Barthlen G M
Sleep Laboratory, University of Freiburg Medical Center, Germany.
Respiration. 1997;64 Suppl 1:35-8. doi: 10.1159/000196734.
Patients with neuromuscular disease may suffer from nocturnal respiratory failure despite normal daytime respiratory function. The physiological reduction in muscle tone during sleep may be life-threatening in a patient with impaired muscle strength. Nocturnal respiratory failure may occur in patients with the postpolio syndrome, amyotrophic lateral sclerosis, myasthenia gravis, myotonic dystrophy, and muscular dystrophy. Diagnosis of obstructive, central and mixed apneas, hypopneas, and hypoventilation is best made using polysomnography. Therapeutic options include noninvasive ventilation such as continuous positive airway pressure, bilevel positive airway pressure, intermittent positive pressure ventilation and, rarely, tracheostomy, oxygen, or protriptyline. Evaluation by a sleep specialist should be initiated in any neuromuscular patient with nocturnal symptoms such as air hunger, intermittent snoring or breathing, orthopnea, cyanosis, restlessness, and insomnia. Daytime symptoms may include morning drowsiness, headaches and excessive daytime sleepiness. Polycythemia, hypertension, and signs of heart failure may also be seen. Effective treatment is available, and may improve the quality of life, and possibly increase survival.
尽管白天呼吸功能正常，但患有神经肌肉疾病的患者可能会出现夜间呼吸衰竭。对于肌肉力量受损的患者，睡眠期间肌肉张力的生理性降低可能会危及生命。夜间呼吸衰竭可能发生在患有小儿麻痹后遗症、肌萎缩侧索硬化症、重症肌无力、强直性肌营养不良和肌肉营养不良的患者中。使用多导睡眠图最有助于诊断阻塞性、中枢性和混合性呼吸暂停、呼吸浅慢及通气不足。治疗选择包括无创通气，如持续气道正压通气、双水平气道正压通气、间歇正压通气，以及很少使用的气管造口术、吸氧或普罗替林。对于任何有夜间症状（如气促、间歇性打鼾或呼吸、端坐呼吸、发绀、烦躁不安和失眠）的神经肌肉疾病患者，都应开始由睡眠专家进行评估。白天症状可能包括晨起嗜睡、头痛和日间过度嗜睡。还可能出现红细胞增多症、高血压和心力衰竭体征。有效的治疗方法是存在的，并且可能改善生活质量，并有可能延长生存期。
