Suh Mi Ri, Choi Won Ah, Choi Young-Chul, Lee Jang Woo, Hong Jung Hwa, Park Jihyun, Kang Seong-Woong
Department of Medicine, The Graduate School, Yonsei University, Seoul, Korea.
Department of Rehabilitation Medicine and Rehabilitation Institute of Neuromuscular Disease, Yonsei University College of Medicine, Seoul, Korea.
Ann Rehabil Med. 2017 Dec;41(6):1055-1064. doi: 10.5535/arm.2017.41.6.1055. Epub 2017 Dec 28.
To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.
Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was assessed using Kaplan-Meier curves and multivariate analyses of the risk of death were performed using the Cox proportional hazards model.
Among 212 suspicious subjects, definite ALS was diagnosed in 182 subjects. The survival rate at 3 and 5 years from onset was 61.5% and 40.1%, respectively, and the survival rate at 3 and 5 years post-diagnosis was 49.5% and 24.2%, respectively. Further, 134 patients (134/182, 73.6%) were initiated on NIV, and among them, 90 patients (90/182, 49.5%) underwent tracheostomy. Male gender and onset age of ≥65 years were independent predictors of adverse survival.
The analysis of long term survival in ALS showed excellent outcomes considering the overall poor prognosis of this disease.
报告肌萎缩侧索硬化症(ALS)的最新长期预后情况,并分析预后的预测因素。
对2005年1月至2009年12月在单一机构被诊断为ALS的患者进行随访,直至死亡或截至2014年12月。收集有关年龄、性别、发病日期、诊断日期、发病时是否存在延髓症状、开始无创通气(NIV)的日期以及气管切开术的日期的数据。使用Kaplan-Meier曲线评估生存率,并使用Cox比例风险模型对死亡风险进行多变量分析。
在212名疑似患者中,182名被确诊为ALS。发病后3年和5年的生存率分别为61.5%和40.1%,诊断后3年和5年的生存率分别为49.5%和24.2%。此外,134例患者(134/182,73.6%)开始接受NIV,其中90例患者(90/182,49.5%)接受了气管切开术。男性和发病年龄≥65岁是生存不良的独立预测因素。
考虑到ALS总体预后较差,对其长期生存的分析显示出良好的结果。