Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, United States.
School of Public Health, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
Muscle Nerve. 2022 Sep;66(3):289-296. doi: 10.1002/mus.27652. Epub 2022 Jun 24.
INTRODUCTION/AIMS: We investigated the age- and sex-specific incidence and survival of Medicare beneficiaries with amyotrophic lateral sclerosis (ALS) in patients 66 to 90 years of age.
We identified all incident ALS cases within a population-based sample of Medicare beneficiaries in 2009 (total: 22 000 177 person-years at risk for ALS). We calculated age- and sex-specific incidence in 2009 according to multiple, progressively more stringent case definitions. Our most inclusive definition required one ALS code, whereas the most restrictive definition required at least one additional ALS code more than 6 months after the first code, including one from a neurologist. We identified associated imaging studies and electrodiagnostic testing and followed all cases through the end of 2014 to determine survival.
The overall incidence for our most inclusive definition was 22.84 per 100 000 person-years for men and 16.05 per 100 000 person-years for women. The overall incidence was 5.72 per 100 000 person-years for men and 3.99 per 100 000 person-years for women for our most restrictive definition. For our most inclusive definition, fewer than 39.7% of cases ever had an ALS diagnosis from a neurologist, more than 50% had an electrodiagnostic test or imaging study, and 40.1% survived less than 1 year after diagnosis, with 25.5% of these cases surviving no more than 6 months. Cases not meeting the most restrictive definition were more likely than those who did meet the restrictive definition to be older, black, or Asian.
The oldest and marginalized Medicare beneficiaries diagnosed with ALS are less likely to be included in epidemiological studies with restrictive definitions, but future studies will need to assess the accuracy of diagnosis.
简介/目的：我们研究了年龄在 66 至 90 岁之间的医疗保险受益人群中肌萎缩侧索硬化症（ALS）的年龄和性别特异性发病率和存活率。
我们在 2009 年的医疗保险受益人群中确定了所有符合条件的 ALS 病例（总计：2200 万 177 人年患有 ALS 的风险）。我们根据多种逐渐严格的病例定义计算了 2009 年的年龄和性别特异性发病率。我们最广泛的定义要求有一个 ALS 代码，而最严格的定义要求在第一个代码后至少 6 个月有一个以上的 ALS 代码，其中一个来自神经科医生。我们确定了相关的影像学研究和电诊断测试，并通过 2014 年底跟踪所有病例以确定存活情况。
对于我们最广泛的定义，男性的总体发病率为每 100000 人年 22.84 例，女性为每 100000 人年 16.05 例。对于我们最严格的定义，男性的总体发病率为每 100000 人年 5.72 例，女性为每 100000 人年 3.99 例。对于我们最广泛的定义，不到 39.7%的病例曾有过神经科医生的 ALS 诊断，超过 50%的病例有过电诊断测试或影像学研究，40.1%的病例在诊断后不到 1 年死亡，其中 25.5%的病例存活时间不超过 6 个月。不符合最严格定义的病例比符合该定义的病例更有可能年龄较大、为黑人或亚裔。
被诊断为 ALS 的最年长和边缘化的医疗保险受益人群不太可能被纳入具有严格定义的流行病学研究，但未来的研究将需要评估诊断的准确性。
