Objectives: To estimate the incidence of amyotrophic lateral sclerosis (ALS) in Moscow by investigating multiple sources of cases. Incidence rates from previous Russian studies ranged from 0.3 to 0.7 per 100,000/year. Methods: A retrospective study of the incidence of ALS was performed in the South district of Moscow (population, 1,760,000, 2015). Several data sources were investigated. The medical records of the calendar year 2015 were examined by an ALS expert who accepted only patients with definite or probable ALS according to the revised El Escorial criteria. Crude and standardized incidence rates were calculated, the latter adjusted to the Russian and the European population. Results: Twenty-two patients were found, accounting for a crude incidence of 1.25/100,000/year (95% CI 0.78–1.89) and a standardized incidence of 1.22 (Russia) and 1.28 (Europe). The sample included 11 men and 11 women aged 36–73 years (mean 56). Five patients (23%) had bulbar onset ALS. The mean diagnostic delay was 12.8 ± 9.2 months (8.5 ± 4.8 in men and 17.2 ± 10.6 in women) (p = 0.02). Family history of ALS was present in two cases. Conclusion: The incidence of ALS in the Russian Federation is higher than previously reported but lower than in other countries. Poor diagnostic ascertainment, particularly in women, and the shorter life expectancy of the Russian population are possible explanations.

目的：通过调查多个病例来源，估计莫斯科肌萎缩侧索硬化 (ALS) 的发病率。先前俄罗斯研究的发病率为每 100,000 人/年 0.3 至 0.7。方法：在莫斯科南区（人口，1,760,000，2015）进行了 ALS 发病率的回顾性研究。调查了几个数据源。2015 日历年的医疗记录由一名 ALS 专家检查，该专家根据修订后的 El Escorial 标准仅接受确诊或疑似 ALS 患者。计算了粗略和标准化的发病率，后者根据俄罗斯和欧洲人口进行了调整。结果：发现了 22 名患者，粗发病率为 1.25/100,000/年（95% CI 0.78–1.89），标准化发病率为 1.22（俄罗斯）和 1.28（欧洲）。样本包括 11 名男性和 11 名女性，年龄在 36-73 岁之间（平均 56 岁）。5 名患者 (23%) 患有延髓起病的 ALS。平均诊断延迟为 12.8 ± 9.2 个月（男性为 8.5 ± 4.8，女性为 17.2 ± 10.6）（p  = 0.02）。两例有 ALS 家族史。结论：俄罗斯联邦的 ALS 发病率高于先前报告的，但低于其他国家。诊断确定性差，尤其是女性，以及俄罗斯人口的预期寿命较短是可能的解释。