title: Nocturnal respiratory failure as an indication of noninvasive ventilation in the patient with neuromuscular disease.
abstract: 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.
authors.affiliation: Sleep Laboratory, University of Freiburg Medical Center, Germany.
authors.forename: G M
authors.initials: GM
authors.lastname: Barthlen
mesh_terms: D018759:Adrenergic Uptake Inhibitors / Q000627:therapeutic use; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications; D003490:Cyanosis / Q000209:etiology; D006261:Headache / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000209:etiology; D007385:Intermittent Positive-Pressure Ventilation; D009119:Muscle Contraction / Q000502:physiology; D009136:Muscular Dystrophies / Q000150:complications; D009157:Myasthenia Gravis / Q000150:complications; D009223:Myotonic Dystrophy / Q000150:complications; D009468:Neuromuscular Diseases / Q000150:complications* / Q000503:physiopathology; D010102:Oxygen Inhalation Therapy; D011086:Polycythemia / Q000209:etiology; D017286:Polysomnography; D011175:Positive-Pressure Respiration / Q000379:methods; D016262:Postpoliomyelitis Syndrome / Q000150:complications; D011530:Protriptyline / Q000627:therapeutic use; D011595:Psychomotor Agitation / Q000209:etiology; D012119:Respiration; D012131:Respiratory Insufficiency / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D012890:Sleep / Q000502:physiology; D012891:Sleep Apnea Syndromes / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology / Q000628:therapy; D007319:Sleep Initiation and Maintenance Disorders / Q000209:etiology; D012894:Sleep Stages; D012913:Snoring / Q000209:etiology; D014139:Tracheostomy
chemical_list: D018759:Adrenergic Uptake Inhibitors; D011530:Protriptyline