Impact of non-invasive ventilation on objective sleep and nocturnal respiration in patients with type I myotonic dystrophy

Abstract

Background: Patients with adult onset type I myotonic dystrophy (DM 1) may develop nocturnal hypoventilation (NH), and initiation of nocturnal positive non-invasive ventilation (NIV) therapy may be indicated and clinically useful. Studies assessing long term adherence to and benefit from NIV in terms of improvements in objective sleep and breathing parameters in DM 1 patients with NH have not yet been published. Methods: We retrospectively collected data on full polysomnography and capnometry results along with clinical data and sleep-related symptom scores in adult patients with genetically proven DM 1 and an initiation of NIV due to proven NH between 2010 and 2017. Follow up studies in our sleep laboratory were scheduled and data was collected therein. Results: Twenty eight patients (age 41.2 ± 11.7 years, BMI 25.8 ± 7.0 kg/m², 50% male) with DM 1 and proven NH were initiated on NIV and hence included into this study. NIV led to significant improvement of ventilation and oxygenation in the first night of treatment. Follow-up sleep studies revealed stable normoxia and normocapnia without deterioration of sleep outcomes. Conclusions: In DM 1 NIV significantly improves sleep and breathing already in the first night of treatment. NIV warrants nocturnal long-term normoventilation without deterioration of sleep quality.