Wolf Joachim, Safer Anton, Wöhrle Johannes C, Palm Frederick, Nix Wilfred A, Maschke Matthias, Grau Armin J
BMC Neurol. 2014 Oct 4;14:197. doi: 10.1186/s12883-014-0197-9.
Survival in amyotrophic lateral sclerosis varies considerably. About one third of the patients die within 12 months after first diagnosis. The early recognition of fast progression is essential for patients and neurologists to weigh up invasive therapeutic interventions. In a prospective, population-based cohort of ALS patients in Rhineland-Palatinate, Germany, we identified significant prognostic factors at time of diagnosis that allow prediction of early death within first 12 months.
Incident cases, diagnosed between October 2009 and September 2012 were enrolled and followed up at regular intervals of 3 to 6 months. Univariate analysis utilized the Log-Rank Test to identify association between candidate demographic and disease variables and one-year mortality. In a second step we investigated a multiple logistic regression model for the optimal prediction of one-year mortality rate.
In the cohort of 176 ALS patients (mean age 66.2 years; follow-up 100%) one-year mortality rate from diagnosis was 34.1%. Multivariate analysis revealed that age over 75 years, interval between symptom onset and diagnosis below 7 months, decline of body weight before diagnosis exceeding 2 BMI units and Functional Rating Score below 31 points were independent factors predicting early death.
Probability of early death within 12 months from diagnosis is predicted by advanced age, short interval between symptom onset and first diagnosis, rapid decline of body weight before diagnosis and advanced functional impairment.
ClinicalTrials.gov (NCT01955369, registered September 28, 2013).
肌萎缩侧索硬化症患者的生存期差异很大。约三分之一的患者在首次诊断后的12个月内死亡。对于患者和神经科医生而言，早期识别快速进展情况对于权衡侵入性治疗干预措施至关重要。在德国莱茵兰-普法尔茨州一项基于人群的肌萎缩侧索硬化症患者前瞻性队列研究中，我们确定了诊断时的显著预后因素，这些因素可预测患者在最初12个月内的早期死亡情况。
纳入2009年10月至2012年9月期间确诊的新发病例，并每隔3至6个月进行定期随访。单因素分析采用对数秩检验来确定候选人口统计学和疾病变量与一年死亡率之间的关联。第二步，我们研究了一个多元逻辑回归模型，以对一年死亡率进行最佳预测。
在176例肌萎缩侧索硬化症患者队列中（平均年龄66.2岁；随访率100%），从诊断开始的一年死亡率为34.1%。多因素分析显示，年龄超过75岁、症状出现至诊断的间隔时间低于7个月、诊断前体重下降超过2个体重指数单位以及功能评定评分低于31分是预测早期死亡的独立因素。
诊断后12个月内早期死亡的概率可通过高龄、症状出现至首次诊断的间隔时间短、诊断前体重快速下降以及严重的功能损害来预测。
ClinicalTrials.gov（NCT01955369，于2013年9月28日注册）。
