Journal
NEUROMUSCULAR DISORDERS
Volume 29, Issue 4, Pages 302-309Publisher
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.nmd.2019.02.006
Keywords
Myotonic dystrophy type 1; Sleep-disordered breathing; Hypoventilation; Capnometry; Non-invasive ventilation
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Funding
- Lowenstein Medical GmbH (Bad Ems, Germany)
- Lowenstein Foundation (Bad Ems, Germany)
- ResMed
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Patients with myotonic dystrophy type I (DM1) may develop nocturnal hypoventilation, requiring non-invasive ventilation. Data on long-term adherence to non-invasive ventilation, or sleep and ventilation outcomes are scarce. We retrospectively collected baseline polysomnography and capnometry results from 36 adult patients with sleep-related symptoms (42.9 +/- 12.5 years, 20 female), plus follow-up sleep study records from those treated with non-invasive ventilation. Sleep-disordered breathing was found in 33 patients (91.7%) including 8 (22.2%) with daytime hypercapnia. Twenty-six patients (72.2%) showed nocturnal hypoventilation on transcutaneous capnometry. The sensitivity of oximetry to detect nocturnal hypoventilation was only 0.38. Twenty-eight patients (77.8%) showed sleep apnea, which was predominantly obstructive (n = 8), central (n = 9), or mixed (n = 11). Thirty-two patients were initiated on non-invasive ventilation which significantly improved ventilation and oxygenation in the first night of treatment. Follow-up revealed stable normoxia and normocapnia without deterioration of sleep outcomes for up to 52 months. Adherence to treatment was low to moderate, with substantial inter-individual variability. Sleep disordered breathing is highly prevalent in adult DM1 patients complaining of daytime sleepiness, and non-invasive ventilation significantly, rapidly and persistently improves nocturnal gas exchange. Capnometry is superior to oximetry for detection of nocturnal hypoventilation. Adherence to non-invasive ventilation remains a major issue in DM1. and long-term treatment benefits should be individually assessed. (C) 2019 Elsevier B.V. All rights reserved.
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