Efficacy of Noninvasive Ventilation in Amyotrophic Lateral Sclerosis (ALS)

This study will test the hypothesis that noninvasive ventilation (NIV) as prescribed in current medical practice for use in amyotrophic Lateral Sclerosis (ALS) patients fails to deliver adequate breathing support over a night of use in the patient's home. ALS patients who come to the ALS Center for their routine 3 month follow up exam and are currently using NIV will be asked to complete questionnaires regarding their quality of sleep, quality of life and general level of function, and to undergo a home sleep study, using a safe, comfortable and reliable breathing monitoring system during a night of sleep. If the questionnaires or the sleep study show failure of the breathing device, the investigators will work with the patient to fix the problem and then offer a second study to make sure that the changes were helpful. The results of this study may help to develop subsequent studies and to improve the guidelines used for care of ALS patients.