Abstract
Respiratory failure is the most common cause of morbidity and mortality in patients with progressive neuromuscular diseases (NMDs) [1–4]. The wide variety of NMDs that can affect respiratory function are listed in Table 32.1. NMDs are often complicated by progressive involvement of the respiratory muscles and can lead to both chronic and acute respiratory failure (ARF). Reduced inspiratory muscle strength can result in ineffective alveolar ventilation, and weakness of expiratory muscles can lead to inadequate clearance of airway secretions. Thus, these conditions can cause chronic respiratory failure as well as potentially life-threatening problems [5–10].
Keywords
- Amyotrophic Lateral Sclerosis
- Duchenne Muscular Dystrophy
- Acute Respiratory Failure
- Spinal Muscular Atrophy
- Spontaneous Breathing Trial
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.
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References
Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care. 2006;51(9):1016–21.
Hill NS. Neuromuscular disease in respiratory and critical care medicine. Respir Care. 2006;51(9):1065–71.
Perrin C, Unterborn JN, Ambrosio CD, et al. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve. 2004;29(1):5–27.
Calvert LD, McKeever TM, Kinnear WJ, et al. Trends in survival from muscular dystrophy in England and Wales and impact on respiratory services. Respir Med. 2006;100(6):1058–63.
Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing anesthesia or sedation. Chest. 2007;132(6):1977–86.
Wang CH, Finkel RS, Bertini ES, et al. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027–49.
Hull J, Aniapravan R, Chan E, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012;67:i1–40.
Bushby K, Finkel R, Birnkrant DJ, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurol. 2010;9(2):177–89.
Wang CH, Bonnemann CG, Rutkowski A, et al. Consensus statement on standard of care for congenital muscular dystrophies. J Child Neurol. 2010;25(12):1559–81.
Racca F, Del Sorbo L, Mongini T, et al. Respiratory management of acute respiratory failure in neuromuscular diseases. Minerva Anestesiol. 2010;76(1):51–62.
Benditt JO. Management of pulmonary complications in neuromuscular disease. Phys Med Rehabil Clin N Am. 1998;9(1):167–85.
Bergofsky EH. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis. 1979;119(4):643–69.
De Troyer A, Borenstein S, Cordier R. Analysis of lung volume restriction in patients with respiratory muscle weakness. Thorax. 1980;35(8):603–10.
Papastamelos C, Panitch HB, Allen JL. Chest wall compliance in infants and children with neuromuscular disease. Am J Respir Crit Care Med. 1996;154(4):1045–8.
Poponick JM, Jacobs I, Supinski G, Di Marco AF. Effect of upper respiratory tract infection in patients with neuromuscular disease. Am J Respir Crit Care Med. 1997;156(2 Pt 1):659–64.
Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest. 2000;118(5):1390–6.
Bach JR, Rajaraman R, Ballanger F, et al. Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use vs oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil. 1998;77(1):8–19.
Simonds AK. Pneumothorax: an important complication of non-invasive ventilation in neuromuscular disease. Neuromuscul Disord. 2004;14(6):351–2.
Schmidt-Nowara WW, Altman AR. Atelectasis and neuromuscular respiratory failure. Chest. 1984;85(6):792–5.
Oppenheimer EA. Treating respiratory failure in ALS: the details are becoming clearer. J Neurol Sci. 2003;209:111–3.
Goodwin FC, Muntoni F. Cardiac involvement in muscular dystrophies: molecular mechanisms. Muscle Nerve. 2005;32(5):577–88.
Sveen ML, Thune JJ, Køber L, et al. Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy. Arch Neurol. 2008;65(9):1196–201.
Finder JD, Birnkrant D, Carl J, Farber HJ, Gozal D, Iannaccone ST, Kovesi T, Kravitz RM, Panitch H, Schramm C, Schroth M, Sharma G, Sievers L, Silvestri JM, Sterni L, American Thoracic Society. ATS consensus statement respiratory care of the patient with Duchenne muscular dystrophy. Am J Respir Crit Care Med. 2004;170(4):456–65.
Racca F, Mongini T, Wolfler A, et al. Recommendations for anesthesia and perioperative management of patients with neuromuscular disorders. Minerva Anestesiol. 2013;79:419–33.
Corrado A, Gorini M, De Paola E. Alternative techniques for managing acute neuromuscular respiratory failure. Semin Neurol. 1995;15(1):84–9.
Servera E, Sancho J, Zafra MJ, et al. Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil. 2005;84:851–7.
Vianello A, Bevilacqua M, Arcaro G, et al. Non-invasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation. Intensive Care Med. 2000;26(4):384–90.
Vianello A, Corrado A, Arcaro G, et al. Mechanical insufflation–exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil. 2005;84(2):83–8.
Gomez-Merino E, Bach JR. Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing. Am J Phys Med Rehabil. 2002;81(6):411–5.
Sancho J, Servera E. Noninvasive ventilation for patients with neuromuscular disease and acute respiratory failure. Chest. 2008;133:314–5.
Bach JR, Niranjan V, Weaver B. Spinal muscular atrophy type 1: a noninvasive respiratory management approach. Chest. 2000;117(4):1100–5.
Piastra M, Antonelli M, Caresta E, et al. Noninvasive ventilation in childhood acute neuromuscular respiratory failure: a pilot study. Respiration. 2006;73(6):791–8.
Racca F, Appendini L, Berta G, et al. Helmet ventilation for acute respiratory failure and nasal skin breakdown in neuromuscular disorders. Case Report Anesth Analg. (in press). Anesth Analg. 2009;109(1):164–7.
Wang CH, Finkel RS, Bertini ES, et al.; Participants of the International Conference on SMA Standard of Care. Consensus statement for standard of care in spinal muscular atrophy. J Child Neurol. 2007;22(8):1027–49.
Wallgren-Pettersson C, Bushby K, Mellies U, et al. 117th ENMC workshop: ventilatory support in congenital neuromuscular disorders – congenital myopathies, congenital muscular dystrophies, congenital myotonic dystrophy and SMA (II) 4–6 April 2003, Naarden, The Netherlands. Neuromuscul Disord. 2004;14(1):56–69.
British Thoracic Society Standards of Care Committee. Non-invasive ventilation in acute respiratory failure. Thorax. 2002;57(3):192–211.
Mehta S, Hill NS. State of the art: noninvasive ventilation. Am J Respir Crit Care Med. 2001;163:540–77.
Bradley MD, Orrell RW, Clarke J, et al. Outcome of ventilatory support for acute respiratory failure in motor neuron disease. J Neurol Neurosurg Psychiatry. 2002;72(6):752–6.
Bach JR, Bianchi C, Aufiero E. Oximetry and indications for tracheotomy for amyotrophic lateral sclerosis. Chest. 2004;126(5):1502–7.
Vianello A, Savoia F, Pipitone E, et al. “Hospital at home” for neuromuscular disease patients with respiratory tract infection: a pilot study. Respir Care. 2013;58(12):2061–8.
Vicken W, Elleker G, Cosio MG. Detection of upper airway muscle involvement in neuromuscular disorders using the flow-volume loop. Chest. 1986;90(1):52–7.
Lechtzin N, Wienner CM, Clawson L, et al. Hospitalization in amyotrophic lateral sclerosis: causes, costs, and outcomes. Neurology. 2001;56(6):753–7.
Polkey MI, Lyall RA, Green M, et al. Expiratory muscle function in amyotrophic lateral sclerosis. Am J Respir Crit Care Med. 1998;158(3):734–41.
Padman R, Lawless S, Von Nessen S. Use of BiPAP by nasal mask in the treatment of respiratory insufficiency in pediatric patients: preliminary investigation. Pediatr Pulmonol. 1994;17(2):119–23.
Sancho J, Servera E, Dıaz J, Marin J. Efficacy of mechanical in-exsufflation in medically stable patients with amyotrophic lateral sclerosis. Chest. 2004;125(4):1400–5.
Niranjan V, Bach JR. Noninvasive management of pediatric neuromuscular ventilatory failure. Crit Care Med. 1998;26(12):2061–5.
Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest. 1997;112(4):1024–8.
Bach JR. Update and perspective on noninvasive respiratory muscle aids: Part 2: the expiratory aids. Chest. 1994;105(5):1538–44.
Miske LJ, Hickey EM, Kolb SM, et al. Use of the mechanical inexsufflator in pediatric patients with neuromuscular disease and impaired cough. Chest. 2004;125:1406–12.
Chatwin M, Ross E, Hart N, et al. Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J. 2003;21(3):502–8.
Fauroux B, Guillemot N, Aubertin G, et al. Physiologic benefits of mechanical insufflation-exsufflation in children with neuromuscular diseases. Chest. 2008;133:161–8.
Bach JR, Goncalves MR, Hamdani I, et al. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest. 2010;137(5):1033–9.
Racca F, Del Sorbo L, Capello E, et al. Neuromuscular patients as candidates for non invasive ventilation during the weaning process. Minerva Anestesiol. 2012;78:391.
Simonds AK. Streamlining weaning: protocols and weaning units. Thorax. 2005;60:175–82.
Epstein SK. Extubation failure: an outcome to be avoided. Crit Care. 2004;8:310–2.
Vianello A, Arcaro G, Braccioni F, et al. Prevention of extubation failure in high-risk neuromuscular disease patients. J Crit Care. 2011;26(5):517–24.
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Racca, F., Robba, C., Dusio, M.P. (2016). Noninvasive Mechanical Ventilation in Patients with Neuromuscular Disease. In: Esquinas, A. (eds) Noninvasive Mechanical Ventilation and Difficult Weaning in Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-04259-6_32
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