Progresses in the multidisciplinary care, particularly in the nutritional and respiratory management, allowed improving the quality of life and survival of ALS patients. Non-invasive ventilation is initially performed overnight but with the progression of diaphragmatic weakness, a continuous ventilatory support is often needed. Advances directives regarding end-of-life issues are important to respect patient's wishes regarding the choice of invasive ventilation with tracheostomy. Riluzole is the only neuroprotective drug that has demonstrated a significant but modest effect on survival and is well tolerated. The failure of therapeutic trials may be partly related to the heterogeneity of the disease. Recent progress in the genetic of ALS may lead to new therapeutic strategies. Results of therapeutic trials with drugs or nutritional intervention are expected in 2014.