Department of Neuroscience, National Institute of Health, Rome, Italy.
National Center for Disease Prevention and Health Promotion, National Institute of Health, Rome, Italy.
Brain Behav. 2021 Dec;11(12):e2378. doi: 10.1002/brb3.2378. Epub 2021 Oct 30.
Prevalence estimate of amyotrophic lateral sclerosis (ALS) ranged between 1.1/100,000 and 11.2/100,000 inhabitants with different design of the study (prospective or retrospective) and sample size. The aim of this study is to conduct for the first time an estimate of the ALS prevalence in the Latium region.
The study was performed in Latium, a region located in the center of Italy, with a population, as of January 1, 2016, of 5888.472 inhabitants. In this region, a network of 15 clinical centers (of which 4 referral ALS centers are located in Rome) and 10 local health authorities involved in the diagnosis and treatment of ALS patients has been identified. Each patient was classified according to the El Escorial revised criteria.
The prevalence study in 2016 identified 353 ALS cases (200 males). By considering population aged >=20 years, the total crude prevalence rate resulted 7.33 (CI95% 6.59-8.14) × 100,000 and 8.75 and 6.05 in males and females, respectively. Age-specific prevalence rates did not differ among males and females in the population aged less than 49 years. The difference emerged in population aged > 50 years. This type of diagnosis was recorded for 343 patients (11 missing). 68% of these patients have a definite diagnosis, 14% likely, 11% possible, and 12% defined as suspect.
The estimate of prevalence rates observed in this study is probably in line with the values reported in the literature for prospective prevalence studies.
采用不同研究设计（前瞻性或回顾性）和样本量，肌萎缩侧索硬化症（ALS）的患病率估计值在 1.1/100000 至 11.2/100000 居民之间有所不同。本研究旨在首次对拉齐奥地区 ALS 的患病率进行估计。
该研究在拉齐奥进行，拉齐奥是位于意大利中心的一个地区，截至 2016 年 1 月 1 日，人口为 5888.472 人。在该地区，确定了一个由 15 个临床中心（其中 4 个位于罗马的 ALS 转诊中心）和 10 个参与 ALS 患者诊断和治疗的地方卫生当局组成的网络。每位患者均根据 El Escorial 修订标准进行分类。
2016 年的患病率研究确定了 353 例 ALS 病例（200 名男性）。考虑到年龄>=20 岁的人口，总粗患病率为 7.33（95%CI95%6.59-8.14）×100000，男性和女性分别为 8.75 和 6.05。在年龄小于 49 岁的人群中，男性和女性的年龄特异性患病率没有差异。这种诊断在年龄大于 50 岁的人群中记录。有 343 例患者（11 例缺失）有这种诊断。这些患者中有 68%的诊断明确，14%可能，11%可能，12%为可疑。
本研究中观察到的患病率估计值可能与前瞻性患病率研究报告的数值一致。
