In this review, we summarize the most important recent developments in the 
treatment of amyotrophic lateral sclerosis (ALS). In terms of disease-modifying 
treatment options, several drugs such as dexpramipexole, pioglitazone, lithium, 
and many others have been tested in large multicenter trials, albeit with 
disappointing results. Therefore, riluzole remains the only directly 
disease-modifying drug. In addition, we discuss antisense oligonucleotides 
(ASOs) as a new and potentially causal treatment option. Progress in symptomatic 
treatments has been more important. Nutrition and ventilation are now an 
important focus of ALS therapy. Several studies have firmly established that 
noninvasive ventilation improves patients' quality of life and prolongs 
survival. On the other hand, there is still no consensus regarding best 
nutritional management, but big multicenter trials addressing this issue are 
currently ongoing. Evidence regarding secondary symptoms like spasticity, muscle 
cramps or sialorrhea remains generally scarce, but some new insights will also 
be discussed. Growing evidence suggests that multidisciplinary care in 
specialized clinics improves survival.