For the past decade or so, the research in ALS has dramatically changed, coinciding with the discovery of SOD1 mutations, the generation of SOD1 transgenic rodents, and the introduction of riluzole. The ALS practice guidelines have been published in a number of countries, whereas the National Database has shown the actual reality of patient practice in ALS. Multidisciplinary ALS Clinics, consisting of a team of health care professionals, provide highly specialized quality care for patients and families, and have become the standard of care in many countries. A number of mostly retrospective studies report that non-invasive positive pressure ventilation prolongs survival and improves the quality of life and other functions. Enteral feeding certainly improves quality of life, but it is still uncertain if it prolongs survival. Although end of life issues in ALS care have been difficult more and more ALS clinicians are paying serious attention to it. Clinical trials are imperative to find effective medications for ALS, and many different pharmacological agents, based on a number of different hypotheses in ALS, are being actively tested or are about to be tested in the near future. Undoubtedly, we are in the midst of incredible progress in ALS.