Neurophysiological index is associated with the survival of patients with amyotrophic lateral sclerosis

Objective: To investigate the association between Neurophysiological Index (NI) and the survival of patients with Amyotrophic Lateral Sclerosis (ALS).Methods: Patients diagnosed with ALS in the Department of Neurology, West China Hospital, Sichuan University from May 2015 to May 2017 were enrolled in this study, and then were followed up every 3 months until 31st May 2018. According to the mean NI values, the participants were categorized into high NI and low NI groups. Differences between groups were compared with parametric or nonparametric test, whichever was more appropriate. Kaplan-Meier method and Cox regression model were used to calculate the survival analysis.Results: One hundred and ninety one sporadic ALS patients including 78 female and 113 male were included in this study. Among them, 5 (2.6%) patients were lost to follow-up, 79 patients were still alive while 102 patients died at the last follow up. The median survival time from symptom onset to death was 33 months, as estimated by Kaplan-Meier analysis. ALS patients with lower NI exhibited to have shorter median survival time compared to the patients with high NI. Through multivariate Cox regression analysis, NI, the revised ALS functional rating scale (ALSFRS-R), diagnostic delay and age at disease onset were found to be associated with the survival of ALS patients.Conclusion: Our findings indicate that NI provides a single number that seems to associate with the survival of ALS.Significance: NI is readily available and reproducible, and it could be utilized as a potential biomarker for survival in further multicenter clinical trials in ALS. (C) 2019 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.

目的：探讨神经生理学指标（NI）与肌萎缩侧索硬化（ALS）患者生存率之间的关联。 方法：本研究纳入2015年5月至2017年5月在四川大学华西医院神经内科确诊为ALS的患者，之后每3个月随访一次，直至2018年5月31日。根据NI平均值将参与者分为高NI组和低NI组。采用更合适的参数检验或非参数检验比较组间差异。采用Kaplan - Meier法和Cox回归模型进行生存分析。 结果：本研究纳入191例散发性ALS患者，其中女性78例，男性113例。其中5例（2.6%）患者失访，最后一次随访时79例患者仍存活，102例患者死亡。通过Kaplan - Meier分析估计，从症状发作到死亡的中位生存时间为33个月。与高NI患者相比，低NI的ALS患者中位生存时间更短。通过多变量Cox回归分析，发现NI、修订的ALS功能评定量表（ALSFRS - R）、诊断延迟和发病年龄与ALS患者的生存率相关。 结论：我们的研究结果表明，NI提供了一个似乎与ALS患者生存率相关的单一数值。 意义：NI易于获取且可重复，在ALS的进一步多中心临床试验中，它可作为一种潜在的生存生物标志物。（C）2019国际临床神经生理学联合会。由爱思唯尔出版集团出版。保留所有权利。