title: Management and therapeutic perspectives in amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting both upper and lower motor neurons. Despite much research and effort, no clear insights into a unifying hypothesis for the pathogenesis has so far emerged for this disease. Areas covered: We review the main pathophysiological hypotheses and the potential therapeutic targets in ALS, as well as the management of these patients (in order to improve their survival and quality of life). Expert commentary: ALS is a complex neurodegenerative disease, these days considered as a multisystem disorder with predominant motor symptoms (and various clinical forms). Further comprehension of the pathophysiology of this disease is required, although pathophysiological mechanisms (such as TDP-43) show promise in the search for new therapies. There is still no curative treatment for ALS, but the emergence of multidisciplinary specialized ALS clinics has increased both the quality of life and the survival of these patients.
authors.affiliation: a Department of Neurology , Neuro-Muscular Unit and ALS Center, CHU de Bordeaux, groupe hospitalier Pellegrin , Bordeaux , France.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Mathis
authors.affiliation: b Department of Neurology , ALS center, Centre de compétence SLA-fédération Tours-Limoges, CHU de Limoges , Limoges , France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Couratier
authors.affiliation: c Department of Neurology, CHU Poitiers , University of Poitiers , Poitiers , France.
authors.forename: Adrien
authors.initials: A
authors.lastname: Julian
authors.affiliation: d Department and Laboratory of Neurology, Centre de Référence 'neuropathies périphériques rares' , University Hospital of Limoges , Limoges , France.
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Vallat
authors.affiliation: e Department of Neurology, ALS center , Centre de compétence SLA-fédération Tours-Limoges, CHU de Tours , Tours , France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Corcia
authors.affiliation: a Department of Neurology , Neuro-Muscular Unit and ALS Center, CHU de Bordeaux, groupe hospitalier Pellegrin , Bordeaux , France.
authors.forename: Gwendal
authors.initials: G
authors.lastname: Le Masson
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D006801:Humans; D009410:Nerve Degeneration; D011788:Quality of Life
keywords: ALS; Amyotrophic lateral sclerosis; animal models; management; riluzole; treatment