR2 antibody associated encephalitis admitted to the Department of Neurology,the Third People's Hospital of Huai'an from March 2018 to March 2020 were retrospectively analyzed and the literature was reviewed.Results The clinical manifestations were limbic system symptoms in 5 cases(3 cases of epilepsy,1 case of abnormal mental behavior,1 case of cognitive impairment with abnormal mental behavior);1 case had limbic system symptoms complicated with Morvans syndrome;2 cases had increased peripheral nerve excitability and autonomic nerve dysfunction;2 cases had obvious MRI abnormalities;1 case had malignant tumor;All the 5 cases had insomnia.All patients were recieved the immunotherapy.The patients were followed up for 3-14 months(median follow-up time was 9 months).The symptoms of 4 cases were improved obviously,except the case with tumor.Conclusions The main manifestations of anti CASPR2 antibody associated encephalitis were limbic system symptoms,increased peripheral nerve excitability,autonomic nerve dysfunction and insomnia.When the above manifestations appear,it is necessary to consider the disease and make early diagnosis through antibody detection and other detection.Immunotherapy and anti epilepsy symptomatic treatment is suggested.Most of the prognosis is good,except case combined with tumor which is not good.Some patients may relapse and may develop new antibodies.

关 键 词：自身免疫性脑炎 抗接触蛋白相关蛋白-2抗体 莫旺综合征 

分 类 号：R742.9[医药卫生—神经病学与精神病学]