In amyotrophic lateral sclerosis (ALS), non-invasive ventilation (NIV) is indicated if sleep-disordered breathing (SDB), daytime hypercapnia, or significant diaphragmatic weakness is present. We investigated both short-term and long-term effects of NIV on objective measures of sleep and nocturnal respiration in patients with ALS. Polysomnography (PSG) and transcutaneous capnography were conducted for diagnosis of SDB (T0), for treatment initiation (T1), and follow-up 3, 9, and 15 months later (T2, T3, and T4, respectively). Records from 65 patients were retrospectively analyzed at T0 and T1. At subsequent timepoints, the number of full data sets decreased since follow-up sleep studies frequently included polygraphy rather than PSG (T2, 38 patients, T3, 17 patients, T4, 11 patients). At T0, mean age was 63.2 years, 29 patients were female, and 22 patients had bulbar ALS. Immediate sequelae of NIV initiation included significant increases of slow wave sleep, rapid eye movement sleep, and oxygen saturation. Mean apnea-hypopnea index, respiratory rate, and the maximum transcutaneous carbon dioxide tension were reduced. At T2-T4, normoxia and normocapnia were preserved. Sleep quality measures showed no alteration as diurnal use of NIV gradually increased reflecting disease progression. In contrast to previous reports, improvement of sleep and respiratory outcomes was found in both non-bulbar and bulbar patients. NIV significantly improves objective sleep quality and SDB in the first night of treatment in patients with bulbar and non-bulbar ALS. NIV warrants nocturnal normoventilation without deterioration of sleep quality in the long run with only minor changes to ventilator settings.
Keywords: Amyotrophic lateral sclerosis; Hypoventilation; Non-invasive ventilation; Polysomnography; Sleep quality.