Brazilian Journal of Anesthesiology
https://bjan-sba.org/article/doi/10.1590/S0034-70942011000600007
Brazilian Journal of Anesthesiology
Clinical Information

Miastenia gravis: relato de dois casos e revisão da literatura

Myasthenia gravis: two case reports and review of the literature

Ana Laura Colle Kauling; Maria Cristina Simões de Almeida; Giovani de Figueiredo Locks; Guilherme Muriano Brunharo

http://dx.doi.org/10.1590/S0034-70942011000600007 Braz J Anesthesiol, vol.61, n6, p.755-763, 2011
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Resumo

JUSTIFICATIVA E OBJETIVOS: A Miastenia gravis (MG) é uma doença neurológica autoimune que afeta a porção pós-sináptica da junção neuromuscular. Trata-se de um desafio ao anestesiologista, pela diversidade das manifestações da doença e pela possibilidade de complicações ventilatórias no pós-operatório. O objetivo deste trabalho é demonstrar a importância da monitoração adequada ao bloqueio neuromuscular (BNM) em virtude das múltiplas formas de apresentação da MG. CONTEÚDO: Neste artigo serão descritos dois casos de pacientes com MG - um que apresentou a forma clássica de sensibilidade ao bloqueador neuromuscular (BNM) e outro com resposta semelhante à de um paciente normal. A revisão da literatura será restrita às características da doença e a descrição de sua fisiopatologia estará voltada às reações aos BNM. CONCLUSÕES: Como conclusão, sugere-se que, em decorrência das múltiplas formas de apresentação e de tratamento da MG, é fundamental o uso de monitores da transmissão neuromuscular quando se usa BNM.

Palavras-chave

ANESTESIA, BLOQUEADOR MUSCULAR, DOENÇAS, DOENÇAS, MONITORAÇÃO, MONITORAÇÃO

Abstract

BACKGROUND AND OBJECTIVES: Myasthenia gravis (MG) is an autoimmune neurologic disease that affects the postsynaptic portion of the neuromuscular junction. It represents a challenge for anesthesiologists due to the diversity of disease manifestations and possibility of postoperative respiratory complications. The objective of this study was to demonstrate the importance of adequate monitoring of the neuromuscular blockade (NMB) due to the multiple presentations of MG. CONTENTS: In this paper we report two cases of patients with MG. The first patient presented with the classical sensitivity to the neuromuscular blocker (NMB) and the second had a similar response to that of a normal patient. The literature review will be restricted to disease characteristics, while the description of its pathophysiology will focus on its reactions to NMB. CONCLUSIONS: We suggest that, due to the multiple presentation and treatment of MG, neuromuscular transmission monitors are fundamental when using NMB.

Keywords

Neuromuscular Blocking Agents, Myasthenia gravis, Electromyography, Atracurium, Anesthesia, Monitoring

References

Grob D, Brunner N, Namba T. Lifetime course of myasthenia gravis. Muscle Nerve. 2008;37:141-149.

Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009;8:475-490.

Vincent A. Autoimmune disorders of the neuromuscular junction. Neurol India. 2008;56:305-313.

Lang B, Vincent A. Autoimmune disorders of the neuromuscular junction. Curr Opin Pharmacol. 2009;9:336-340.

Ruff RL, Lennon VA. How myasthenia gravis alters the safety factor for neuromuscular transmission. J Neuroimmunol. 2008;201-202:13-20.

Lang B, Vincent A. Autoantibodies to ion channels at the neuromuscular junction. Autoimmun Rev. 2003;2:94-100.

Pasnoor M, Wolfe GI, Nations S. Clinical findings in MuSK-antibody positive myasthenia gravis: a U.S. experience. Muscle Nerve. 2009;41:370-374.

Provenzano C, Marino M, Scuderi F. Anti-acetylcholinesterase antibodies associate with ocular myasthenia gravis. J Neuroimmunol. 2009;218:102-106.

Blobner M, Mann R. Anesthesia in patients with myasthenia gravis. Anaesthesist. 2001;50:484-493.

Souza Neto D, Módolo N. Miastenia gravis: implicações anestésicas. Rev Bras Anestesiol. 1993;43:373-382.

Almeida MCS. Uso de Bloqueadores Neuromusculares em Pacientes com Miastenia gravis: Relato de dois casos. Rev Bras Anestesiol. 2001;51:133-140.

Mann R, Blobner M, Jelen-Esselborn S. Preanesthetic trainof-four fade predicts the atracurium requirement of myasthenia gravis patients. Anesthesiology. 2000;93:346-350.

Romi F, Gilhus NE, Aarli JA. Myasthenia gravis: clinical, immunological, and therapeutic advances. Acta Neurol Scand. 2005;111:134-141.

Meriggioli MN. Myasthenia gravis with anti-acetylcholine receptor antibodies. Front Neurol Neurosci. 2009;26:94-108.

Meyer A, Levy Y. Geoepidemiology of myasthenia gravis. Autoimmun Rev. 2009;9:383-386.

Chiu HC, Vincent A, Newsom-Davis J. Myasthenia gravis: population differences in disease expression and acetylcholine receptor antibody titers between Chinese and Caucasians. Neurology. 1987;37:1854-1857.

Phillips LH. The epidemiology of myasthenia gravis. Semin Neurol. 2004;24:17-20.

Ercolini AM, Miller SD. Role of immunologic cross-reactivity in neurological diseases. Neurol Res. 2005;27:726-733.

McGuire LJ, Huang DP, Teoh R. Epstein-Barr virus genome in thymoma and thymic lymphoid hyperplasia. Am J Pathol. 1988;131:385-390.

Mori M, Kuwabara S, Nemoto Y. Concomitant chronic inflammatory demyelinating polyneuropathy and myasthenia gravis following cytomegalovirus infection. J Neurol Sci. 2006;240:103-106.

Lalive PH, Allali G, Truffert A. Myasthenia gravis associated with HTLV-I infection and atypical brain lesions. Muscle Nerve. 2007;35:525-528.

von Herrath MG, Fujinami RS, Whitton JL. Microorganisms and autoimmunity: making the barren field fertile?. Nat Rev Microbiol. 2003;1:151-157.

Roxanis I, Micklem K, Willcox N. True epithelial hyperplasia in the thymus of early-onset myasthenia gravis patients: implications for immunopathogenesis. J Neuroimmunol. 2001;112:163-173.

Giraud M, Beaurain G, Yamamoto AM. Linkage of HLA to myasthenia gravis and genetic heterogeneity depending on anti-titin antibodies. Neurology. 2001;57:1555-1560.

Kaminski HJ, Li Z, Richmonds C. Complement regulators in extraocular muscle and experimental autoimmune myasthenia gravis. Exp Neurol. 2004;189:333-342.

Lennon VA, Lambert EH. Myasthenia gravis induced by monoclonal antibodies to acetylcholine receptors. Nature. 1980;285:238-240.

Newsom-Davis J, Pinching AJ, Vincent A. Function of circulating antibody to acetylcholine receptor in myasthenia gravis: investigation by plasma exchange. Neurology. 1978;28:266-272.

Protti MP, Manfredi AA, Straub C. Immunodominant regions for T helper-cell sensitization on the human nicotinic receptor alpha subunit in myasthenia gravis. Proc Natl Acad Sci U S A. 1990;87:7792-7796.

Wang ZY, Okita DK, Howard Jr J. T-cell recognition of muscle acetylcholine receptor subunits in generalized and ocular myasthenia gravis. Neurology. 1998;50:1045-1054.

Naguib M, Brull SJ. Update on neuromuscular pharmacology. Curr Opin Anaesthesiol. 2009;22:483-490.

Ellison M, Feng ZP, Park AJ. Alpha-RgIA, a novel conotoxin that blocks the alpha9alpha10 nAChR: structure and identification of key receptor-binding residues. J Mol Biol. 2008;377:1216-1227.

Saez-Briones P, Krauss M, Dreger M. How do acetylcholine receptor ligands reach their binding sites?. Eur J Biochem. 1999;265:902-910.

Vernino S, Adamski J, Kryzer TJ. Neuronal nicotinic ACh receptor antibody in subacute autonomic neuropathy and cancer-related syndromes. Neurology. 1998;50:1806-1813.

Osserman KE, Genkins G. Studies in myasthenia gravis: review of a twenty-year experience in over 1200 patients. Mt Sinai J Med. 1971;38:497-537.

Ngo ST, Noakes PG, Phillips WD. Neural agrin: a synaptic stabiliser. Int J Biochem Cell Biol. 2007;39:863-867.

Brejc K, van Dijk WJ, Klaassen RV. Crystal structure of an ACh-binding protein reveals the ligand-binding domain of nicotinic receptors. Nature. 2001;411:269-276.

Poo MM. Neurotrophins as synaptic modulators. Nat Rev Neurosci. 2001;2:24-32.

Nitkin RM, Smith MA, Magill C. Identification of agrin, a synaptic organizing protein from Torpedo electric organ. J Cell Biol. 1987;105:2471-2478.

Mossman S, Vincent A, Newsom-Davis J. Myasthenia gravis without acetylcholine-receptor antibody: a distinct disease entity. Lancet. 1986;1:116-119.

Fuhrer C, Sugiyama JE, Taylor RG. Association of muscle-specific kinase MuSK with the acetylcholine receptor in mammalian muscle. EMBO J. 1997;16:4951-4960.

Wallace B. scFvs get down to basics: how MuSK makes synapses. Nat Biotechnol. 1997;15:721-722.

Naguib M, Flood P, McArdle JJ. Advances in neurobiology of the neuromuscular junction: implications for the anesthesiologist. Anesthesiology. 2002;96:202-231.

Hughes BW, Kusner LL, Kaminski HJ. Molecular architecture of the neuromuscular junction. Muscle Nerve. 2006;33:445-461.

Changeux JP, Taly A. Nicotinic receptors, allosteric proteins and medicine. Trends Mol Med. 2008;14:93-102.

Lindstrom JM. Acetylcholine receptors and myasthenia. Muscle Nerve. 2000;23:453-477.

Celesia GG. Disorders of membrane channels or channelopathies. Clin Neurophysiol. 2001;112:2-18.

Catterall WA. From ionic currents to molecular mechanisms: the structure and function of voltage-gated sodium channels. Neuron. 2000;26:13-25.

Sine SM, Engel AG. Recent advances in Cys-loop receptor structure and function. Nature. 2006;440:448-455.

Unwin N. Refined structure of the nicotinic acetylcholine receptor at 4A resolution. J Mol Biol. 2005;346:967-989.

Ohno K, Brengman J, Tsujino A. Human endplate acetylcholinesterase deficiency caused by mutations in the collagen-like tail subunit (ColQ) of the asymmetric enzyme. Proc Natl Acad Sci USA. 1998;95:9654-9659.

Croxen R, Newland C, Beeson D. Mutations in different functional domains of the human muscle acetylcholine receptor alpha subunit in patients with the slow-channel congenital myasthenic syndrome. Hum Mol Genet. 1997;6:767-774.

Vincent A, Beeson D, Lang B. Molecular targets for autoimmune and genetic disorders of neuromuscular transmission. Eur J Biochem. 2000;267:6717-6728.

Nakata M, Kuwabara S, Kawaguchi N. Is excitation-contraction coupling impaired in myasthenia gravis?. Clin Neurophysiol. 2007;118:1144-1148.

Nocite J. Miastenia gravis e Anestesia. Rev Bras Anestesiol. 1990;40:443-448.

Baraka A, Wakid N, Mansour R. Effect of neostigmine and pyridostigmine on the plasma cholinesterase activity. Br J Anaesth. 1981;53:849-85.

Naguib M, el Dawlatly AA, Ashour M. Multivariate determinants of the need for postoperative ventilation in myasthenia gravis. Can J Anaesth. 1996;43:1006-1013.

Baraka A. Anaesthesia and myasthenia gravis. Can J Anaesth. 1992;39:476-486.

Eisenkraft JB, Book WJ, Mann SM. Resistance to succinylcholine in myasthenia gravis: a dose-response study. Anesthesiology. 1988;69:760-763.

Naguib M, Sari-Kouzel A, Ashour M. Myasthenia gravis and pipecuronium--report of two cases. Middle East J Anesthesiol. 1992;11:381-390.

Itoh H, Shibata K, Nitta S. Sensitivity to vecuronium in seropositive and seronegative patients with myasthenia gravis. Anesth Analg. 2002;95:109-113.

Kim JM, Mangold J. Sensitivity to both vecuronium and neostigmine in a sero-negative myasthenic patient. Br J Anaesth. 1989;63:497-500.

Nilsson E, Meretoja OA. Vecuronium dose-response and maintenance requirements in patients with myasthenia gravis. Anesthesiology. 1990;73:28-32.

Smith CE, Donati F, Bevan DR. Cumulative dose-response curves for atracurium in patients with myasthenia gravis. Can J Anaesth. 1989;36:402-406.

Baraka A. Suxamethonium block in the myasthenic patient: Correlation with plasma cholinesterase. Anaesthesia. 1992;47:217-219.

Mann R, Blobner M. Neuromuscular monitoring in myasthenia gravis. Anaesthesist. 2000;49(^s1):26-28.

Unterbuchner C, Fink H, Blobner M. The use of sugammadex in a patient with myasthenia gravis. Anaesthesia. 2010;65:302-305.

de Boer HD, van Egmond J, Driessen JJ. Sugammadex in patients with myasthenia gravis. Anaesthesia. 2010;65.

Tripathi M, Kaushik S, Dubey P. The effect of use of pyridostigmine and requirement of vecuronium in patients with myasthenia gravis. J Postgrad Med. 2003;49:311-314.

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