The changing picture of amyotrophic lateral sclerosis: lessons from European registers (original) (raw)

The changing picture of amyotrophic lateral sclerosis: lessons from European registers

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  1. Orla Hardiman1,2,
  2. Ammar Al-Chalabi3,
  3. Carol Brayne4,
  4. Ettore Beghi5,
  5. Leonard H van den Berg6,
  6. Adriano Chio7,
  7. Sarah Martin3,
  8. Giancarlo Logroscino8,
  9. James Rooney1
  10. 1 Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College, Dublin, Ireland
  11. 2 Department of Neurology, Beaumont Hospital, Beaumont, Ireland
  12. 3 Department of Basic and Clinical Neuroscience, Maurice Wohl Clinical Neuroscience Institute, Institute of Psychiatry, Psychology and Neuroscience, King’s College London, London, UK
  13. 4 Cambridge Institute of Public Health, University of Cambridge School of Clinical Medicine, Cambridge, United Kingdom
  14. 5 Neurological Diseases Laboratory, Department of Neurosciences, IRCCS Mario Negri, Milano, Italy
  15. 6 Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, Utrecht, The Netherlands
  16. 7 Department of Neurosciences, University of Turin, Turin, Italy
  17. 8 Department of Neuroscience, University of Bari, Bari, Italy
  18. Correspondence to Orla Hardiman, Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College, Dublin, Ireland; orla{at}hardiman.net

Abstract

Prospective population based-registers of amyotrophic lateral sclerosis (ALS) have operated in Europe for over two decades, and have provided important insights into our understanding of ALS. Here, we review the benefits that population registers have brought to the understanding of the incidence, prevalence, phenotype and genetics of ALS and outline the core operating principles that underlie these registers and facilitate international collaboration. Going forward, we offer lessons learned from our collective experience of operating population-based ALS registers in Europe for over two decades, focusing on register design, maintenance, identification and management of bias and the value of cross-national harmonisation and integration.

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