title: The epidemiology and treatment of ALS: focus on the heterogeneity of the disease and critical appraisal of therapeutic trials.
abstract: Abstract Effective treatments for amyotrophic lateral sclerosis (ALS) have remained elusive. Only riluzole, a drug thought to affect glutamate metabolism, improves survival albeit to modest extent. Explanations for the negative results of therapeutic trials include a likely heterogeneity, both in disease susceptibility and pathogenic mechanisms, and faulty methodology of clinical trials. Further understanding of these factors will lead to improvements in patient stratification, and in the design of future clinical trials.
authors.affiliation: Istituto di Ricerche Farmacologiche "Mario Negri", Via G. la Masa 19, 20156 Milano, Italy. ettore.beghi@marionegri.it
authors.forename: Ettore
authors.initials: E
authors.lastname: Beghi
authors.forename: Adriano
authors.initials: A
authors.lastname: Chiò
authors.forename: Philippe
authors.initials: P
authors.lastname: Couratier
authors.forename: Jesùs
authors.initials: J
authors.lastname: Esteban
authors.forename: Orla
authors.initials: O
authors.lastname: Hardiman
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Logroscino
authors.forename: Andrea
authors.initials: A
authors.lastname: Millul
authors.forename: Douglas
authors.initials: D
authors.lastname: Mitchell
authors.forename: Pierre-Marie
authors.initials: PM
authors.lastname: Preux
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Pupillo
authors.forename: Zorica
authors.initials: Z
authors.lastname: Stevic
authors.forename: Robert
authors.initials: R
authors.lastname: Swingler
authors.forename: Bryan J
authors.initials: BJ
authors.lastname: Traynor
authors.forename: Leonard H
authors.initials: LH
authors.lastname: Van den Berg
authors.forename: Jan H
authors.initials: JH
authors.lastname: Veldink
authors.forename: Stefano
authors.initials: S
authors.lastname: Zoccolella
mesh_terms: D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy / Q000453:epidemiology* / Q000378:metabolism / Q000628:therapy*; D000975:Antioxidants / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D004750:Enteral Nutrition; D005060:Europe / Q000453:epidemiology; D018698:Glutamic Acid / Q000378:metabolism; D006801:Humans; D015994:Incidence; D036341:Intercellular Signaling Peptides and Proteins / Q000627:therapeutic use; D008875:Middle Aged; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D012042:Registries; D012107:Research Design; D012121:Respiration, Artificial; D019782:Riluzole / Q000627:therapeutic use; D016896:Treatment Outcome; D014481:United States / Q000453:epidemiology; D044465:White People / Q000706:statistics & numerical data
chemical_list: D000975:Antioxidants; D036341:Intercellular Signaling Peptides and Proteins; D018698:Glutamic Acid; D019782:Riluzole