Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease which was thought to be untreatable for a long time. However, recent evidence in men indicates that antiglutamatergic strategies are the first to have an influence on its pathogenesis and slow down the disease process. Since the effect of the drugs is still small, this progress cannot only be seen as a success of the present but most also be acknowledged as a starting point for the future. How will these future studies look like? They will have to take into account that ALS presumably has a long preclinical period and they will use a number of novel compounds and treatment strategies which have recently been shown to be effective in a transgenic animal model. This also implies that we are likely to use combination therapies and have to try to treat patients early. The latter will be necessarily connected with the demand for a novel clinical attitude to the diagnosis of the disease.