Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018;51(1-2):96-103.
doi: 10.1159/000488891. Epub 2018 Jul 10.

Amyotrophic Lateral Sclerosis Mortality in the United States, 2011-2014

Theodore C Larson  1 , Wendy Kaye  2 , Paul Mehta  1 , D Kevin Horton  1
Affiliations

Amyotrophic Lateral Sclerosis Mortality in the United States, 2011-2014

Theodore C Larson et al. Neuroepidemiology. 2018.

Abstract

Background: The International Classification of Disease, 10th Revision (ICD-10) did not include a code specific for Amyotrophic lateral sclerosis (ALS) until 2017. Instead, code G12.2 included both ALS and other motor neuron diseases (MND). Our objective was to determine US mortality rates for ALS exclusively by excluding other MND and progressive supranuclear palsy.

Methods: All mortality data coded as G12.2 under the pre-2017 rubric were obtained for 2011-2014. Deaths without ALS listed in one of the un-coded cause-of-death fields were excluded. ALS death rates per 100,000 persons were age-adjusted to the 2000 US standard population using the direct method.

Results: The proportion of excluded records coded G12.2 but not ALS was 0.21, resulting in 24,328 ALS deaths. The overall age-adjusted mortality rate was 1.70 (95% CI 1.68-1.72). The rate among males was 2.09 (95% CI 2.05-2.12) and females was 1.37 (95% CI 1.35-1.40). The overall rate among whites was 1.84, blacks 1.03, and other races 0.70. For both sexes and all races, the rate increased with age and peaked among 75-79 year-olds. Rates tended to be greater in states at higher latitudes.

Conclusions: Previous reports of ALS mortality in the United States showed similar age, sex, and race distributions but with greater age-adjusted mortality rates due to the inclusion of other diseases in the case definition. When using ICD-10 data collected prior to 2017, additional review of multiple-cause of death data is required for the accurate estimation of ALS deaths.

Keywords: Amyotrophic lateral sclerosis; Mortality; Motor neuron disease.

PubMed Disclaimer

Figures

Fig. 1.
Fig. 1.
ALS-associated average annual death rates in the United States by age group and sex, 2011–2014. ALS, amyotrophic lateral sclerosis.
Fig. 2.
Fig. 2.
ALS-associated average annual death rates in the United States by age group and race, 2011–2014. ALS, amyotrophic lateral sclerosis.
Fig. 3.
Fig. 3.
ALS-associated average annual death rates in the United States by age group and Hispanic origin, 2011–2014. ALS, amyotrophic lateral sclerosis.
Fig. 4.
Fig. 4.
Geographic distribution of average annual age-adjusted ALS mortality rates per 100,000 population in the United States, 2011–2014. The rate for Washington DC is in the 1.1–1.5 category.
See this image and copyright information in PMC

References

    1. Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC. Amyotrophic lateral sclerosis. Lancet. 2011;377:942–955. - PubMed
    1. Mehta P, Kaye W, Raymond J, Wu R, Larson T, Punjani R, Heller D, Cohen J, Peters T, Muravov O, Horton K. Prevalence of Amyotrophic Lateral Sclerosis - United States, 2014. MMWR Morb Mortal Wkly Rep. 2018;67:216–218. - PMC - PubMed
    1. Galvin M, Corr B, Madden C, Mays I, McQuillan R, Timonen V, Staines A, Hardiman O. Caregiving in - a mixed methods approach to the study of Burden. BMC Palliat Care. 2016;15:81. - PMC - PubMed
    1. Dharmadasa T, Henderson RD, Talman PS, Macdonell RA, Mathers S, Schultz DW, Needham M, Zoing M, Vucic S, Kiernan MC. Motor neurone disease: progress and challenges. Med J Aust. 2017;206:357–362. - PubMed
    1. Mehta P, Kaye W, Bryan L, Larson T, Copeland T, Wu J, Muravov O, Horton K. Prevalence of Amyotrophic Lateral Sclerosis - United States, 2012–2013. MMWR Surveill Summ. 2016;65:1–12. - PubMed