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

J Neurol Neurosurg Psychiatry. 2017 Jul;88(7):557-563. doi: 10.1136/jnnp-2016-314495. Epub 2017 Mar 11.

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.

Keywords: ALS; EPIDEMIOLOGY; MOTOR NEURON DISEASE; NEUROEPIDEMIOLOGY.

Publication types

  • Review

MeSH terms

  • Amyotrophic Lateral Sclerosis / epidemiology*
  • Amyotrophic Lateral Sclerosis / genetics*
  • Ethnicity
  • Europe / epidemiology
  • Humans
  • Incidence
  • Phenotype
  • Population Surveillance
  • Prevalence
  • Prospective Studies
  • Registries / statistics & numerical data*
  • Risk Factors