Standard of Practice vs Polysomnography-Directed Nocturnal Non-Invasive Positive Pressure Ventilation in Preserving Respiratory Function and Quality of Life in ALS

To prospectively determine, in ALS patients who are prescribed nocturnal NIV as per current practice parameters, whether PSG titrated and adjusted NIV (P-NIV) is associated with improved preservation of diurnal respiratory function and quality of life over the course of the first 6 months after prescription of NIV, compared with standard of practice prescription and adjustment of NIV (S-NIV). Ancillary aims are to determine to what extent the prescription for P-NIV differs from S-NIV; whether respiratory function is improved in the morning following a night of P-NIV compared with S-NIV; to what extent S-NIV is associated with failure of nocturnal oxygenation and/or ventilation; and if P-NIV use is associated with improved survival, compared with S-NIV use.