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

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-ofdeath 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 ageadjusted 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.

Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016

“…The lowest prevalence in the South and West Census regions are most likely related to the population diversity in these states. These findings are also consistent with previous ALS mortality analyses showing that states in more northern latitudes have higher age-adjusted mortality rates than states in southern latitudes (25,26).…”

Mortality of 196,826 Men and Women Working in U.S.-Based Petrochemical and Refinery Operations

“…Examination of the death certificates showed that 81% (99/122) of the causes of death contained the terms ALS, bulbar ALS, progressive ALS or Lou Gehrig's disease. Non-ALS disorders made-up 13% of the death certificate causes including progressive supranuclear palsy ( n = 13), which is not considered a subtype of MND, 32 , 33 bulgar palsy ( n = 1), progressive muscular atrophy ( n = 1) and primary lateral sclerosis ( n = 1). The remaining seven certificates indicated the nonspecific terms of motor neuron disease, of which two specified familial MND and Kennedy's syndrome MND, which is a hereditary disorder.…”

“…Examination of the death certificates showed that 26 of the 30 certificates (87%) indicated ALS as the cause of death and the remaining four stated MND of progressive supranuclear palsy, which is not ALS and is not considered a subtype of MND. 32 , 33 Military service was indicated on one of the thirty death certificates.…”

“…Examination of the death certificates showed that seven of the eleven causes of death indicated ALS and four indicated progressive supranuclear palsy which is not a subtype of MND. 32 , 33 …”

“…This proportion in the MND class of diseases is consistent with the literature. 32 About 2% ( n = 3) of DCs indicated other MNDs such as bulbar palsy, primary lateral sclerosis and progressive muscular atrophy. For 11% ( n = 17) of the DCs, progressive supranuclear palsy ( n = 17), also known as Steele-Richardson Oslzewski syndrome 39 was indicated.…”