Recently, formal standards for the management of amyotrophic lateral sclerosis (ALS) have been proposed by the American Academy of Neurology (AAN). However, there are few information about the actual care of ALS. We have assessed the management of ALS in Italy in various clinical settings, on basis of a self-reported questionnaire. Thirty-six out of the 80 Italian ALS neurological departments with a particular interest in ALS care answered the questionnaire. The centers were subdivided according the mean number of patients currently followed-up (> or =30 vs. <30). An integrated health-care team for ALS existed in all large centers but only in 14% of small centers (p=0.0001). Diagnosis was communicated to most but not all patients. Symptomatic therapies were generally offered to patients in all centers. Nutritional interventions, including percutaneous endoscopic gastrostomy (PEG), were proposed by most centers, but the percentage of patients who underwent PEG was significantly higher in large centers (p=0.04). Respiratory management seemed to be lacking both in large and in small centers since non-invasive positive pressure ventilation (NIPPV) was proposed by only 70% of large and 50% of small centers; however, the percentage of patients who underwent NIPPV was significantly higher in large centers (p=0.03). Moreover, the discussion of respiratory issues was performed quite late in the course of the disease, usually when the patients have first respiratory symptoms. Therefore, there are considerable opportunities to improve the care of ALS patients in Italy, primarily through the education of neurologists on AAN standards of care for ALS.