Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome
Long-term survival in paraneoplastic Lambert-Eaton myasthenic syndrome
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DOI：
10.1212/wnl.0000000000003794
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发表时间：
2017-04-04
期刊：
影响因子：
9.9
通讯作者：
Lang, Bethan
中科院分区：
文献类型：
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作者：
Maddison, Paul;Gozzard, Paul;Lang, Bethan
目的：确定Lambert-Eaton肌无力综合征（LEMS）和小细胞肺癌（SCLC）患者肿瘤生存率的改善是由于已知的预后危险因素还是LEMS的独立影响，可能是循环因素的结果。方法：我们对在英国诺丁汉大学医院或通过英国神经监测单位就诊的LEMS患者进行了一项前瞻性观察队列研究。同时，对活检证实为SCLC的新诊断患者进行登记，检查神经系统疾病，并随访至死亡或研究结束。结果：2005年5月至2014年11月，我们招募了31例LEMS合并SCLC患者和279例无神经系统疾病的SCLC患者。考虑到已知的SCLC生存预后因素，如疾病程度、年龄、性别、运动状态和钠值，多因素Cox回归分析显示，lems合并SCLC的存在具有独立于其他预后变量的显著生存优势（风险比1.756,95%置信区间1.137-2.709,p = 0.011）。结论：在LEMS和SCLC患者中观察到的SCLC肿瘤生存率的提高可能不仅仅是由于前置时间偏差，考虑到其他预后因素后生存优势仍然存在，并且在SCLC诊断前出现LEMS以外的副肿瘤神经综合征患者中没有观察到相同程度的生存优势。
Objective: To establish whether improved tumor survival in patients with Lambert-Eaton myasthenic syndrome (LEMS) and small-cell lung cancer (SCLC) was due to known prognostic risk factors or an effect of LEMS independently, perhaps as a result of circulating factors.Methods: We undertook a prospective observational cohort study of patients with LEMS attending Nottingham University Hospitals, UK, or via the British Neurological Surveillance Unit. In parallel, patients with a new diagnosis of biopsy-proven SCLC were enrolled, examined for neurologic illness, and followed up until death or study end.Results: Between May 2005 and November 2014, we recruited 31 patients with LEMS and SCLC and 279 patients with SCLC without neurologic illness. Allowing for known SCLC survival prognostic factors of disease extent, age, sex, performance status, and sodium values, multivariate Cox regression analysis showed that the presence of LEMSwith SCLC conferred a significant survival advantage independently of the other prognostic variables (hazard ratio 1.756, 95% confidence interval 1.137-2.709, p = 0.011).Conclusions: Improved SCLC tumor survival seen in patients with LEMS and SCLC may not be due solely to lead time bias, given that survival advantage remains after allowing for other prognostic factors and that the same degree of survival advantage is not seen in patients with paraneoplastic neurologic syndromes other than LEMS presenting before SCLC diagnosis.