抗二肽基肽酶样蛋白6抗体相关脑炎二例临床和实验室研究  

Clinical and laboratory findings of patients with anti-dipeptidyl-peptidase-like protein 6 encephalitis:two cases report

作　　者：任海涛[1] 张在强[2] 周东[3] 卢强[1] 范思远[1] 费贵军[1] 彭斌[1] 崔丽英[1] 关鸿志[1] Ren Haitao;Zhang Zaiqiang;Zhou Dong;Lu Qiang;Fan Siyuan;Fei Guijun;Peng Bin;Cui Liying;Guan Hongzhi(Department of Neurology,Peking Union Medical College Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100730,China;Department of Neurology,Beijing Tiantan Hospital,Capital Medical University,Beijing 100070,China;Department of Neurology,West China Hospital of Sichuan University,Chengdu 610041,China)

机构地区：[1]中国医学科学院北京协和医院神经科,100730 [2]首都医科大学附属北京天坛医院神经病学中心,100070 [3]四川大学华西医院神经内科,成都610041

出　　处：《中华神经科杂志》2021年第5期479-482,共4页Chinese Journal of Neurology

摘　　要：目的:在病因未明的脑炎病例中筛查抗二肽基肽酶样蛋白6(DPPX)抗体,分析中国抗DPPX抗体相关脑炎患者的临床特征。方法:对2016—2019年北京协和医院《脑炎与副肿瘤综合征登记项目》登记入组的患者进行自身免疫性脑炎常规抗体谱、副肿瘤抗体谱筛查检测。对结果阴性者继续采用基于组织底物的实验(TBA)方法与基于细胞底物的实验(CBA)方法,针对DPPX抗体进行检测。收集阳性病例并总结报道其临床表现。结果:2016—2019年共筛查近15000例患者的样本,仅确诊2例抗DPPX抗体阳性患者。经TBA与CBA检测均为血清和脑脊液同时阳性,TBA荧光形式符合抗神经元表面蛋白抗体。2例均为女性,年龄分别为46岁和75岁。1例患者出现腹泻、消瘦、认知功能障碍和躁动、肌阵挛、震颤、癫痫发作等症状,另1例患者临床表现为认知障碍、躁动、记忆力减退、定向力障碍和睡眠障碍,既往曾诊断为系统性红斑狼疮和干燥综合征。结论:TBA与CBA相结合可以用于筛查鉴定抗DPPX抗体等自身免疫性脑炎相关的罕见抗体。抗DPPX抗体相关脑炎具有脑病伴腹泻、消瘦的临床表现,并可以与系统性红斑狼疮合并存在。Objective To identify anti-dipeptidyl-peptidase-like protein 6(DPPX)antibody in patients with encephalitis of unknown etiology and describe the clinical features of anti-DPPX antibody-associated encephalitis in Chinese patients.Methods For patients registered in the Peking Union Medical College Hospital Encephalitis and Paraneoplastic Syndrome Registration Project from 2016 to 2019 with negative findings in autoimmune encephalitis routine antibody profile and paraneoplastic antibody profile,but with positive tissue-based assay(TBA)results,further tests for rare antibodies,including cell-based assay(CBA)of anti-DPPX antibody,were performed.Patients positive for anti-DPPX antibody were enrolled and the clinical data were collected.Results Two patients with anti-DPPX antibody-associated encephalitis were found from 2016 to 2019 among about 15000 patients.Both were females,aged 46 and 75 years.One patient had diarrhea,cachexia,cognitive dysfunction,agitation,myoclonus,tremor,and seizures.The other had cognitive impairment,restlessness,memory loss,disorientation,and sleep disturbance.The second patient had medical history of systemic lupus erythematosus and secondary Sjögren′s syndrome.Conclusions TBA should be combined with CBA in identification of anti-DPPX antibody to confirm the diagnosis.Anti-DPPX antibody-associated encephalitis has clinical manifestations of encephalopathy with diarrhea and cachexia,and can coexist with systemic lupus erythematosus.

关 键 词：自身抗体 脑炎 红斑狼疮 系统性 二肽基肽酶样蛋白 腹泻 

分 类 号：R742.9[医药卫生—神经病学与精神病学]