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Cited by 772Amyotrophic Lateral Sclerosis ResearchAmyloidosis: Diagnosis, Treatment, OutcomesNeurogenetic and Muscular Disorders Research

Incidence of amyotrophic lateral sclerosis in Europe

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Logroscino, G., Traynor, B. J, Hardiman, O., Chio, A., Mitchell, D., Swingler, R. J, Millul, A., Benn, E. +2 more·2010·Journal of Neurology, Neurosurgery & Psychiatry
DOI 10.1136/jnnp.2009.183525PMC PMC2850819
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Abstract

BACKGROUND: Geographical differences in the incidence of amyotrophic lateral sclerosis (ALS) have been reported in the literature but comparisons across previous studies are limited by different methods in case ascertainment and by the relatively small size of the studied populations. To address these issues, the authors undertook a pooled analysis of European population based ALS registries. METHODS: All new incident ALS cases in subjects aged 18 years old and older were identified prospectively in six population based registries in three European countries (Ireland, UK and Italy) in the 2 year period 1998-1999, with a reference population of almost 24 million. RESULTS: Based on 1028 identified incident cases, the crude annual incidence rate of ALS in the general European population was 2.16 per 100 000 person years; 95% CI 2.0 to 2.3), with similar incidence rates across all registries. The incidence was higher among men (3.0 per 100 000 person years; 95% CI 2.8 to 3.3) than among women (2.4 per 100 000 person years; 95% CI 2.2 to 2.6). Spinal onset ALS was more common among men compared with women, particularly in the 70-80 year age group. Disease occurrence decreased rapidly after 80 years of age. CONCLUSIONS: ALS incidence is homogeneous across Europe. Sex differences in incidence may be explained by the higher incidence of spinal onset ALS among men, and the age related disease pattern suggests that ALS occurs within a susceptible group within the population rather than being a disease of ageing.

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  • Incidence estimates for ALS range from about 2 to 3 per 100,000 people per year; ALS is more common in males and military veterans.

    “Incidence estimates for ALS range from about 2 to 3 per 100 000 people per year; ALS is more common in males and military veterans (11,12).”

    From Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort (2018) · cites this paper as [12]

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  • Environmental factors are believed to play an important role in development of ALS.

    “As a result, environmental factors are believed to play an important role in the development of ALS (12).”

    From Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort (2018) · cites this paper as [12]

    The Introduction (page unavailable) says environmental risk factors may be relevant to ALS pathogenesis, and the Discussion speculates that an environmental risk factor may contribute to differences in disease onset between men and women. The paper does not establish or assert that environmental factors play an important role in ALS development generally; it describes the cause of ALS as unknown. (LLM, conf=0.82)

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  • ALS onset is most often sporadic, with approximately 10% of cases considered familial; there is a known genetic component for approximately 5%-10% of sporadic cases and 60% of familial cases.

    “ALS onset is most often sporadic, with approximately 10% of cases considered familial.There is a known genetic component for approximately 5%-10% of sporadic cases and 60% of familial cases (12,13).”

    From Chronic Traumatic Encephalopathy Within an Amyotrophic Lateral Sclerosis Brain Bank Cohort (2018) · cites this paper as [12]

    The citation follows the sentence claiming known genetic components in 5%–10% of sporadic cases and 60% of familial cases. The Results section discusses regional differences in familial ALS cases, but neither it nor the passages retrieved for genetic components and those percentages addresses the claimed figures. (LLM, conf=0.75)

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Cites

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Research works printed but not linked: 19

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  4. [4]Traynor BJ Codd MB Corr B Forde C Frost E Hardiman O Incidence and prevalence of ALS in Ireland, 1995-1997: a population-based study Neurology 1999 52 504 9 10025778
  5. [5]Incidence of ALS in Italy: evidence for a uniform frequency in Western countries Neurology 2001 56 239 44 11160962
  6. [6]Logroscino G Beghi E Zoccolella S Palagano R Fraddosio A Simone IL Lamberti P Lepore V Serlenga L Incidence of amyotrophic lateral sclerosis in southern Italy: a population based study J Neurol Neurosurg Psychiatry 2005 76 1094 8 16024886 PMC1739746
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  9. [9]Brooks BR El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. Subcommittee on Motor Neuron Diseases/Amyotrophic Lateral Sclerosis of the World Federation of Neurology Research Group on Neuromuscular Diseases and the El Escorial “Clinical limits of amyotrophic lateral sclerosis” workshop contributors J Neurol Sci 1994 124 Suppl 96 107 7807156
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Authors
Logroscino, G., Traynor, B. J., Hardiman, O., Chio, A., Mitchell, D., Swingler, R. J., Millul, A., Benn, E., Beghi, E., & EURALS, F.
Affiliations
  • University of Bari Aldo Moro (IT)
  • National Institutes of Health (US)
  • Trinity College Dublin (IE)
  • Royal Preston Hospital (GB)
  • Ninewells Hospital (GB)
  • Mario Negri Institute for Pharmacological Research (IT)
  • Columbia University (US)
Corresponding author
Logroscino, G.
ORCID
Venue
Journal of Neurology, Neurosurgery & Psychiatry
Publisher
BMJ
Published
2009-08-25
Volume
81
Issue
4
Pages
385-390
ISSN
0022-3050 (ISSN-L)
DOI
10.1136/jnnp.2009.183525
PMC
PMC2850819
OpenAlex
W2001432662
Cited by
772
References
2
Citations by year
2026: 19 · 2025: 28 · 2024: 34 · 2023: 40 · 2022: 48 · 2021: 63 · 2020: 58 · 2019: 41 · 2018: 61 · 2017: 63 · 2016: 57 · 2015: 55 · 2014: 54 · 2013: 52 · 2012: 53
FWCI
20.70
Type
journal article
Language
en
Open access
Locations
  • Journal of Neurology Neurosurgery & Psychiatry (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • PubMed Central (acceptedVersion)
  • Institutional Research Information System University of Turin (University of Turin) (submittedVersion)
  • HighWire Press Open Archive (submittedVersion)
  • CINECA IRIS Institutional Research Information System (University of Bari Aldo Moro) (submittedVersion)
Funders
National Institutes of Health (Z01-AG000949)
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
  • Health Sciences › Medicine › Genetics › Neurogenetic and Muscular Disorders Research
Keywords
amyotrophic lateral sclerosis, incidence, sex differences, spinal-onset ALS, age-related incidence, population-based registry, pooled analysis, European populations, case ascertainment
MeSH terms
Adult, Aged, Amyotrophic Lateral Sclerosis, Europe, Female, Humans, Male, Middle Aged, Prospective Studies, Incidence

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