Inserm UMR1094, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, CNRS FR 3503 GEIST, Limoges, France.
Neuroepidemiology. 2017;49(1-2):64-73. doi: 10.1159/000479969. Epub 2017 Sep 5.
Amyotrophic Lateral Sclerosis (ALS) is an age-related neurodegenerative disease with unclear characteristics and prognosis in the oldest old (80 years and over). The aim of this study was to compare the oldest old and younger ALS patients in terms of clinical and socio-demographic characteristics, and prognosis.
ALS incident cases from the register of ALS in Limousin (FRALim), diagnosed between January 2000 and July 2013, were included. Descriptive and comparative analyses by age group were carried out. For time to event univariate analysis, Kaplan-Meier estimator and log rank test were used. Univariate and multivariate survival analyses were carried out with Cox's proportional hazard model.
Out of 322 patients, 50 (15.5%) were aged 80 or over ("oldest old" ALS) at the time of diagnosis. Among them, the male:female gender-ratio was 1.27, and 32.6% had a bulbar onset (not different from subjects aged less than 80 years). With increasing age, there was a worsening of the clinical state of the patients at time of diagnosis in terms of weight loss, forced vital capacity, ALSFRS-R and manual muscular testing. Access to ALS referral centres decreased with age, and the use of riluzole tended to be lower in the oldest old group. The median survival of oldest old patients appeared to be 10 months shorter than that of subjects aged less than 80 years (7.4 vs. 17.4 months).
The survival of oldest old ALS patients is particularly short. It relates to prognostic features at baseline and to an independent effect of advanced age.
肌萎缩侧索硬化症（ALS）是一种与年龄相关的神经退行性疾病，在最年长的人群（80 岁及以上）中其特征和预后尚不清楚。本研究旨在比较最年长的 ALS 患者和年轻 ALS 患者的临床和社会人口学特征以及预后。
本研究纳入了 2000 年 1 月至 2013 年 7 月期间在法国奥弗涅地区 ALS 登记处（FRALim）诊断的 ALS 发病病例。按年龄组进行描述性和比较性分析。对于生存时间的单变量分析，采用 Kaplan-Meier 估计量和对数秩检验。采用 Cox 比例风险模型进行单变量和多变量生存分析。
在 322 名患者中，有 50 名（15.5%）在诊断时年龄为 80 岁或以上（“最年长的”ALS）。其中，男女比例为 1.27，32.6%为球部起病（与年龄小于 80 岁的患者无差异）。随着年龄的增长，诊断时患者的体重减轻、用力肺活量、ALSFRS-R 和手部肌肉测试等临床状态恶化。进入 ALS 转诊中心的机会随年龄增加而减少，而在最年长的患者组中，使用利鲁唑的趋势较低。最年长的患者的中位生存期比年龄小于 80 岁的患者短 10 个月（7.4 个月比 17.4 个月）。
最年长的 ALS 患者的生存时间特别短。这与基线时的预后特征以及年龄的独立影响有关。
