肌萎缩侧索硬化症患者生存期的影响因素分析

Factors affecting survival of patients with amyotrophic lateral sclerosis

目的探讨肌萎缩侧索硬化症(ALS)患者的临床特点及其与生存期的关系。方法分析2010年3月至2015年12月就诊于西安交通大学第一附属医院神经内科且符合El Escorial revised诊断标准的ALS患者101例。首诊时记录患者起病年龄、体质指数(BMI)、起病部位、诊断延迟时间、Ellis上运动神经元评分、ALSFRS-r、疾病进展率、从起病到第二部位受累经历时间以及诊断级别。每3月门诊或电话随访1次。将上述临床特点进行单因素及多因素生存分析。结果 (1)单因素分析显示:起病年龄(HR1.556,95%CI 1.005~2.409,P=0.047)、诊断延迟时间(HR0.227,95%CI 0.134~0.383,P<0.001)、疾病进展率(HR2.300,95%CI 1.462~3.617,P<0.001)、从起病到第二部位受累经历时间(HR0.593,95%CI 0.377~0.934,P=0.024)、诊断级别(HR1.709,95%CI 1.065~2.743,P=0.026)对生存时间均有影响。(2)多因素分析显示:诊断延迟时间(HR0.948,95%CI 0.912~0.985,P=0.006)、疾病进展率(HR2.146,95%CI 1.538~2.995,P<0.001)对生存时间有影响。结论 ALS患者确诊时的诊断延迟时间及疾病进展率是影响生存期的独立预测因素。

Objective To investigate the relationship between clinical features and survival time in amyotrophic lateral sclerosis(ALS)patients.Methods For this study we enrolled 101 ALS patients who visited Department of Neurology,the First Affiliated Hospital of Xi'an Jiaotong University,from March 2010 to December 2015 and met the revised El Escorial Criteria.We documented the clinical features known up to the time of diagnosis,such as age of onset,BMI,body part of onset,diagnostic delay time,Ellis score,ALSFRS-r,ΔALSFRS-r,time from onset to second part involved and categories of diagnostic certainty.All the patients were regularly followed up every three months clinically or by phone.The clinical features mentioned above were analyzed for survival probability using the univariate and multivariate survival analyses.Results①Univariate log-rank test results:age at onset(HR 1.556,95%CI 1.005-2.409,P=0.047),diagnostic delay time(HR 0.227,95%CI 0.134-0.383,P<0.001),ΔALSFRS-r(HR 2.300,95%CI 1.462-3.617,P<0.001),time from onset to second part involved(HR 0.593,95%CI 0.377-0.934,P=0.024),and categories of diagnostic certainty(HR 1.709,95%CI 1.065-2.743,P=0.026)were correlated to the survival time.②Multivariate Cox regression test results:diagnostic delay time(HR 0.948,95%CI 0.912-0.985,P=0.006)andΔALSFRS-r(HR 2.146,95%CI 1.538-2.995,P<0.001)were correlated to the survival time.Conclusion Diagnostic delay time andΔALSFRS-r are independent factors that affect the survival time in ALS.