Effects of non-invasive ventilation on objective sleep and nocturnal respiration in patients with amyotrophic lateral sclerosis

Abstract

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.

Reduced survival in patients with ALS with upper airway obstructive events on non-invasive ventilation

“…This study confirms that upper airway obstruction is one of the mechanisms of NIV failure in ALS 10 11 21 22. The prevalence of upper airway obstruction, which has not been previously evaluated systematically, is high (45% of our patients after correction of leaks).…”

Non-invasive ventilation and hypercapnia-associated symptoms in amyotrophic lateral sclerosis

“…13,14 In this study, we found an overall median survival of 9.6 months, which is in line with previous publications. 16,17 In agreement with these findings, other studies found an improvement of quality of sleep, daytime fatigue, depression, 18 and quality of life. 16,17 In agreement with these findings, other studies found an improvement of quality of sleep, daytime fatigue, depression, 18 and quality of life.…”

Year in review 2016: Interstitial lung disease, pulmonary vascular disease, pulmonary function, paediatric lung disease, cystic fibrosis and sleep

“…Consequently, the authors questioned the value of EPAP in ALS. In contrast, other reports have identified a high incidence of upper airway obstruction in these patients at baseline 142 and a major cause of ineffective NIV and worse survival. 143 Optimizing NIV is an important issue with the potential for significant clinical consequences and needs to be addressed in future studies.…”