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. 2024 Dec 12;272(1):44.
doi: 10.1007/s00415-024-12743-0.

Incidence and mortality of ALS: a 42-year population-based nationwide study

Lotte Sahin Levison  1 , Jakob Udby Blicher  2 , Henning Andersen  2
Affiliations

Incidence and mortality of ALS: a 42-year population-based nationwide study

Lotte Sahin Levison et al. J Neurol. .

Erratum in

Abstract

Background and aim: Recent studies have suggested that the incidence rate (IR) and the rate of death (MR) of amyotrophic lateral sclerosis (ALS) are increasing. Still, it remains unclear whether this is due to improved case ascertainment or represents a true increase. We examined the development in the incidence and mortality of ALS in Denmark for 42 years.

Methods: We retrieved individual-level data of all patients aged above 18 years with first-time ALS diagnosed at any Danish department of neurology. The IR and MR were calculated based on data from 1980 to 2021, stratified by gender and age.

Results: We identified 5,943 patients with ALS and identified a total of 5,069 deaths in the nationwide population. Overall, the IR was 3.4 per 100,000 persons per year (95% CI 3.4-3.5). ALS incidence rose gradually during the study period, and the IR was 2.8 times higher (95% CI 2.4-3.2) when comparing the latest period (2018-2021) with the first (1980-1983). Parallel to the IR, the MR increased over time and was associated with male gender and rose with age at diagnosis, peaking in the 70-79-year age group.

Conclusion: In Denmark, the IR and MR of ALS increased threefold from 1980 to 2021, with steadily increasing risk related to male gender and in particular to higher age. Considering our aging societies, the number of elderly patients with ALS can be expected to increase considerably.

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

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Conflict of interest statement

Declarations. Conflicts of interest: On behalf of all authors, the corresponding author states that there is no conflict of interest. Ethical approval: The study was approved by The Central Jutland Regional Committee on Health Research Ethics (record number 1–45–70–63–21). Approval from the Ethical Committee is not required to conduct registry-based studies in Denmark.54

Figures

Fig. 1
Fig. 1
a ALS incidence rates per 100,000 from 1980 to 2021 in total and for males and females. ALS amyotrophic lateral sclerosis, IR incidence rate, PY person years. b ALS mortality rates per 100,000 from 1980 to 2021 in total and for males and females. ALS amyotrophic lateral sclerosis, MR mortality rate, PY person years
Fig. 2
Fig. 2
a ALS incidence rate stratified by age and year of diagnosis. b Male ALS incidence rate stratified by age and year of diagnosis. c Female ALS incidence rate stratified by age and year of diagnosis. d ALS mortality rate stratified by age and year of diagnosis. ALS amyotrophic lateral sclerosis, IR incidence rate, MR mortality rate, PY person years
Fig. 3
Fig. 3
a ALS incidence rate per 100,000 during the period from 1980 to 2021 stratified by gender and age. b ALS mortality rate per 100,000 during the period from 1980 to 2021 stratified by gender and age. ALS amyotrophic lateral sclerosis, IR incidence rate, MR mortality rate, PY person years
Fig. 4
Fig. 4
a Cumulative mortality in total and for men and women. b Cumulative mortality stratified by decennials. c Cumulative mortality stratified by age at diagnosis. ALS amyotrophic lateral sclerosis
See this image and copyright information in PMC

References

    1. Feldman EL, Goutman SA, Petri S et al (2022) Amyotrophic lateral sclerosis. Lancet 400(10360):1363–1380. 10.1016/s0140-6736(22)01272-7 - PMC - PubMed
    1. Goutman SA, Hardiman O, Al-Chalabi A et al (2022) Emerging insights into the complex genetics and pathophysiology of amyotrophic lateral sclerosis. Lancet Neurol 21(5):465–479. 10.1016/s1474-4422(21)00414-2 - PMC - PubMed
    1. Al-Chalabi A, Hardiman O (2013) The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol 9(11):617–628. 10.1038/nrneurol.2013.203 - PubMed
    1. Cicero CE, Mostile G, Vasta R et al (2017) Metals and neurodegenerative diseases. A systematic review. Environ Res 159:82–94. 10.1016/j.envres.2017.07.048 - PubMed
    1. Peters TL, Kamel F, Lundholm C et al (2017) Occupational exposures and the risk of amyotrophic lateral sclerosis. Occup Environ Med 74(2):87–92. 10.1136/oemed-2016-103700 - PMC - PubMed

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