Therapies in amyotrophic lateral sclerosis–beyond riluzole

Several mechanisms have been proposed to account for the progressive motor neurone death evident in amyotrophic lateral sclerosis. These include oxidative stress, neurofilament damage, mitochondrial abnormalities, glutamate-mediated excitotoxicity and altered responses to hypoxia. Current quests for therapies involve studying combinations of agents that act by various mechanisms and evaluating stem-cell therapies in model systems. High-throughput cell-culture screening systems are also being developed. The goal for therapy is to define agents that clearly prevent progression of, and it is hoped even reverse, motor neurone loss.