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目的:探讨抗Y染色体性别决定区相关高迁移率组盒蛋白1（SOX1）抗体阳性神经系统副肿瘤综合征（PNS）的临床特征、诊疗及预后情况。方法:回顾性报道首都医科大学宣武manbet官网登录 神经内科2020年、2021年收治的2例抗SOX1抗体阳性PNS患者的临床资料，并检索PubMed、万方数据知识服务平台、中国知网，收集自1999年1月1日至2021年3月31日发表的关于抗SOX1抗体阳性PNS的文献报道，对已报道的13例抗SOX1抗体阳性PNS患者及本组2例患者的临床资料进行临床特征、诊疗及预后情况的汇总分析。结果:15例患者中，男12例，女3例；年龄（59.1±15.1）岁，范围为45~77岁；Lambert-Eaton肌无力综合征7例，边缘叶脑炎4例，副肿瘤性小脑变性3例，自身免疫性舞蹈病1例。15例患者均合并肿瘤，其中肺癌13例（小细胞肺癌8例）、食管隆起处小细胞癌1例、结节硬化亚型霍奇金淋巴瘤1例。14例患者在肿瘤确诊之前出现神经系统症状，肿瘤诊断延迟最长者达1年。13例患者接受治疗，其中10例接受肿瘤治疗、11例接受免疫治疗，11例治疗后神经系统症状好转，2例治疗后无好转。结论:抗SOX1抗体阳性PNS的临床表现多样，以Lambert-Eaton肌无力综合征最为多见，其次为边缘叶脑炎、副肿瘤性小脑变性；合并肿瘤中以小细胞肺癌最为多见；其总体预后差，早期诊断并及时治疗原发性肿瘤可能是改善其预后的关键。

Objective:To investigate the clinical features, diagnoses, treatments and prognoses of anti-sex-determining region of Y chromosome-related high mobility group box 1 (SOX1) antibody-positive paraneoplastic neurologic syndrome (PNS).Methods:Clinical data of 2 patients with anti-SOX1 antibody positive PNS admitted to Department of Neurology, Xuanwu Hospital, Capital Medical University in 2020 and 2021 were retrospectively analyzed. Eleven papers about 13 patients with anti-SOX1 antibody positive PNS published from January 1, 1999 to March 31, 2021 were retrieved from PubMed, Wanfang Data and CNKI. Clinical features, diagnoses, treatments and prognoses of these 15 patients were summarized.Results:Twelve males and 3 females were included, with age of (59.1±15.1) years (ranged 45-77 years); 7 patients had Lamber-Eaton myasthenic syndrome (LEMS), 4 had limbic encephalitis, 3 had paraneoplastic cerebellar degeneration, and 1 had autoimmune chorea. All 15 patients had tumors: 13 complicated with lung cancer (8 with small cell lung cancer), 1 had small cell carcinoma of esophagus, and 1 had nodular sclerosis Hodgkin lymphoma. Fourteen patients developed neurological symptoms before tumor diagnosis, and the longest delay of diagnosis was 1 year. Thirteen patients received treatments: 10 received tumor therapy and 11 received immunotherapy; 11 patients had improved neurological symptoms and 2 didn't respond to treatments.Conclusion:Clinical manifestations of anti SOX1 antibody-positive PNS are various, among which LEMS is the most common type, followed by limbic encephalitis and paraneoplastic cerebellar degeneration; small cell lung cancer is the most common complicated tumor; the overall prognosis is poor; early diagnosis and timely treatment of the primary tumors may be the key to improve the prognosis.