Bujanov Moscow City Clinical Hospital, Moscow, Russian Federation.
Moscow Research and Clinical Center for Neuropsychiatry of the Healthcare Department, Moscow, Russian Federation.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Aug;21(5-6):410-415. doi: 10.1080/21678421.2020.1752252. Epub 2020 Jun 1.
: 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. : 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. : 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) ( = 0.02). Family history of ALS was present in two cases. : 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.
通过调查多种病例来源，估算莫斯科地区肌萎缩侧索硬化症（ALS）的发病率。先前俄罗斯的研究中发病率范围为 0.3 至 0.7/10 万/年。
在莫斯科南部地区（人口为 176 万，2015 年）进行了一项 ALS 发病率的回顾性研究。调查了多个数据源。一位 ALS 专家对 2015 年的所有病历进行了检查，仅接受符合修订版埃尔埃斯科里亚尔标准的明确或可能 ALS 患者。计算了粗发病率和标准化发病率，后者根据俄罗斯和欧洲人口进行了调整。
共发现 22 例患者，粗发病率为 1.25/10 万/年（95%CI：0.78-1.89），标准化发病率为 1.22（俄罗斯）和 1.28（欧洲）。该样本包括 11 名男性和 11 名女性，年龄 36-73 岁（平均 56 岁）。5 例（23%）患者为球部起病 ALS。平均诊断延迟时间为 12.8±9.2 个月（男性为 8.5±4.8 个月，女性为 17.2±10.6 个月）（P=0.02）。有两例患者有 ALS 家族史。
俄罗斯联邦的 ALS 发病率高于先前报道，但低于其他国家。较差的诊断发现率，特别是在女性中，以及俄罗斯人口预期寿命较短，可能是造成这种情况的原因。
