Anesthesia Issues in the Perioperative Management of Myasthenia Gravis

Myasthenia gravis (MG) is an autoimmune disease of the neuromuscular junction. Current anesthetic practice may require the use of neuromuscular blocking (NMB) drugs that act at this junction to facilitate control of the airway and allow procedures to be performed on a motionless MG patient. This competes with the goals of rapid emergence and recapture of preoperative muscle strength following anesthesia. In particular, avoiding prolonged periods of postoperative mechanical ventilation is a paramount concern of patients, families, and physicians. Standard anesthetic agents and, if needed, judiciously titrated NMB drugs generally allow safe emergence and immediate extubation for most low-risk MG patients. If necessary, postoperative mechanical ventilation is accomplished with the use of specialized monitoring devices that help monitor awareness and depth of sedation. Currently used intravenous sedatives allow titrated depth of sedation and rapid emergence when extubation is appropriate. Communication to the patient, family, and other caregivers of the goals of the anesthetic plan and of the patient's evolving status are also very important duties of the anesthesiologist.

Neuromuscular blocking drug - succinylcholine - nondepolarizing muscle relaxant - myasthenia gravis - mechanical ventilation - anticholinesterase

REFERENCES

• 1 Drachman D B. Myasthenia gravis.  N Engl J Med. 1994;  330 1797-1810
• 2 Eisenkraft J B, Book W J, Mann S M et al.. Resistance to succinylcholine in myasthenia gravis: a dose-response study.  Anesthesiology. 1988;  69 760-763
• 3 Mann R, Blobner M, Jelen-Esselborn S et al.. Preanesthetic train-of-four fade predicts the atracurium requirement of myasthenia gravis patients.  Anesthesiology. 2000;  93 346-350
• 4 Leventhal R, Orkin F, Hirsch R. Prediction of the need for postoperative mechanical ventilation in myasthenia gravis.  Anesthesiology. 1980;  53 26-30
• 5 Vareles P N, Chua H C, Natterman J. Ventilatory care in myasthenia gravis crisis: assessing the baseline adverse event rate.  Crit Care Med. 2002;  30 2663-2668
• 6 Lucchi M, Mussi A, Ricciardi R et al.. Thymectomy in ocular myasthenia gravis.  J Thorac Cardiovasc Surg. 2003;  125 740-741
• 7 Evoli A, Batocchi A P, Minisci C et al.. Clinical characteristics and prognosis of myasthenia gravis in older people.  J Am Geriatr Soc. 2000;  48 1442-1448
• 8 Nakagawa K, Asamura H, Matsuno Y et al.. Thymoma: a clinicopathologic study based on the new World Health Organization classification.  J Thorac Cardiovasc Surg. 2003;  126 1134-1140
• 9 Poulas K, Tsibri E, Kokla A et al.. Epidemiology of seropositive myasthenia gravis in Greece.  J Neurol Neurosurg Psychiatry. 2001;  71 352-356
• 10 Chitnis T, Khoury S J. Immunologic neuromuscular disorders.  J Allergy Clin Immunol. 2003;  111 S659-S668
• 11 Carding P. Voice pathology in the United Kingdom.  BMJ. 2003;  327 514-515
• 12 Fried M J, Protheroe T. d-Penicillamine induced myasthenia gravis: its relevance for the anesthetist.  Br J Anaesth. 1986;  58 1191-1193
• 13 Calhoun R F, Ritter J H, Guthrie T J et al.. Results of transcervical thymectomy for myasthenia gravis in 100 consecutive patients.  Ann Surg. 1999;  230 555-561
• 14 Hart N, Simonds A K. The pulmonary physician in critical care. Illustrative case 4: neuromusculoskeletal disorders.  Thorax. 2003;  58 547-549
• 15 Wittbrodt E T. Drugs and myasthenia gravis: an update.  Arch Intern Med. 1997;  157 399-408
• 16 Kokontis L, Gutmann L. Current treatment of neuromuscular diseases.  Arch Neurol. 2000;  57 939-943
• 17 Kupersmith M J, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis.  Arch Neurol. 2003;  60 243-248
• 18 Miller R G, Milner-Brown H S, Mirka A. Prednisone-induced worsening of neuromuscular function in myasthenia gravis.  Neurology. 1986;  36 729-732
• 19 Ciafaloni E, Nikhar N K, Massey J M et al.. Retrospective analysis of the use of cyclosporine in myasthenia gravis.  Neurology. 2000;  55 448-450
• 20 Meriggioli M N, Ciafaloni E, Al-Hayk K A et al.. Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability.  Neurology. 2003;  61 1438-1440
• 21 Bathon J M, Martin J W, Fleishman R M et al.. A comparison of etanercept and methotrexate in patients with early rheumatoid arthritis.  N Engl J Med. 2000;  343 1586-1593
• 22 Dalakas M C. Mechanisms of action of IVIg and therapeutic considerations in the treatment of acute and chronic demyelinating neuropathies.  Neurology. 2002;  59 S13-S21
• 23 Wylam M E, Anderson P M, Kuntz N L et al.. Successful treatment of refractory myasthenia gravis using rituximab: a pediatric case report.  J Pediatr. 2003;  143 674-677
• 24 Newsom-Davis J, Vincent A, Wilson S G et al.. Long term effects of repeated plasma exchange in myasthenia gravis.  Lancet. 1979;  1 464-468
• 25 Gronset G, Barohn R. Practice parameter: thymectomy for autoimmune myasthenia gravis.  Neurology. 2000;  55 7-15
• 26 Ruff R L, Batchelor T T, Taylor L P et al.. Steroid myopathy in cancer patients.  Neurology. 1997;  48 1234-1238
• 27 Ruff R L. Acute illness myopathy.  Neurology. 1996;  46 600-601
• 28 Gutmann L, Blumenthal D, Schochet S S. Acute type II myofiber atrophy in critical illness.  Neurology. 1996;  46 819-821
• 29 Rich M M, Teener J W, Raps E C et al.. Muscle is electrically inexcitable in acute quadriplegic myopathy.  Neurology. 1996;  46 731-736
• 30 Eisenkraft J B, Papatestas A E, Kahn C H et al.. Predicting the need for postoperative mechanical ventilation in myasthenia gravis.  Anesthesiology. 1986;  65 79-82
• 31 Erlacher W, Kontaratos M, Muller C et al.. Intubation without muscle relaxation: alfentanil/propofol as an alternative induction regime for children.  Anesthesiology. 1998;  89(suppl) 1316A
• 32 Bell C F, Florence A M, Hunter J M et al.. Atracurium in the myasthenic patient.  Anaesthesia. 1984;  39 961-968
• 33 Hunter J M, Bell C F, Florence A M et al.. Vecuronium in the myasthenic patient.  Anaesthesia. 1985;  40 848-853
• 34 Rowlee S. Monitoring neuromuscular blockade in the intensive care unit: the peripheral nerve stimulator.  Heart Lung. 1999;  28 352-362
• 35 Goldman L, Caldera D L, Nussbaum S R et al.. Multifactorial index of cardiac risk in non-cardiac surgical procedures.  N Engl J Med. 1977;  297 845-850
• 36 Ely E W, Baker A M, Dunagan D P et al.. Effect on the duration of mechanical ventilation of identifying patients capable of breathing spontaneously.  N Engl J Med. 1996;  335 1864-1869
• 37 Esteban A, Frutos F, Tobin M J et al.. A comparison of four methods of weaning patients from mechanical ventilation.  N Engl J Med. 1995;  332 345-350
• 38 Adrogué H J, Madias N E. Management of life-threatening acid-base disorders, I and II.  N Engl J Med. 1998;  338 26-34 107-111
• 39 Yende S, Wunderink R. Causes of prolonged mechanical ventilation after coronary artery bypass surgery.  Chest. 2002;  122 245-252
• 40 Adnet F, Baud F. Relation between Glasgow Coma Scale and aspiration pneumonia.  Lancet. 1996;  348 123-124
• 41 Kim K, Lew S, Cho H Y et al.. Residual paralysis induced by either vecuronium or rocuronium after reversal with pyridostigmine.  Anesth Analg. 2002;  95 1656-1660
• 42 Gennari F J. Hypokalemia.  N Engl J Med. 1998;  339 451-458
• 43 Subramanian R, Khardori R M. Severe hypophosphatemia: pathophysiologic implications, clinical presentations, and treatment.  Medicine (Baltimore). 2000;  79 1-8
• 44 Oelkers W. Adrenal insufficiency.  N Engl J Med. 1996;  335 1206-1212
• 45 Nair S R, Pearson S B. Mucous plug in the bronchus causing lung collapse.  N Engl J Med. 2002;  347 1079
• 46 Fawcett W J, Stone J P. Recurarization in the recovery room following the use of magnesium sulphate.  Br J Anaesth. 2003;  91 435-438
• 47 McDonald C M. Physical activity, health impairments, and disability in neuromuscular disease.  Am J Phys Med Rehabil. 2002;  81 S108-S120
• 48 Ashworth S F, Cordingly J J. New modes of ventilation.  Curr Anesth Crit Care. 2003;  14 90-99
• 49 Vivien B, Di Maria S, Ouattara A et al.. Overestimation of bispectral index in sedated intensive care unit patients revealed by administration of muscle relaxant.  Anesthesiology. 2003;  99 9-17
• 50 Messner M, Beese U, Romstöck J K. The bispectral index declines during neuromuscular block in fully awake persons.  Anesth Analg. 2003;  97 488-491
• 51 Antonelli M, Conti G, Rocco M et al.. A comparison of noninvasive positive-pressure ventilation and conventional mechanical ventilation in patients with acute respiratory failure.  N Engl J Med. 1998;  339 429-435
• 52 Hart N, Simonds A K. The pulmonary physician in critical care. Illustrative case 4: neuromusculoskeletal disorders.  Thorax. 2003;  58 547-549
• 53 Rabinstein A, Wijdicks E FM. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation.  Neurology. 2002;  59 1647-1649
• 54 Djelmis J, Sostarko M, Mayer D et al.. Myasthenia gravis in pregnancy: report on 69 cases.  Eur J Obstet Gynecol Reprod Biol. 2002;  104 21-25
• 55 Hoff J M, Daltveit A K, Gilhus N E. Myasthenia gravis: consequences for pregnancy, delivery, and the newborn.  Neurology. 2003;  61 1362-1366