The majority of patients with amyotrophic lateral sclerosis (ALS) develop chronic ventilatory failure which substantially adds to disease burden and massively affects prognosis. Both quality of life and life expectancy can be influenced by non-invasive ventilation (NIV). Initiation of NIV is indicated in case of sleep-disordered breathing (SDB), daytime hypercapnia, or significant diaphragmatic weakness. Since nocturnal hypercapnia and sleep apnea substantially reduce sleep quality we investigated the effects of NIV on objective measures of both sleep quality and ventilation in patients with ALS. We retrospectively analysed polysomnography (PSG) and oxycapnometry (OC) data obtained from patients with ALS in whom NIV was initiated based on the presence of SDB. PSG and OC were used in all patients for establishing the diagnosis of SDB (t0), for treatment initiation (t1) and for follow-up examination at 3, 9, and 15 months (t2, t3, and t4, respectively). As outcome parameters we chose sleep efficiency (SE), slow wave sleep percentage (N3), REM sleep percentage (REM), apnea hypopnea index (AHI), oxygen desaturation index (ODI), desaturation time (T < 90), respiratory rate (RR) and maximal nocturnal transcutaneous carbon dioxide tension (tc-CO2). Records from 62 patients were analysed. Mean age was 63.2 years, and mean disease duration was 23.1 months. 28 patients were female, and 22 patients reported bulbar onset of ALS symptoms. All patients were started on pressure and volume-controlled bilevel ventilation via a nasal or oronasal interface. Comparison of the above parameters at t0 and t1 showed significant improvement of AHI and ODI, oxygenation, CO2 elimination, and sleep stage duration (N3, REM) in the first night on NIV. Sleep efficiency was neither improved nor reduced by NIV. At t2, t3 and t4 the number of PSG records available became smaller because in many patients, follow-up studies included rather polygraphy than PSG (t2, 33 patients, t3, 15 patients, t4, 11 patients with PSG data). ANOVA for repeated measures revealed stable normoxia and eucapnia under bilevel ventilatory support. Objective measures of sleep quality did not show any significant change over time. Increasing duration of NIV per day was found in all patients in the course of the disease, reflecting disease progression. In patients with ALS, NIV initiation significantly improves objective sleep quality, oxygenation and CO2 elimination in the first night of treatment. Over time, NIV is able to warrant nocturnal normoventilation with only minor changes to ventilator settings. Objective sleep quality does not significantly change in the first 15 months after onset of NIV.
Read full abstract