多重抗神经元抗体阳性的神经系统副肿瘤综合征临床分析  

Clinical analysis of paraneoplastic neurological syndrome with co-existence of multiple anti-neuronal antibodies

作　　者：邱占东[1] 刘峥[1] 李大伟[1] 宋晓东 王静思 董会卿[1] QIU Zhandong;LIU Zheng;LI Dawei;SONG Xiaodong;WANG Jingsi;DONG Huiqing(不详;Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经内科,100053

出　　处：《中国神经免疫学和神经病学杂志》2020年第4期261-265,共5页Chinese Journal of Neuroimmunology and Neurology

基　　金：首都医科大学宣武医院院级课题(XWJL-2019015);“十三五”“精准医学研究专项”神经系统疾病专病队列研究(2017YFC0907700)。

摘　　要：目的探讨神经系统副肿瘤综合征患者出现多重抗神经元抗体的临床特征及潜在意义。方法检索2015年7月至2019年12月至宣武医院住院治疗并诊断为“神经系统副肿瘤综合征”“癌性副肿瘤综合征”“副肿瘤相关性周围神经病”“边缘性脑炎”及“自身免疫性脑炎”的患者,筛选出两种或两种以上抗神经元抗体均为阳性的患者。结果在134例符合诊断的患者中,6例存在多重抗神经元抗体,包括抗Amphiphysin抗体合并抗γ-氨基丁酸受体B型(GABABR)抗体阳性2例〔其中1例抗Hu抗体及抗谷氨酸脱羧酶(GAD65)抗体亦呈阳性〕,以及抗SOX1抗体合并抗Titin抗体阳性、抗Yo抗体合并抗富亮氨酸胶质瘤失活蛋白1(LGI1)抗体阳性、抗接触蛋白相关蛋白2(CASPR2)抗体合并抗LGI1抗体阳性和抗Hu抗体合并抗Ri抗体阳性各1例。6例患者临床表现均符合副肿瘤综合征,4例为周围神经病或肌肉接头病(其中2例合并边缘性脑炎,1例合并亚急性小脑变性),另2例单纯表现为边缘性脑炎;2例对免疫治疗有效;3例明确诊断癌症,分别为乳腺癌、胆管癌、小细胞肺癌。结论多重抗神经元抗体的存在,可导致患者的临床表现复杂多样,免疫治疗可能有效。存在某种抗神经元抗体的患者即使无相应神经综合征,该抗体亦对恶性肿瘤有提示意义。Objective To investigate the clinical characteristics and potential significance of multiple anti-neuronal antibodies in patients with paraneoplastic neurological syndrome(PNS).Methods Patients hospitalized in Xuanwu Hospital from July 2015 to December 2019,diagnosed as“paraneoplastic neurological syndrome”,“cancerous paraneoplastic syndrome”,“paraneoplastic related peripheral neuropathy”,“limbic encephalitis”,or“autoimmune encephalitis”were searched,the patients with two or more anti-neuronal antibodies were screened out.Results We screened 134 patients meeting the PNS diagnostic criteria,and found 6 patients with multiple anti-neuronal antibodies.Anti-amphiphysin and anti-GABABR antibodies were positive in 2 patients,in 1 of whom anti-Hu and anti-GAD65 antibodies were also identified.Anti-SOX1 and anti-Titin antibodies,anti-Yo and anti-LGI1 antibodies,anti-CASPR2 and LGI1 antibodies,anti-Hu and anti-Ri antibodies were positive in 1 patient,respectively.Clinical presentation of all the cases was consistent with PNS,4 cases manifested asperipheral neuropathy or neuromuscular junction disease(2 combined with limbic encephalitis,1 combined with subacute cerebellar degeneration),and the other 2 patients presented with limbic encephalitis.2 patients responded well to immunotherapy.3 cases were diagnosed as breast cancer,cholangiocarcinoma,and small cell lung cancer,respectively.Conclusions Co-existence of multiple anti-neuronal antibodies makes clinical manifestations complicated and diverse,immunotherapy may be effective.An anti-neuronal antibody found in PNS patient directs the search for occult tumor.

关 键 词：神经系统副肿瘤综合征 抗神经元抗体 边缘性脑炎 周围神经病 

分 类 号：R730.6[医药卫生—肿瘤]