title: Current view and perspectives in amyotrophic lateral sclerosis.
abstract: Amyotrophic lateral sclerosis (ALS), identified as a distinct clinical entity by Charcot since the end of the nineteenth century, is a devastating and fatal neurodegenerative disorder that affects motor neurons in the brain, brainstem and spinal cord. Survival of patients with ALS is associated with several factors such as clinical phenotype, age at onset, gender, early presence of respiratory failure, weight loss and treatment with Riluzole (the only disease-modifying drug approved for this disease). Nowadays, there is still no curative treatment for ALS: palliative care and symptomatic treatment are therefore essential components in the management of these patients. Nevertheless, the scientific knowledge in the field of ALS motor neuron degeneration is growing, with the prospect of new treatments. Based on this physiopathological knowledge, several new therapeutic targets are being studied, involving various mechanisms such as excitotoxicity, neuroinflammation, mitochondrial dysfunction, oxidative stress, RNA metabolism and other attractive concepts. Moreover, it is also important to identify reliable biomarkers that will be essential components for future therapeutic development and study design in ALS. In this review, we present the main recent advances and promising therapeutics and biomarkers in the field of ALS.
authors.affiliation: Department of Neurology, Neuro-Muscular Unit and ALS Center, CHU de Bordeaux (groupe hospitalier Pellegrin), Bordeaux cedex, France.
authors.forename: Stéphane
authors.identifier: 0000-0003-0775-4625
authors.initials: S
authors.lastname: Mathis
authors.affiliation: 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: Department of Neurology, CHU Poitiers, University of Poitiers, Poitiers, France.
authors.forename: Adrien
authors.initials: A
authors.lastname: Julian
authors.affiliation: Department of Neurology, ALS Center, Centre de compétence SLA-fédération Tours-Limoges, CHU de Tours, Tours cedex 9, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Corcia
authors.affiliation: Department of Neurology, Neuro-Muscular Unit and ALS Center, CHU de Bordeaux (groupe hospitalier Pellegrin), Bordeaux cedex, France.
authors.forename: Gwendal
authors.initials: G
authors.lastname: Le Masson
keywords: Charcot; amyotrophic lateral sclerosis; biomarkers; motor neuron; neurodegeneration; research