The El Escorial criteria: Strengths and weaknesses

Abstract

The El Escorial criteria for the diagnosis of amyotrophic lateral sclerosis (ALS) were established 20 years ago and have been used as inclusion criteria for clinical trials. However, concerns have been raised concerning their use as diagnostic criteria in clinical practice. Moreover, as modern genetics have shed new light on the heterogeneity of ALS and the close relationship between ALS and frontotemporal dementia (FTD) recognized, the World Federation of Neurology Research Group on ALS/MND has initiated discussions to amend and update the criteria, while preserving the essential components for clinical trial enrolment purposes.

Key words::

• ALS
• El Escorial

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

Notice of correction

The version of this article published online ahead of print on 08 Dec 2014 contained an error in the affiliation details. Massimo Filippi’s address should have read San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, Italy. The error has been corrected for this version.