Abstract
Assessing where a patient is along the spectrum of neuromuscular respiratory failure requires knowledge of respiratory physiology and pathophysiology, the evolution of clinical features, and knowledge of how to interpret diagnostic studies in the context of their limitations. Ultimately, this information can be employed to discern or predict the need for ICU admission and ventilatory support. Of equal importance is a thorough understanding of the most common causes of neuromuscular respiratory failure, how to diagnose them, and their associated complications and treatments. This chapter informs the reader how to approach and manage the patient with limb, bulbar, and respiratory weakness and equips them with the tools to diagnose and treat the most common causes of primary and secondary neuromuscular weakness.
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References
Wijdicks EFM. The neurology of acutely failing respiratory mechanics. Ann Neurol. 2017;81:485–94.
Wijdicks EFM. Management of acute neuromuscular disorders. Handb Clin Neurol. 2017;140(3):229–37.
Greene-Chandos D, Torbey M. Critical care of neuromuscular disorders. Continuum. 2018;24(6):1753–75.
Howard RS. Respiratory failure because of neuromuscular disease. Curr Opin Neurol. 2016;29(5):592–601.
Irfan M, Selim B, Rabinstein AA, St. Louis EK. Neuromuscular disorders and sleep in critically ill patients. Crit Care Clin. 2015;31(3):533–50.
Cabrera Serrano M, Rabinstein AA. Usefulness of pulmonary function tests and blood gases in acute neuromuscular respiratory failure. Eur J Neurol. 2012;19(3):452–6.
Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barre syndrome. Arch Neurol. 2001;58(6):893–8.
Walterspacher S, Kirchberger A, Lambeck J, et al. Respiratory muscle assessment in acute Guillain-Barre syndrome. Lung. 2016;194:821–8.
Kramer CL, McCullough M, Wijdicks EF. Teaching video neuroimages: how to unmask respiratory strength confounded by facial diplegia. Neurology. 2015;84:e57–8.
Boon AJ, O’Gorman C. Ultrasound in the assessment of respiration. J Clin Neurophysiol. 2016;33:112–9.
Caulfield AF, Flower O, Pineda JA, Uddin S. Emergency neurological life support: acute non-traumatic weakness. Neurocrit Care. 2017;27(Suppl 1):29–50. https://doi.org/10.1007/s12028-017-0450-3.
Walgaard C, Lingsma HF, van Doorn PA, et al. Tracheostomy or not: prediction of prolonged mechanical ventilation in Guillain-Barre syndrome. Neurocrit Care. 2017;26:6–13.
Lawn ND, Wijdicks EF. Post-intubation pulmonary function test in Guillain-Barre syndrome. Muscle Nerve. 2000;23:613–6.
Cabrera Serrano M, Rabinstein AA. Causes and outcomes of acute neuromuscular respiratory failure. Arch Neurol. 2010;67(9):1089–94.
Yuki N, Hartung HP. Guillain-Barre syndrome. N Engl J Med. 2012;366:2294–304.
Dos Santos T, Rodriguez A, Almiron M, et al. Zika virus and the Guillain-Barre syndrome - case series from seven countries. N Engl J Med. 2016;375:1598–601.
Gold CA, Josephson SA. Anticipating the challenges of Zika virus and the incidence of Guillain-Barre syndrome. JAMA Neurol. 2016;73:905–6.
van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barre syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10:469–82.
Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barre syndrome. Ann Neurol. 2010;67:781–7.
Zaeem Z, Siddiqi ZA, Zochodne DW. Autonomic involvement in Guillain-Barre syndrome: an update. Clin Auton Res. 2018;29(3):289–99.
Vucic S, Cairns KD, Black KR, Chong PS, Cros D. Neurophysiologic findings in early acute inflammatory demyelinating polyradiculoneuropathy. Clin Neurophysiol. 2004;115:2329–35.
Kokubun N, Nishibayashi M, Uncini A, Odaka M, Hirata K, Yuki N. Conduction block in acute motor axonal neuropathy. Brain. 2010;133:2897–908.
Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2014;(7):CD002063.
Raphael JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2012;(2):CD001798.
Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barre syndrome and validation of Brighton criteria. Brain. 2014;137:33–43.
van den Berg B, Bunschoten C, van Doorn PA, Jacobs BC. Mortality in Guillain-Barre syndrome. Neurology. 2013;80:1650–4.
Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC. Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology. 2011;76:968–75.
Drachman DB. Myasthenia Gravis. Semin Neurol. 2016;36:419–24.
Nicolle MW. Myasthenia Gravis and Lambert-Eaton Myasthenic syndrome. Continuum (Minneap Minn). 2016;22:1978–2005.
Skeie GO, Apostolski S, Evoli A, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol. 2010;17:893–902.
Gilchrist JM, Massey JM, Sanders DB. Single fiber EMG and repetitive stimulation of the same muscle in myasthenia gravis. Muscle Nerve. 1994;17:171–5.
Gajdos P, Chevret S, Toyka KV. Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev. 2012;12:CD002277.
Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group. Ann Neurol. 1997;41:789–96.
Mandawat A, Kaminski HJ, Cutter G, Katirji B, Alshekhlee A. Comparative analysis of therapeutic options used for myasthenia gravis. Ann Neurol. 2010;68:797–805.
Iorio R, Damato V, Alboini PE, Evoli A. Efficacy and safety of rituximab for myasthenia gravis: a systematic review and meta-analysis. J Neurol. 2015;262:1115–9.
Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Noninvasive ventilation in myasthenic crisis. Arch Neurol. 2008;65:54–8.
Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Predictors of extubation failure in myasthenic crisis. Arch Neurol. 2008;65:929–33.
Rabinstein AA, Mueller-Kronast N. Risk of extubation failure in patients with myasthenic crisis. Neurocrit Care. 2005;3:213–5.
Kalita J, Kohat AK, Misra UK. Predictors of outcome of myasthenic crisis. Neurol Sci. 2014;35:1109–14.
Hermans G, Van den Berghe G. Clinical review: intensive care unit acquired weakness. Crit Care. 2015;19:274.
Jolley SE, Bunnell AE, Hough CL. ICU-acquired weakness. Chest. 2016;150:1129–40.
Kress JP, Hall JB. ICU-acquired weakness and recovery from critical illness. N Engl J Med. 2014;370:1626–35.
Kramer CL. ICU-acquired weakness. Neurol Clin. 2017;35(4):723–36. https://doi.org/10.1016/j.ncl.2017.06.008.
Batt J, dos Santos CC, Cameron JI, Herridge MS. Intensive care unit-acquired weakness: clinical phenotypes and molecular mechanisms. Am J Respir Crit Care Med. 2013;187:238–46.
Jung B, Moury PH, Mahul M, et al. Diaphragmatic dysfunction in patients with ICU-acquired weakness and its impact on extubation failure. Intensive Care Med. 2016;42:853–61.
Kramer CL, Boon AJ, Harper CM, Goodman BP. Compound muscle action potential duration in critical illness neuromyopathy. Muscle Nerve. 2018;57(3):395–400. https://doi.org/10.1002/mus.25732.
Hermans G, De Jonghe B, Bruyninckx F, Van den Berghe G. Interventions for preventing critical illness polyneuropathy and critical illness myopathy. Cochrane Database Syst Rev. 2014:CD006832.
Kayambu G, Boots R, Paratz J. Physical therapy for the critically ill in the ICU: a systematic review and meta-analysis. Crit Care Med. 2013;41:1543–54.
Burtin C, Clerckx B, Robbeets C, et al. Early exercise in critically ill patients enhances short-term functional recovery. Crit Care Med. 2009;37:2499–505.
Kho ME, Truong AD, Zanni JM, et al. Neuromuscular electrical stimulation in mechanically ventilated patients: a randomized, sham-controlled pilot trial with blinded outcome assessment. J Crit Care. 2015;30:32–9.
Jones S, Man WD, Gao W, Higginson IJ, Wilcock A, Maddocks M. Neuromuscular electrical stimulation for muscle weakness in adults with advanced disease. Cochrane Database Syst Rev. 2016;10:CD009419.
Hermans G, Van Mechelen H, Clerckx B, et al. Acute outcomes and 1-year mortality of intensive care unit-acquired weakness. A cohort study and propensity-matched analysis. Am J Respir Crit Care Med. 2014;190:410–20.
Sharshar T, Bastuji-Garin S, Stevens RD, et al. Presence and severity of intensive care unit-acquired paresis at time of awakening are associated with increased intensive care unit and hospital mortality. Crit Care Med. 2009;37:3047–53.
Wieske L, Dettling-Ihnenfeldt DS, Verhamme C, et al. Impact of ICU-acquired weakness on post-ICU physical functioning: a follow-up study. Crit Care. 2015;19:196.
Harvey MA, Davidson JE. Postintensive care syndrome: right care, right now…and later. Crit Care Med. 2016;44:381–5.
Sacanella E, Perez-Castejon JM, Nicolas JM, et al. Functional status and quality of life 12 months after discharge from a medical ICU in healthy elderly patients: a prospective observational study. Crit Care. 2011;15:R105.
Guarneri B, Bertolini G, Latronico N. Long-term outcome in patients with critical illness myopathy or neuropathy: the Italian multicentre CRIMYNE study. J Neurol Neurosurg Psychiatry. 2008;79:838–41.
Rabinstein AA. Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid. Curr Opin Crit Care. 2016;22(2):94–9.
Wijdicks EF, Henderson RD, McClelland RL. Emergency intubation for respiratory failure in Guillain-Barre syndrome. Arch Neurol. 2003;60:947–8.
Wijdicks EF, Roy TK. BiPAP in early Guillain-Barre syndrome may fail. Can J Neurol Sci. 2006;33:105–6.
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Plasma exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Lancet. 1997;349:225–30.
van Koningsveld R, Schmitz PI, Meche FG, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barre syndrome: randomised trial. Lancet. 2004;363:192–6.
Barth D, Nabavi Nouri M, Ng E, Nwe P, Bril V. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology. 2011;76:2017–23.
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Kramer, C.L., Rabinstein, A.A. (2020). Neuromuscular Disease in the ICU. In: Hyzy, R.C., McSparron, J. (eds) Evidence-Based Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-030-26710-0_44
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DOI: https://doi.org/10.1007/978-3-030-26710-0_44
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