[Etiological drug therapy for amyotrophic lateral sclerosis]

Progress in the last decades has greatly strengthened our understanding of the etiopathogenic mechanisms of amyotrophic lateral sclerosis. Such progress has lead to an evaluation of several compounds affecting excitotoxicity, oxidative stress, apoptosis, growth factors and inflammation. Riluzole has clearly proven efficiency on mortality with a 35 p.cent reduction of death or tracheotomy at 18 months. While the mechanism of action of this compound remains to be fully elucidated, there is evidence in favor of a neuroprotective effect. Other compounds have been tried in ALS. To date, results have been disappointing with worsening even being noted in some cases. These negative studies offer some insight into the pathophysiological mechanism regulating the disease, raising pertinent questions concerning the transposition of animals models to humans and about interractions between these different compounds and riluzole.