Voulgaris Athanasios, Antoniadou Maria, Agrafiotis Michalis, Steiropoulos Paschalis
MSc Program in Sleep Medicine, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
Department of Pneumonology, Medical School, Democritus University of Thrace, Alexandroupolis, Greece.
Pulm Med. 2019 Dec 26;2019:2734054. doi: 10.1155/2019/2734054. eCollection 2019.
Respiratory muscle weakness is a major cause of morbidity and mortality in patients with neuromuscular diseases (NMDs). Respiratory involvement in NMDs can manifest broadly, ranging from milder insufficiency that may affect only sleep initially to severe insufficiency that can be life threatening. Patients with neuromuscular diseases exhibit very often sleep-disordered breathing, which is frequently overlooked until symptoms become more severe leading to irreversible respiratory failure necessitating noninvasive ventilation (NIV) or even tracheostomy. Close monitoring of respiratory function and sleep evaluation is currently the standard of care. Early recognition of sleep disturbances and initiation of NIV can improve the quality of life and prolong survival. This review discusses the respiratory impairment during sleep in patients with NMDs, the diagnostic tools available for early recognition of sleep-disordered breathing and the therapeutic options available for overall respiratory management of patients with NMDs.
呼吸肌无力是神经肌肉疾病（NMDs）患者发病和死亡的主要原因。NMDs中的呼吸受累表现广泛，从最初可能仅影响睡眠的较轻功能不全到可能危及生命的严重功能不全。神经肌肉疾病患者经常出现睡眠呼吸紊乱，在症状变得更严重导致不可逆的呼吸衰竭而需要无创通气（NIV）甚至气管切开之前，这种情况常常被忽视。目前，密切监测呼吸功能和进行睡眠评估是标准的治疗方法。早期识别睡眠障碍并开始使用NIV可以提高生活质量并延长生存期。本文综述了NMDs患者睡眠期间的呼吸功能损害、可用于早期识别睡眠呼吸紊乱的诊断工具以及可用于NMDs患者整体呼吸管理的治疗选择。
