Subscribe to RSS
DOI: 10.1055/s-0038-1676838
Acute Manifestations of Neuromuscular Disease
Publication History
Publication Date:
11 February 2019 (online)
Abstract
Neuromuscular emergencies may be defined as disorders or exacerbation of diseases of the peripheral nervous system that are rapidly progressive and potentially life-threatening. Such disorders can affect any level of the peripheral nervous system, from the muscle to the anterior horn cell. While their clinical manifestations may vary, severe morbidity and mortality is most frequently the result of neuromuscular respiratory failure. Some disorders, such as Guillain–Barré syndrome, provide the additional threat of severe, and potentially irreversible, nerve loss. Others, such as rhabdomyolysis and malignant hyperthermia, may produce serious medical complications. This article reviews neuromuscular emergencies by localization in the peripheral nervous system of the underlying disorder, as well as the identification and management of neuromuscular respiratory failure.
-
References
- 1 Rabinstein AA, Wijdicks EF. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol 2003; 23 (01) 97-104
- 2 Harik-Khan RI, Wise RA, Fozard JL. ; The Baltimore Longitudinal Study of Aging. Determinants of maximal inspiratory pressure. Am J Respir Crit Care Med 1998; 158 (5, Pt 1): 1459-1464
- 3 Elsheikh B, Arnold WD, Gharibshahi S, Reynolds J, Freimer M, Kissel JT. Correlation of single-breath count test and neck flexor muscle strength with spirometry in myasthenia gravis. Muscle Nerve 2016; 53 (01) 134-136
- 4 Luo F, Annane D, Orlikowski D. , et al. Invasive versus non-invasive ventilation for acute respiratory failure in neuromuscular disease and chest wall disorders. Cochrane Database Syst Rev 2017; 12: CD008380
- 5 Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol 2001; 58 (06) 893-898
- 6 Girard TD, Kress JP, Fuchs BD. , et al. Efficacy and safety of a paired sedation and ventilator weaning protocol for mechanically ventilated patients in intensive care (Awakening and Breathing Controlled trial): a randomised controlled trial. Lancet 2008; 371 (9607): 126-134
- 7 Ely EW, Baker AM, Dunagan DP. , et al. Effect on the duration of mechanical ventilation of identifying patients capable of breathing spontaneously. N Engl J Med 1996; 335 (25) 1864-1869
- 8 Ouellette DR, Patel S, Girard TD. , et al. Liberation from mechanical ventilation in critically ill adults: an official American College of Chest Physicians/American Thoracic Society Clinical Practice Guideline: inspiratory pressure augmentation during spontaneous breathing trials, protocols minimizing sedation, and noninvasive ventilation immediately after extubation. Chest 2017; 151 (01) 166-180
- 9 Schmidt GA, Girard TD, Kress JP. , et al; ATS/CHEST Ad Hoc Committee on Liberation from Mechanical Ventilation in Adults. Official executive summary of an American Thoracic Society/American College of Chest Physicians Clinical Practice Guideline: liberation from mechanical ventilation in critically ill adults. Am J Respir Crit Care Med 2017; 195 (01) 115-119
- 10 Bird SJ. Diagnosis of myasthenia gravis. Post TW. , ed. UpToDate. 2018 . Available at: https://www.uptodate.com/contents/diagnosis-of-myasthenia-gravis . Accessed July 30, 2018
- 11 Castleman B. The pathology of the thymus gland in myasthenia gravis. Ann N Y Acad Sci 1966; 135 (01) 496-505
- 12 Sanders DB, Wolfe GI, Benatar M. , et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016; 87 (04) 419-425
- 13 Wendell LC, Levine JM. Myasthenic crisis. Neurohospitalist 2011; 1 (01) 16-22
- 14 Alshekhlee A, Miles JD, Katirji B, Preston DC, Kaminski HJ. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals. Neurology 2009; 72 (18) 1548-1554
- 15 Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med 1997; 25 (07) 1228-1235
- 16 Rabinstein AA, Mueller-Kronast N. Risk of extubation failure in patients with myasthenic crisis. Neurocrit Care 2005; 3 (03) 213-215
- 17 O'Riordan JI, Miller DH, Mottershead JP, Hirsch NP, Howard RS. The management and outcome of patients with myasthenia gravis treated acutely in a neurological intensive care unit. Eur J Neurol 1998; 5 (02) 137-142
- 18 London ZN, Mundwiler A, Oral H, Gallagher GW. Nerve conduction studies are safe in patients with central venous catheters. Muscle Nerve 2017; 56 (02) 321-323
- 19 Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Predictors of extubation failure in myasthenic crisis. Arch Neurol 2008; 65 (07) 929-933
- 20 Thomas CE, Mayer SA, Gungor Y. , et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48 (05) 1253-1260
- 21 Wolfe GI, Kaminski HJ, Aban IB. , et al; MGTX Study Group. Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016; 375 (06) 511-522
- 22 Centers for Disease Control and Prevention (CDC). National Botulism Surveillance Summary. 2016 . Available at: https://www.cdc.gov/botulism/pdf/Botulism-2016-SUMMARY-508.pdf . Accessed on July 25, 2018
- 23 Barash JR, Arnon SS. A novel strain of Clostridium botulinum that produces type B and type H botulinum toxins. J Infect Dis 2014; 209 (02) 183-191
- 24 Rossetto O, Megighian A, Scorzeto M, Montecucco C. Botulinum neurotoxins. Toxicon 2013; 67: 31-36
- 25 Kumar R, Dhaliwal HP, Kukreja RV, Singh BR. The botulinum toxin as a therapeutic agent: molecular structure and mechanism of action in motor and sensory systems. Semin Neurol 2016; 36 (01) 10-19
- 26 Sobel J. Botulism. Clin Infect Dis 2005; 41 (08) 1167-1173
- 27 Chatham-Stephens K, Fleck-Derderian S, Johnson SD, Sobel J, Rao AK, Meaney-Delman D. Clinical features of foodborne and wound botulism: a systematic review of the literature, 1932-2015. Clin Infect Dis 2017; 66 (Suppl. 01) S11-S16
- 28 Rao AK, Lin NH, Griese SE, Chatham-Stephens K, Badell ML, Sobel J. Clinical criteria to trigger suspicion for botulism: an evidence-based tool to facilitate timely recognition of suspected cases during sporadic events and outbreaks. Clin Infect Dis 2017; 66 (Suppl. 01) S38-S42
- 29 O'Horo JC, Harper EP, El Rafei A. , et al. Efficacy of antitoxin therapy in treating patients with foodborne botulism: a systematic review and meta-analysis of cases, 1923-2016. Clin Infect Dis 2017; 66 (Suppl. 01) S43-S56
- 30 Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011; 36 (02) 123-133
- 31 Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology 2008; 70 (18) 1608-1613
- 32 Hadden RD, Cornblath DR, Hughes RA. , et al; Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Ann Neurol 1998; 44 (05) 780-788
- 33 Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012; 366 (24) 2294-2304
- 34 Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet 2016; 388 (10045): 717-727
- 35 Anandan C, Khuder SA, Koffman BM. Prevalence of autonomic dysfunction in hospitalized patients with Guillain-Barré syndrome. Muscle Nerve 2017; 56 (02) 331-333
- 36 Sharshar T, Chevret S, Bourdain F, Raphaël JC. ; French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Early predictors of mechanical ventilation in Guillain-Barré syndrome. Crit Care Med 2003; 31 (01) 278-283
- 37 Ogawara K, Kuwabara S, Mori M, Hattori T, Koga M, Yuki N. Axonal Guillain-Barré syndrome: relation to anti-ganglioside antibodies and Campylobacter jejuni infection in Japan. Ann Neurol 2000; 48 (04) 624-631
- 38 Ye Y, Wang K, Deng F, Xing Y. Electrophysiological subtypes and prognosis of Guillain-Barré syndrome in northeastern China. Muscle Nerve 2013; 47 (01) 68-71
- 39 Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain 2014; 137 (Pt 1): 33-43
- 40 Wong AH, Umapathi T, Nishimoto Y, Wang YZ, Chan YC, Yuki N. Cytoalbuminologic dissociation in Asian patients with Guillain-Barré and Miller Fisher syndromes. J Peripher Nerv Syst 2015; 20 (01) 47-51
- 41 Odaka M, Yuki N, Hirata K. Anti-GQ1b IgG antibody syndrome: clinical and immunological range. J Neurol Neurosurg Psychiatry 2001; 70 (01) 50-55
- 42 Chiba A, Kusunoki S, Obata H, Machinami R, Kanazawa I. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies. Neurology 1993; 43 (10) 1911-1917
- 43 Willison HJ, Veitch J, Paterson G, Kennedy PG. Miller Fisher syndrome is associated with serum antibodies to GQ1b ganglioside. J Neurol Neurosurg Psychiatry 1993; 56 (02) 204-206
- 44 Bertorini T, Halford H, Lawrence J, Vo D, Wassef M. Contrast-enhanced magnetic resonance imaging of the lumbosacral roots in the dysimmune inflammatory polyneuropathies. J Neuroimaging 1995; 5 (01) 9-15
- 45 Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2017; 2: CD001798
- 46 Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev 2014; (09) CD002063
- 47 Nance JR, Mammen AL. Diagnostic evaluation of rhabdomyolysis. Muscle Nerve 2015; 51 (06) 793-810
- 48 Melli G, Chaudhry V, Cornblath DR. Rhabdomyolysis: an evaluation of 475 hospitalized patients. Medicine (Baltimore) 2005; 84 (06) 377-385
- 49 Gabow PA, Kaehny WD, Kelleher SP. The spectrum of rhabdomyolysis. Medicine (Baltimore) 1982; 61 (03) 141-152
- 50 Zager RA. Combined mannitol and deferoxamine therapy for myohemoglobinuric renal injury and oxidant tubular stress. Mechanistic and therapeutic implications. J Clin Invest 1992; 90 (03) 711-719
- 51 Liu ZZ, Mathia S, Pahlitzsch T. , et al. Myoglobin facilitates angiotensin II-induced constriction of renal afferent arterioles. Am J Physiol Renal Physiol 2017; 312 (05) F908-F916
- 52 Llach F, Felsenfeld AJ, Haussler MR. The pathophysiology of altered calcium metabolism in rhabdomyolysis-induced acute renal failure. Interactions of parathyroid hormone, 25-hydroxycholecalciferol, and 1,25-dihydroxycholecalciferol. N Engl J Med 1981; 305 (03) 117-123
- 53 Akmal M, Bishop JE, Telfer N, Norman AW, Massry SG. Hypocalcemia and hypercalcemia in patients with rhabdomyolysis with and without acute renal failure. J Clin Endocrinol Metab 1986; 63 (01) 137-142
- 54 Scharman EJ, Troutman WG. Prevention of kidney injury following rhabdomyolysis: a systematic review. Ann Pharmacother 2013; 47 (01) 90-105
- 55 de Meijer AR, Fikkers BG, de Keijzer MH, van Engelen BG, Drenth JP. Serum creatine kinase as predictor of clinical course in rhabdomyolysis: a 5-year intensive care survey. Intensive Care Med 2003; 29 (07) 1121-1125
- 56 Mikkelsen TS, Toft P. Prognostic value, kinetics and effect of CVVHDF on serum of the myoglobin and creatine kinase in critically ill patients with rhabdomyolysis. Acta Anaesthesiol Scand 2005; 49 (06) 859-864
- 57 Wappler F. Malignant hyperthermia. Eur J Anaesthesiol 2001; 18 (10) 632-652
- 58 Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB. Clinical presentation, treatment, and complications of malignant hyperthermia in North America from 1987 to 2006. Anesth Analg 2010; 110 (02) 498-507
- 59 Litman RS, Griggs SM, Dowling JJ, Riazi S. Malignant hyperthermia susceptibility and related diseases. Anesthesiology 2018; 128 (01) 159-167
- 60 Riazi S, Larach MG, Hu C, Wijeysundera D, Massey C, Kraeva N. Malignant hyperthermia in Canada: characteristics of index anesthetics in 129 malignant hyperthermia susceptible probands. Anesth Analg 2014; 118 (02) 381-387
- 61 Schneiderbanger D, Johannsen S, Roewer N, Schuster F. Management of malignant hyperthermia: diagnosis and treatment. Ther Clin Risk Manag 2014; 10: 355-362
- 62 Paul-Pletzer K, Yamamoto T, Bhat MB. , et al. Identification of a dantrolene-binding sequence on the skeletal muscle ryanodine receptor. J Biol Chem 2002; 277 (38) 34918-34923
- 63 Suresh S, Forgie S, Robinson J. Non-polio enterovirus detection with acute flaccid paralysis: A systematic review. J Med Virol 2018; 90 (01) 3-7
- 64 Petersen LR, Brault AC, Nasci RS. West Nile virus: review of the literature. JAMA 2013; 310 (03) 308-315
- 65 Greninger AL, Naccache SN, Messacar K. , et al. A novel outbreak enterovirus D68 strain associated with acute flaccid myelitis cases in the USA (2012-14): a retrospective cohort study. Lancet Infect Dis 2015; 15 (06) 671-682
- 66 Ooi MH, Wong SC, Lewthwaite P, Cardosa MJ, Solomon T. Clinical features, diagnosis, and management of enterovirus 71. Lancet Neurol 2010; 9 (11) 1097-1105
- 67 Howard RS. Poliomyelitis and the postpolio syndrome. BMJ 2005; 330 (7503): 1314-1318
- 68 Davis LE, DeBiasi R, Goade DE. , et al. West Nile virus neuroinvasive disease. Ann Neurol 2006; 60 (03) 286-300
- 69 Tyler KL, Pape J, Goody RJ, Corkill M, Kleinschmidt-DeMasters BK. CSF findings in 250 patients with serologically confirmed West Nile virus meningitis and encephalitis. Neurology 2006; 66 (03) 361-365
- 70 Hart Jr J, Tillman G, Kraut MA. , et al; NIAID Collaborative Antiviral Study Group West Nile Virus 210 Protocol Team. West Nile virus neuroinvasive disease: neurological manifestations and prospective longitudinal outcomes. BMC Infect Dis 2014; 14: 248
- 71 Farrar JJ, Yen LM, Cook T. , et al. Tetanus. J Neurol Neurosurg Psychiatry 2000; 69 (03) 292-301
- 72 Lalli G, Bohnert S, Deinhardt K, Verastegui C, Schiavo G. The journey of tetanus and botulinum neurotoxins in neurons. Trends Microbiol 2003; 11 (09) 431-437
- 73 Centers for Disease Control and Prevention (CDC). Tetanus surveillance --- United States, 2001-2008. MMWR Morb Mortal Wkly Rep 2011; 60 (12) 365-369
- 74 Afshar M, Raju M, Ansell D, Bleck TP. Narrative review: tetanus-a health threat after natural disasters in developing countries. Ann Intern Med 2011; 154 (05) 329-335
- 75 Saltoglu N, Tasova Y, Midikli D, Burgut R, Dündar IH. Prognostic factors affecting deaths from adult tetanus. Clin Microbiol Infect 2004; 10 (03) 229-233
- 76 Bunch TJ, Thalji MK, Pellikka PA, Aksamit TR. Respiratory failure in tetanus: case report and review of a 25-year experience. Chest 2002; 122 (04) 1488-1492
- 77 Thwaites CL, Yen LM, Cordon SM. , et al. Urinary catecholamine excretion in tetanus. Anaesthesia 2006; 61 (04) 355-359
- 78 Thwaites CL, Beeching NJ, Newton CR. Maternal and neonatal tetanus. Lancet 2015; 385 (9965): 362-370
- 79 Campbell JI, Lam TM, Huynh TL. , et al. Microbiologic characterization and antimicrobial susceptibility of Clostridium tetani isolated from wounds of patients with clinically diagnosed tetanus. Am J Trop Med Hyg 2009; 80 (05) 827-831
- 80 Ganesh Kumar AV, Kothari VM, Krishnan A, Karnad DR. Benzathine penicillin, metronidazole and benzyl penicillin in the treatment of tetanus: a randomized, controlled trial. Ann Trop Med Parasitol 2004; 98 (01) 59-63
- 81 Ahmadsyah I, Salim A. Treatment of tetanus: an open study to compare the efficacy of procaine penicillin and metronidazole. Br Med J (Clin Res Ed) 1985; 291 (6496): 648-650
- 82 Thwaites CL, Yen LM, Loan HT. , et al. Magnesium sulphate for treatment of severe tetanus: a randomised controlled trial. Lancet 2006; 368 (9545): 1436-1443