From El Escorial to Awaji: Where do We Go Next With the Amyotrophic Lateral Sclerosis criteria?

Abstract

SUMMARY Making an early and accurate diagnosis in amyotrophic lateral sclerosis is important for patients and their families and for entry in clinical trials. Amyotrophic lateral sclerosis remains a clinical diagnosis, requiring the presence of upper and lower motor neuron symptoms and signs in multiple body regions, in patients with a progressive disease course and after exclusion of other diseases that can mimic the clinical presentation. Research criteria have been developed to allow uniform diagnosis. The original El Escorial criteria have been revised twice to improve the sensitivity. In this report, the current scientific status of these criteria is reviewed and suggestions for further adaptations are made.