Research Group On Statistics, Econometrics and Health (GRECS), University of Girona, Carrer de la Universitat de Girona 10, Campus de Montilivi, 17003, Girona, Spain.
CIBER of Epidemiology and Public Health (CIBERESP), Madrid, Spain.
Sci Rep. 2021 Mar 18;11(1):6207. doi: 10.1038/s41598-021-85395-z.
According to the degree of upper and lower motor neuron degeneration, motor neuron diseases (MND) can be categorized into amyotrophic lateral sclerosis (ALS), primary lateral sclerosis (PLS) or progressive muscular atrophy (PMA). Although several studies have addressed the prevalence and incidence of ALS, there is a high heterogeneity in their results. Besides this, neither concept has been previously studied in PLS or PMA. Thus, the objective of this study was to estimate the prevalence and incidence of MND, (distinguishing ALS, PLS and PMA), in the Spanish regions of Catalonia and Valencia in the period 2011-2019. Two population-based Spanish cohorts were used, one from Catalonia and the other from Valencia. Given that the samples that comprised both cohorts were not random, i.e., leading to a selection bias, we used a two-part model in which both the individual and contextual observed and unobserved confounding variables are controlled for, along with the spatial and temporal dependence. The prevalence of MND was estimated to be between 3.990 and 6.334 per 100,000 inhabitants (ALS between 3.248 and 5.120; PMA between 0.065 and 0.634; and PLS between 0.046 and 1.896), and the incidence between 1.682 and 2.165 per 100,000 person-years for MND (ALS between 1.351 and 1.754; PMA between 0.225 and 0.628; and PLS between 0.409-0.544). Results were similar in the two regions and did not differ from those previously reported for ALS, suggesting that the proposed method is robust and that neither region presents differential risk or protective factors.
根据上下运动神经元退化的程度，运动神经元病（MND）可分为肌萎缩侧索硬化症（ALS）、原发性侧索硬化症（PLS）或进行性肌肉萎缩症（PMA）。尽管有几项研究探讨了 ALS 的患病率和发病率，但结果存在很大的异质性。此外，以前从未在 PLS 或 PMA 中研究过这两个概念。因此，本研究的目的是估计 2011 年至 2019 年期间在西班牙加泰罗尼亚和瓦伦西亚地区 MND（区分 ALS、PLS 和 PMA）的患病率和发病率。本研究使用了两个基于人群的西班牙队列，一个来自加泰罗尼亚，另一个来自瓦伦西亚。由于两个队列所包含的样本并非随机，即存在选择偏差，因此我们使用了两部分模型，该模型同时控制了个体和观察到的和未观察到的混杂变量以及空间和时间依赖性。MND 的患病率估计为每 10 万人中 3.990 至 6.334 人（ALS 为 3.248 至 5.120；PMA 为 0.065 至 0.634；PLS 为 0.046 至 1.896），MND 的发病率为每 10 万人中 1.682 至 2.165 人/年（ALS 为 1.351 至 1.754；PMA 为 0.225 至 0.628；PLS 为 0.409 至 0.544）。这两个地区的结果相似，与以前报告的 ALS 结果没有差异，这表明所提出的方法是稳健的，而且这两个地区都没有不同的风险或保护因素。
