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DOI: 10.1055/s-2004-829595
Myasthenia Gravis: Management of Myasthenic Crisis and Perioperative Care
Publikationsverlauf
Publikationsdatum:
01. Juli 2004 (online)
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Myasthenic crisis may be defined as respiratory failure or delayed postoperative extubation for more than 24 hours resulting from myasthenic weakness. Myasthenic crisis results from weakness of upper airway muscles leading to obstruction and aspiration, weakness of respiratory muscles leading to reduced tidal volumes, or from weakness of both muscle groups. About one-fifth of patients with myasthenia gravis experience crisis, usually within the first year of illness. Over the last four decades, prognosis from myasthenic crisis has dramatically improved from a mortality rate of 75% to the current rate of less than 5%. Common precipitating factors for myasthenic crisis include respiratory infections, aspiration, sepsis, surgical procedures, rapid tapering of immune modulation, beginning treatment with corticosteroids, exposure to drugs that may increase myasthenic weakness, and pregnancy. Myasthenic crisis should not be fatal, as long as patients receive timely respiratory support and appropriate immunotherapy to reduce myasthenic weakness of the upper airway and respiratory muscles. Myasthenic patients with oropharyngeal or respiratory muscle weakness should receive preoperative plasma exchange or intravenous immunoglobulin therapy to a minimal level of weakness to prevent postoperative complications.
KEYWORDS
Myasthenia gravis - myasthenic crisis - respiratory failure
REFERENCES
- 1 Wilks S. On cerebritis, hysteria, and bulbar paralysis. Guys Hosp Rep. 1877; 22 7-55
- 2 Keesey J C. Myasthenia Gravis: An Illustrated History. Roseville, CA; Publishers Design Group 2002
- 3 Bedlack R S, Sanders D B. On the concept of myasthenic crisis. J Clin Neuromusc Dis. 2002; 4 40-42
- 4 Tether J E. Management of myasthenic and cholinergic crises. Am J Med. 1955; 19 740-742
- 5 Ashworth B, Hunter A R. Respiratory failure in myasthenia gravis. Proc R Soc Med. 1971; 64 489-490
- 6 Gracey D R, Divertie M B, Howard F M. Mechanical ventilation for respiratory failure in myasthenia gravis: two-year experience with 22 patients. Mayo Clin Proc. 1983; 58 597-602
- 7 Thomas C E, Mayer S A, Gungor Y et al.. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology. 1997; 48 1253-1260
- 8 Sellman M S, Mayer R F. Treatment of myasthenic crisis in late life. South Med J. 1985; 78 1208-1210
- 9 Donaldson D H, Ansher M, Horan S, Rutherford R B, Ringel S P. The relationship of age to outcome in myasthenia gravis. Neurology. 1990; 40 786-790
- 10 Oosterhuis H J. The natural course of myasthenia gravis: a long term follow up study. J Neurol Neurosurg Psychiatry. 1989; 52 1121-1127
- 11 Garrity E R. Respiratory failure due to disorders of the chest wall and respiratory muscles. In: MacDonnell KF, Fahey PJ, Segal MS Respiratory Intensive Care. Boston; Little Brown 1987: 312-320
- 12 Mier-Jedrzejowicz A K, Brophy C, Green M. Respiratory muscle function in myasthenia gravis. Am Rev Respir Dis. 1988; 138 867-873
- 13 Borel C O, Teitelbaum J S, Hanley D F. Ventilatory failure and carbon dioxide response in ventilatory failure due to myasthenia gravis and Guillain-Barre syndrome. Crit Care Med. 1993; 21 1717-1726
- 14 Putman M T, Wise R A. Myasthenia gravis and upper airway obstruction. Chest. 1996; 109 400-404
- 15 Cridge P B, Allegra J, Gerhard H. Myasthenic crisis presenting as isolated vocal cord paralysis. Am J Emerg Med. 2000; 18 232-233
- 16 Schmidt-Nowara W W, Marder E J, Feil P A. Respiratory failure in myasthenia gravis due to vocal cord paresis. Arch Neurol. 1984; 41 567-568
- 17 Rabinstein A A, Wijdicks E FM. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol. 2003; 23 97-104
- 18 Rieder P, Louis M, Jolliet P, Chevrolet J C. The repeated measurement of vital capacity is a poor predictor of the need for mechanical ventilation in myasthenia gravis. Intensive Care Med. 1995; 21 663-668
- 19 Juel V C, Bleck T P. Neuromuscular disorders in critical care. In: Shoemaker WC, Ayers SM, Grenvik ANA, Holbrook PR Textbook of Critical Care, 4th ed. New York; W.B. Saunders 2000: 1886-1894
- 20 Rabinstein A, Wijdicks E FM. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation. Neurology. 2002; 59 1647-1649
- 21 Mayer S A. Intensive care of the myasthenic patient. Neurology. 1997; 48(suppl 5) S70-S75
- 22 Varelas P N, Chua H C, Natterman J et al.. Ventilatory care in myasthenia gravis crisis: assessing the baseline adverse event rate. Crit Care Med. 2002; 30 2663-2668
- 23 Bosch E P, Subbiah B, Ross M A. Cholinergic crisis after conventional doses of anticholinesterase medications in chronic renal failure. Muscle Nerve. 1991; 14 1036-1037
- 24 Borel C O. Management of myasthenic crisis: continuous anticholinesterase infusions. Crit Care Med. 1993; 21 821-822
- 25 Pinching A J, Peters D K. Remission of myasthenia gravis following plasma exchange. Lancet. 1976; 2(8000) 1373-1376
- 26 Dau P C, Lindstrom J M, Cassel C K et al.. Plasmapheresis and immunosuppressive drug therapy in myasthenia gravis. N Engl J Med. 1977; 297 1134-1140
- 27 Kornfield P, Ambinder E P, Papatestas A E, Bender A N, Genkins G. Plasmapheresis in myasthenia gravis: controlled study [letter]. Lancet. 1979; 2 629
- 28 Antozzi C, Gemma M, Regi B et al.. A short plasma exchange protocol is effective in severe myasthenia gravis. J Neurol. 1991; 238 103-107
- 29 Gracey D R, Howard F M, Divertie M B. Plasmapheresis in the treatment of ventilator-dependent myasthenia gravis patients: report of four cases. Chest. 1984; 85 739-743
- 30 Qureshi A I, Choundry M A, Akbar M S et al.. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology. 1999; 52 629-632
- 31 Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Myasthenia Gravis Clinical Study Group. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Ann Neurol. 1997; 41 789-796
- 32 Stricker R B, Kwiatkowska B J, Habis J A, Kiprov D D. Myasthenic crisis: response to plasmapheresis following failure of intravenous gamma-globulin. Arch Neurol. 1993; 50 837-840
- 33 Huang C-S, Hsu H-S, Kao K-P, Huang M-H, Huang B-S. Intravenous immunoglobulin in the preparation of thymectomy for myasthenia gravis. Acta Neurol Scand. 2003; 108 136-138
- 34 Saltis L M, Martin B R, Traeger S M, Bonfiglio M F. Continuous infusion of pyridostigmine in the management of myasthenic crisis. Crit Care Med. 1993; 21 938-940
- 35 Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med. 1997; 25 1228-1235
- 36 Mayer S A, Thomas C E. Therapy of myasthenic crisis. Crit Care Med. 1998; 26 1136-1137
- 37 Howard J F. Adverse drug effects on neuromuscular transmission. Semin Neurol. 1990; 10 89-102
- 38 Younger D S, Braun N MT, Jaretzki A, Penn A S, Lovelace R E. Myasthenia gravis: determinants for independent ventilation after transsternal thymectomy. Neurology. 1984; 34 336-340
- 39 Jaretzki A, Aarli J A, Kaminski H J, Phillips L H, Sanders D B. Preoperative preparation of patients with myasthenia gravis forestalls postoperative respiratory complications after thymectomy. Ann Thorac Surg. 2003; 75 1068
- 40 Leventhal S R, Orkin F K, Hirsh R A. Prediction of the need for postoperative mechanical ventilation in myasthenia gravis. Anesthesiology. 1980; 53 26-30
- 41 d'Empaire G, Hoaglin D C, Perlo V P et al.. Effect of prethymectomy plasma exchange on postoperative respiratory function in myasthenia gravis. J Thorac Cardiovasc Surg. 1985; 89 592-596
- 42 Cumming W JK, Hudgson P. The role of plasma phoresis in preparing patients with myasthenia for thymectomy [abstract]. Muscle Nerve. 1986; 9 S155
- 43 Kirsch J R, Diringer M N, Borel C O et al.. Preoperative lumbar epidural morphine improves postoperative analgesia and ventilatory function after transsternal thymectomy in patients with myasthenia gravis. Crit Care Med. 1991; 19 1474-1479
Vern C JuelM.D.
P.O. Box 800394, University of Virginia Health System, Charlottesville, VA 22908-0394