Keren Noa, Scott Kirsten M, Tsuda Miho, Barnwell Jessica, Knibb Jonathan A, Ellis Cathy M, Leigh P Nigel, Shaw Christopher E, Al-Chalabi Ammar
King's College London Medical School , London.
Amyotroph Lateral Scler Frontotemporal Degener. 2014 Dec;15(7-8):528-33. doi: 10.3109/21678421.2014.911326. Epub 2014 May 27.
Amyotrophic lateral sclerosis (ALS, motor neuron disease) is a neurodegenerative disorder of motor neurons leading to paralysis and eventual death by respiratory failure. Median survival is 2-3 years. Susceptibility genes, environmental triggers and disease related prognostic factors have been established, but environmental effects on survival are yet to be investigated. We analysed survival in the South-East England ALS register (SEALS register). Kaplan-Meier and Cox regression analyses were used to investigate survival in London, coastal and rural areas according to postcode at diagnosis. Results showed that there were 933 cases of ALS identified in the catchment area during the study period (1994-January 2012). Cox regression demonstrated a highly significant model for survival with significant protective variables: coastal residency, riluzole use and younger age at onset. Significantly worse survival was associated with London residency, older age as well as definite and probable El Escorial classifications. In conclusion, these findings suggest the possibility of an environmental effect on survival in ALS.
肌萎缩侧索硬化症（ALS，运动神经元病）是一种运动神经元的神经退行性疾病，可导致瘫痪，并最终因呼吸衰竭而死亡。中位生存期为2至3年。已确定了易感基因、环境触发因素和疾病相关的预后因素，但环境对生存期的影响尚待研究。我们分析了英格兰东南部ALS登记册（SEALS登记册）中的生存期情况。采用Kaplan-Meier法和Cox回归分析，根据诊断时的邮政编码，对伦敦、沿海和农村地区的生存期进行研究。结果显示，在研究期间（1994年至2012年1月），集水区共确诊933例ALS病例。Cox回归分析显示了一个高度显著的生存期模型，其中具有显著保护作用的变量为：居住在沿海地区、使用利鲁唑以及发病时年龄较轻。生存期明显较差与居住在伦敦、年龄较大以及明确和可能的埃尔埃斯科里亚尔分类有关。总之，这些发现提示环境因素可能对ALS的生存期产生影响。
