Long-term survival in amyotrophic lateral sclerosis: A population-based study
Long-term survival in amyotrophic lateral sclerosis: A population-based study
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DOI：
10.1002/ana.24096
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发表时间：
2014-02-01
影响因子：
11.2
通讯作者：
Beghi, Ettore
中科院分区：
文献类型：
--
作者：
Pupillo, Elisabetta;Messina, Paolo;Beghi, Ettore
目的确定肌萎缩侧索硬化症 (ALS) 的长期生存率，并确定新诊断患者的人群延长生存的预测因素。方法对 1998 年至 2002 年意大利伦巴第大区基于人群的登记中的事件队列进行随访，直至死亡或 2013 年 2 月 28 日。年龄、性别、症状发作日期、发病部位、诊断日期和 埃斯科里亚尔诊断类别被收集。使用卡普兰-迈耶曲线评估生存率。 Cox 比例风险函数用于识别独立的预后预测因子。采用标准化死亡率（SMR）评估ALS患者的5年和10年超额死亡率。结果包括280名男性和203名女性，年龄18至93岁。 312 例 (64.6%) 存在脊柱发病 ALS。 213 例 (44.1%) 被诊断为确诊 ALS，130 例 (26.9%) 被诊断为可能 ALS，93 例 (19.3%) 可能被诊断为 ALS，47 例 (9.7%) 被诊断为疑似 ALS。诊断后1年、5年和10年的累积时间依赖性生存率分别为76.2%、23.4%和11.8%。独立预测因素包括年龄较小、可能/疑似 ALS 的诊断、脊柱发病以及诊断时 12 个月以上开始出现症状。 5 年时的 SMR 为 9.4，10 年时的 SMR 为 5.4。 10 岁时的 SMR 较高，直到 75 岁，在女性中占主导地位，此后对男性而言变得不显着。解释 ALS 的结果因表型而异。年龄较小、脊柱发病、男性和疑似 ALS 预测生存期较长。 75 岁后，ALS 男性的 10 年生存率与普通人群相似。安·尼罗尔 2014；75:287-297
ObjectiveTo determine the long-term survival in amyotrophic lateral sclerosis (ALS) and identify predictors of prolonged survival in a population-based cohort of newly diagnosed patients.MethodsAn incident cohort from a population-based registry during the years 1998 through 2002 in Lombardy, Italy was followed until death or to February 28, 2013. Age, sex, date of onset of symptoms, site of onset, date of diagnosis, and El Escorial diagnostic category were collected. Survival was assessed using Kaplan-Meier curves. Cox proportional hazards function was used to identify independent prognostic predictors. Standardized mortality ratios (SMRs) were used to assess the 5-year and 10-year excess mortality of ALS patients.ResultsIncluded were 280 men and 203 women aged 18 to 93 years. Spinal onset ALS was present in 312 cases (64.6%). Definite ALS was diagnosed in 213 cases (44.1%), probable ALS in 130 (26.9%), possible ALS in 93 (19.3%), and suspected ALS in 47 (9.7%). The cumulative time-dependent survival at 1, 5, and 10 years from diagnosis was 76.2%, 23.4%, and 11.8%, respectively. Independent predictors included younger age, the diagnosis of possible/suspected ALS, spinal onset, and symptoms having started >12 months previously at diagnosis. SMR was 9.4 at 5 years and 5.4 at 10 years. SMR at 10 years was higher until age 75 year, predominating in women, and became nonsignificant for males thereafter.InterpretationThe outcome in ALS varies with phenotype. Longer survival is predicted by younger age, spinal onset, male gender, and suspected ALS. After age 75 years, 10-year survival in men with ALS is similar to the general population. Ann Neurol 2014;75:287-297