ALS is characterized by the degeneration of upper and lower motor neurons. In about 70% of patients with ALS the disease has an spinal onset, while about 30% of the patients have a bulbar onset. Cognitive dysfunction and behavioral changes are seen in about 50% of the patients, and 15% develop frontotemporal dementia. There is no single test that provides the ALS diagnosis. The diagnosis is based on clinical and electrophysiological signs, and the exclusion of other diseases. The only disease modulating treatment approved for ALS in Sweden is Riluzole, sadly only with limited effect. Other treatments are symptomatic and the goal is to help patients achieve the best possible quality of life through multidiciplinary ALS teams.