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Population based epidemiology of amyotrophic lateral sclerosis using capture–recapture methodology
  1. Mark H B Huisman1,
  2. Sonja W de Jong1,
  3. Perry T C van Doormaal1,
  4. Stephanie S Weinreich2,
  5. H Jurgen Schelhaas3,
  6. Anneke J van der Kooi4,
  7. Marianne de Visser4,
  8. Jan H Veldink1,
  9. Leonard H van den Berg1
  1. 1Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Centre Utrecht, Utrecht, The Netherlands
  2. 2Dutch Association for Neuromuscular Diseases, Baarn, The Netherlands
  3. 3Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Centre for Neuroscience, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
  4. 4Department of Neurology, Amsterdam Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
  1. Correspondence to M H B Huisman, Department of Neurology, G03.228, University Medical Centre Utrecht, PO Box 85500, 3508 GA Utrecht, The Netherlands; m.h.b.huisman-3{at}


Background Variation in the incidence rate in epidemiological studies on amyotrophic lateral sclerosis (ALS) may be due to a small population size and under ascertainment of patients. The previously reported incidence decline in the elderly and a decrease in the male:female ratio in postmenopausal age groups have yet to be confirmed.

Methods ALS epidemiology in a large population based register in The Netherlands was studied between 1 January 2006 and 31 December 2009, and applied capture–recapture methodology in separate age and gender groups to adjust for the number of unobserved patients.

Results 1217 incident patients were observed, and a capture–recapture incidence of 2.77 per 100 000 person-years (95% CI 2.63 to 2.91). Prevalence on 31 December 2008 was 10.32 per 100 000 individuals (95% CI 9.78 to 10.86). The incident cohort had a higher median age at onset (63.0 vs 58.1 years) and more bulbar onset patients (30.0% vs 19.1%) compared with the prevalent cohort. Incidence and prevalence peaked in the 70–74 year age group followed by a rapid decline in older age. The male:female ratio in the premenopausal age group (1.91, 95% CI 1.32 to 2.79) was not significantly higher than that in the postmenopausal age group (1.50, 95% CI 1.34 to 1.67).

Conclusion The marked difference in patient characteristics between incident and prevalent cohorts underscores the importance of including incident patients when studying susceptibility or disease modifying factors in ALS. The incidence decline in the elderly may suggest that ALS is not merely the result of ageing. Absence of a significant postmenopausal drop in the male:female ratio suggests that the protective role of female sex hormones in ALS is limited.

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  • See Editorial commentary, p 1066

  • JHV and LHvdB contributed equally to the manuscript.

  • Linked article 246876.

  • Funding This project was funded by the Prinses Beatrix Fonds, VSB fonds, H Kersten and M Kersten (Kersten Foundation), The Netherlands ALS Foundation and J R van Dijk and the Adessium Foundation. The research leading to these results has received funding from the European Community's Health Seventh Framework Program (FP7/2007–2013) under grant agreement No 259867.

  • Competing interests LHvdB received travel grants and consultancy fees from Baxter.

  • Ethics approval This study was conducted with the approval of the institutional review board of the University Medical Centre Utrecht.

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

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