Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving damage to the upper and lower motoneurones. The etiopathogenesis of the disease is probably associated with many factors, but still unclear. Such variables as oxidative damage, glutamate toxicity, trophic factors deficit, genetic, environmental and autoimmunological factors are taken into consideration. The treatment should be combined so as to affect various potential neurodegeneration mechanisms. Medicines inhibiting glutamate hyperactivity and drugs eliminating free radicals should be administered together with the application of neurotrophic factors and gene therapy.