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Incidence of amyotrophic lateral sclerosis in Europe
  1. Giancarlo Logroscino1,
  2. Bryan J Traynor2,
  3. Orla Hardiman3,
  4. Adriano Chiò4,
  5. Douglas Mitchell5,
  6. Robert J Swingler6,
  7. Andrea Millul7,
  8. Emma Benn8,
  9. Ettore Beghi7
  10. for EURALS
  1. 1Department of Neurology and Psychiatry, University of Bari, Bari, Italy
  2. 2Neuromuscular Diseases Research Group, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, Maryland, USA
  3. 3Department of Neurology, Beaumont Hospital and Trinity College, Dublin, Ireland
  4. 4Dipartimento di Neuroscienze, Università di Torino, Torino, Italy
  5. 5ALS Care and Research Centre, Royal Preston Hospital, Preston, UK
  6. 6Department of Neurology, Ninewells Hospital and Medical School, Dundee, UK
  7. 7Istituto Ricerche Farmacologiche Mario Negri Milano and Clinica Neurologica, Università di Milano-Bicocca, Monza, Italy
  8. 8Getrude Sergievsky Center, Columbia University, New York, USA
  1. Correspondence to G Logroscino, Department of Neurology and Psychiatry, Piazza Giulio Cesare, Policlinico, 70121 Bari, Italy; giancarlo.logroscino{at}neurol.uniba.it

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.

  • EPIDEMIOLOGY
  • MOTOR NEURON DISEASE
  • ALS

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Footnotes

  • GL and BJT contributed equally to this paper.

  • Funding This work was supported by the Intramural Research Program of the National Institute on Aging (project Z01 AG000949-02) and the National Institute on Neurological Disorders and Stroke (NINDS), Istituto Superiore di Sanità, Contract No 526D/8, Rome, Italy and the ALS Association.

  • Competing interests None.

  • Ethics approval Ethics approval was obtained at each local registry site.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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