title: 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.
authors.affiliation: San Raffaele Scientific Institute and Vita-Salute San Raffaele University , Milan , Italy.
authors.forename: Federica
authors.initials: F
authors.lastname: Agosta
authors.forename: Ammar
authors.initials: A
authors.lastname: Al-Chalabi
authors.forename: Massimo
authors.initials: M
authors.lastname: Filippi
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
authors.forename: Ryuji
authors.initials: R
authors.lastname: Kaji
authors.forename: Vincent
authors.initials: V
authors.lastname: Meininger
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
authors.forename: Pamela
authors.initials: P
authors.lastname: Shaw
authors.forename: Jeremy
authors.initials: J
authors.lastname: Shefner
authors.forename: Leonard H
authors.initials: LH
authors.lastname: van den Berg
authors.forename: Albert
authors.initials: A
authors.lastname: Ludolph
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000503:physiopathology; D002986:Clinical Trials as Topic; D057180:Frontotemporal Dementia / Q000175:diagnosis*; D006801:Humans; D059906:Neuroimaging; D009460:Neurologic Examination / Q000379:methods / Q000592:standards; D012720:Severity of Illness Index
keywords: ALS; El Escorial