Anaesthesia and neuromuscular disorders

Czech version

Authors: S. Voháňka ¹; P. Štourač ²; M. Klincová ²
Authors‘ workplace: Neuromuskulární centrum, Neurologická klinika LF MU a FN Brno ¹; Klinika dětské anesteziologie a resuscitace, LF MU a FN Brno ²
Published in: Cesk Slov Neurol N 2018; 81(5): 501-514
Category: Minimonography
doi: https://doi.org/10.14735/amcsnn2018501

Overview

fragile balance can lead to postponing of necessary interventions, but on the other hand, it can lead to serious risks with improper approaches. Myasthenia is a disease where there is no risk of malignant hyperthermia, and there is an altered sensitivity to peripheral myorelaxants. We prefer to avoid benzodiazepines as a premedication, and after the procedure we always place the patient on a monitored bed with the possibility of artificial ventilation. We do not give suxamethonium to muscular dystrophy and myotonic dystrophy patients and do not use volatile gases due to risk of rhabdomyolysis. There is also no risk of malignant hyperthermia in this group. Malignant hyperthermia is a pharmacogenetic disorder manifested by abnormal hypermetabolic response when exposed to halogenated inhalation anesthetics (halothane, isoflurane, desflurane, sevoflurane) or peripheral muscle relaxants of the depolarizing type (suxamethonium). It can rarely occur even after physical, excessive or heat stress. It is associated with electromechanical coupling and most often occurs with mutations in the ryanodine receptor, but rarely with some other mutations in the genes that are related to calcium metabolism.

Key words:
anaesthesia – myasthenia gravis – muscular dystrophy – myotonic dystrophy – neuromuscular blockade – malignant hyperthermia

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Sources

1. Katz JA, Murphy GS. Anesthetic consideration for neuromuscular diseases. Curr Opin Anaesthesiol 2017; 30(3): 435– 440. doi: 10.1097/ ACO.0000000000000466.

2. Klingler W, Lehmann-Horn F, Jurkat-Rott K. Complications of anaesthesia in neuromuscular disorders. Neuromuscul Disord 2005; 15(3): 195– 206. doi: 10.1016/ j.nmd.2004.10.017.

3. Adamus M, Cvachovec K, Černý V et al. Doporučený postup: zásady bezpečné anesteziologické péče. Anest Intenziv Med 2018; 29(2): 107– 110.

4. Tschiedel E, Müller O, Schara U et al. Sedation monitoring during open muscle biopsy in children by Comfort Score and Bispectral Index - a prospective analysis. Paediatr Anaesth 2015; 25(3): 265– 271. doi: 10.1111/ pan.12547.

5. Isnardon S, Vinclair M, Genty C et al. Pupillometry to detect pain response during general anaesthesia following unilateral popliteal sciatic nerve block: a prospective, observational study. Eur J Anaesthesiol 2013; 30(7): 429– 434. doi: 10.1097/ EJA.0b013e32835f0030.

6. Guglielminotti J, Grillot N, Paule M et al. Prediction of movement to surgical stimulation by the pupillary dilatation reflex amplitude evoked by a standardized noxious test. Anesthesiology 2015; 122(5): 985– 993. doi: 10.1097/ ALN.0000000000000624.

7. Mukaihara K, Godai K, Yamada T et al. Successful airway management using a MultiViewScope handle with a stylet scope in a patient with Schwartz-Jampel syndrome. JA Clin Rep 2016; 2(1): 36. doi: 10.1186/ s40981-016-0062-5.

8. Gritti P, Carrara B, Khotcholava M et al. The use of desflurane or propofol in combination with remifentanil in myasthenic patients undergoing a video-assisted thoracoscopic-extended thymectomy. Acta Anaesthesiol Scand 2009; 53(3): 380– 389. doi: 10.1111/ j.1399-6576.2008.01853.x.

9. Stourac P, Krikava I, Seidlova J et al. Sugammadex in a parturient with myotonic dystrophy. Br J Anaesth 2013; 110(4): 657– 658. doi: 10.1093/ bja/ aet037.

10. Kosinova M, Stourac P, Harazim H et al. Anaesthesia and orphan disease: rocuronium and sugammadex in the anaesthetic management of a parturient with Becker’s myotonia congenita. Eur J Anaesthesiol 2016; 33(7): 545– 547. doi: 10.1097/ EJA.0000000000000442.

11. Racca TM. Recommendations for anesthesia and perioperative management of patients with neuromuscular disorders. Minerva Anestesiol 2013; 79(4): 419– 433.

12. Abel M, Eisenkraft JB. Anesthetic implications of myasthenia gravis. Mt Sinai J Med 2002; 69(1– 2): 31– 37.

13. Fischer T. Anaesthesia recommendations for patients suffering from Myasthenia gravis. Anästh Intensivmed 2015; 56: S654– S661.

14. Vymazal T, Horáček M, Bicek V et al. Myasthenia gravis a anestezie – nový bezpečnější postup. Anest Intenziv Med 2014; 25(1): 21– 24.

15. Bicek V, Vymazal T. Desfluran jako vhodné inhalační anestetikum u pacientky s těžkou formou myasthenia gravis. Anest Intenziv Med 2013; 24(3): 160– 162.

16. Vymazal T, Krecmerova M, Bicek V et al. Feasibility of full and rapid neuromuscular blockade recovery with sugammadex in myasthenia gravis patients undergoing surgery – a series of 117 cases. Ther Clin Risk Manag 2015; 11: 1593– 1596. doi: 10.2147/ TCRM.S93009.

17. Romero A, Joshi GP. Neuromuscular disease and anesthesia. Muscle Nerve 2013; 48(3): 451– 460. doi: 10.1002/ mus.23817.

18. Brambrink AM, Kirsch JR. Perioperative care of patients with neuromuscular disease and dysfunction. Anesthesiol Clin 2007; 25(3): 483– 509, viii– ix. doi: 10.1016/ j.anclin.2007.05.005.

19. Voháňka S. Léky a nervosvalový přenos. Neurol Praxi 2017; 18(1): 11– 14.

20. Bae JS, Go SM, Kim BJ. Clinical predictors of steroid-induced exacerbation in myasthenia gravis. J Clin Neurosci Off J Neurosurg Soc Australas 2006; 13: 1006– 1010. doi: 10.1016/ j.jocn.2005.12.041.

21. Rangasamy V, Kumar K, Rai A et al. Sevoflurane and thoracic epidural anesthesia for trans-sternal thymectomy in a child with juvenile myasthenia gravis. J Anaesthesiol Clin Pharmacol 2014; 30(2): 276– 278. doi: 10.4103/ 0970-9185.130088.

22. Dunsire MF, Clarke SG, Stedmon JJ. Undiagnosed myasthenia gravis unmasked by neuromuscular blockade. Br J Anaesth 2001; 86(5): 727– 730.

23. Eisenkraft JB, Book WJ, Mann SM et al. Resistance to succinylcholine in myasthenia gravis: a dose-response study. Anesthesiology 1988; 69(5): 760– 763.

24. Bowie RA. Myasthenia gravis unmasked by neuromuscular blockade. Br J Anaesth 2002; 88(1): 153– 154.

25. Marsh S, Pittard A. Neuromuscular disorders and anaesthesia. Part 2: specific neuromuscular disorders. Contin Educ Anaesth Crit Care Pain 2011; 11(4): 119– 123. doi: 10.1093/ bjaceaccp/ mkr019.

26. Wefki Abdelgawwad Shousha AA, Sanfilippo M, Sabba A et al. Sugammadex and reversal of neuromuscular block in adult patient with duchenne muscular dystrophy. Case Rep Anesthesiol 2014; 2014: e680568. doi: 10.1155/ 2014/ 680568.

27. Batistaki C, Tentes P, Deligiannidi P et al. Residual neuromuscular blockade in a real life clinical setting. Correlation with sugammadex or neostigmine administration. Minerva Anestesiol 2015; 82(5): 550– 558.

28. White MC, Stoddart PA. Anesthesia for thymectomy in children with myasthenia gravis. Paediatr Anaesth 2004; 14(8): 625– 635. doi: 10.1111/ j.1460-9592.2004.01292.x.

29. Racca F, Mongini T, Wolfler A et al. Recommendations for anesthesia and perioperative management of patients with neuromuscular disorders. Minerva Anestesiol 2013; 79(4): 419– 433.

30. Rosenberg H, Pollock N, Schiemann A et al. Malignant hyperthermia: a review. Orphanet J Rare Dis 2015; 10: 93. doi: 10.1186/ s13023-015-0310-1.

31. Halliday NJ. Malignant hyperthermia. J Craniofac Surg 2003; 14(5): 800– 802.

32. Ording H. Incidence of malignant hyperthermia in Denmark. Anesth Analg 1985; 64(7): 700– 704.

33. Riazi S, Larach MG, Hu C et al. Malignant hyperthermia in Canada: characteristics of index anesthetics in 129 malignant hyperthermia susceptible probands. Anesth Analg 2014; 118(2): 381– 387. doi: 10.1213/ ANE.0b013e3182937d8b.

34. Brady JE, Sun LS, Rosenberg H et al. Prevalence of malignant hyperthermia due to anesthesia in New York State, 2001-2005. Anesth Analg 2009; 109(4): 1162– 1166. doi: 10.1213/ ane.0b013e3181ac1548.

35. Brandom BW, Callahan PM. Malignant hyperthermia: an update. Adv Anesth 2015; 33(1): 113– 128. doi: 10.1016/ j.aan.2015.07.007.

36. Campion GH, Hadi AS, Berman AJ et al. Questions regarding the diagnosis of malignant hyperthermia. Anesthesiology 2015; 123(3): 731– 732. doi: 10.1097/ ALN.0000000000000760.

37. Gonsalves SG, Ng D, Johnston JJ et al. Using exome data to identify malignant hyperthermia susceptibility mutations. Anesthesiology 2013; 119(5): 1043– 1053. doi: 10.1097/ ALN.0b013e3182a8a8e7.

38. Groom L, Muldoon SM, Tang ZZ et al. Identical de novo mutation in the type 1 ryanodine receptor gene associated with fatal, stress-induced malignant hyperthermia in two unrelated families. Anesthesiology 2011; 115(5): 938– 945. doi: 10.1097/ ALN.0b013e3182320068.

39. Thomas J, Crowhurst T. Exertional heat stroke, rhabdomyolysis and susceptibility to malignant hyperthermia. Intern Med J 2013; 43(9): 1035– 1038. doi: 10.1111/ imj.12232.

40. Carpenter D, Ringrose C, Leo V et al. The role of CACNA1Sin predisposition to malignant hyperthermia. BMC Med Genet 2009; 10: 104. doi: 10.1186/ 1471-2350-10-104.

41. Stewart SL, Hogan K, Rosenberg H et al. Identification of the Arg1086His mutation in the alpha subunit of the voltage-dependent calcium channel (CACNA1S) in a North American family with malignant hyperthermia. Clin Genet 2001; 59(3): 178– 184.

42. Fiszer D, Shaw M-A, Fisher NA et al. Next-generation Sequencing of RYR1 and CACNA1S in malignant hyperthermia and exertional heat illness. Anesthesiology 2015; 122(5): 1033– 1046. doi: 10.1097/ ALN.00000 00000000610.

43. Jurkat-Rott K, McCarthy T, Lehmann-Horn F. Genetics and pathogenesis of malignant hyperthermia. Muscle Nerve 2000; 23(1): 4– 17.

44. Jungbluth H, Dowling JJ, Ferreiro A et al. 217th ENMC International Workshop: RYR1-related myopathies, Naarden, The Netherlands, 29–31 January 2016. Neuromuscul Disord 2016; 26(9): 624– 633. doi: 10.1016/ j.nmd.2016.06.001.

45. Lehmann-Horn F, Klingler W, Jurkat-Rott K. Nonanesthetic malignant hyperthermia. Anesthesiology 2011; 115(5): 915– 917. doi: 10.1097/ ALN.0b013e318232008f.

46. Davis PJ, Brandom BW. The association of malignant hyperthermia and unusual disease: when you’re hot you’re hot or maybe not. Anesth Analg 2009; 109(4): 1001– 1003. doi: 10.1213/ ane.0b013e3181b493d4.

47. McCarthy TV, Quane KA, Lynch PJ. Ryanodine receptor mutations in malignant hyperthermia and central core disease. Hum Mutat 2000; 15(5): 410– 417. doi: 10.1002/ (SICI)1098-1004(200005)15: 5<410::AID-HUMU2>3.0.CO; 2-D.

48. Sanoudou D, Beggs AH. Clinical and genetic heterogeneity in nemaline myopathy--a disease of skeletal muscle thin filaments. Trends Mol Med 2001; 7(8): 362– 368.

49. Fattori F, Maggi L, Bruno C et al. Centronuclear myopathies: genotype-phenotype correlation and frequency of defined genetic forms in an Italian cohort. J Neurol 2015; 262(7): 1728– 1740. doi: 10.1007/ s00415-015-7757-9.

50. Vymazal T. Maligní hypertermie. Anest Intenziv Med 2016; 27(2): 71– 74.

51. McKenney KA, Holman SJ. Delayed postoperative rhabdomyolysis in a patient subsequently diagnosed as malignant hyperthermia susceptible. Anesthesiology 2002; 96(3): 764– 765.

52. Larach MG, Brandom BW, Allen GC et al. Malignant hyperthermia deaths related to inadequate temperature monitoring, 2007-2012: a report from the North American malignant hyperthermia registry of the malignant hyperthermia association of the United States. Anesth Analg 2014; 119(6): 1359– 1366. doi: 10.1213/ ANE.0000000000000421.

53. Ording H, Brancadoro V, Cozzolino S et al. In vitro contracture test for diagnosis of malignant hyperthermia following the protocol of the European MH Group: results of testing patients surviving fulminant MH and unrelated low-risk subjects. The European Malignant Hyperthermia Group. Acta Anaesthesiol Scand 1997; 41(8): 955– 966.

54. Allen GC, Larach MG, Kunselman AR. The sensitivity and specificity of the caffeine-halothane contracture test: a report from the North American Malignant Hyperthermia Registry. The North American Malignant Hyperthermia Registry of MHAUS. Anesthesiology 1998; 88(3): 579– 588.

55. European malignant hyperthermia group. Diagnostic MH mutations. [online]. Available form URL: https: / / www.emhg.org/ diagnostic-mutations/ .

56. Isaak RS, Stiegler MP. Review of crisis resource management (CRM) principles in the setting of intraoperative malignant hyperthermia. J Anesth 2016; 30(2): 298– 306. doi: 10.1007/ s00540-015-2115-8.

57. Yemen TA, McClain C. Muscular dystrophy, anesthesia and the safety of inhalational agents revisited; again. Paediatr Anaesth 2006; 16(2): 105– 108. doi: 10.1111/ j.1460-9592.2005.01801.x.

58. Girshin M, Mukherjee J, Clowney R et al. The postoperative cardiovascular arrest of a 5-year-old male: an initial presentation of Duchenne’s muscular dystrophy. Paediatr Anaesth 2006; 16(2): 170– 173. doi: 10.1111/ j.1460-9592.2005.01698.x.

59. Hayes J, Veyckemans F, Bissonnette B. Duchenne muscular dystrophy: an old anesthesia problem revisited. Paediatr Anaesth 2008; 18(2): 100– 106. doi: 10.1111/ j.1460-9592.2007.02302.x.

60. Hayes J, Veyckemans F, Bissonnette B. Rhabdomyolysis and anesthesia. Paediatr Anaesth 2008; 18(9): 897– 898. doi: 10.1111/ j.1460-9592.2008.02599.x.

61. de Boer HD, van Esmond J, Booij LH et al. Reversal of rocuronium-induced profound neuromuscular block by sugammadex in Duchenne muscular dystrophy. Paediatr Anaesth 2009; 19(12): 1226– 1228. doi: 10.1111/ j.1460-9592.2009.03178.x.

62. Bang SU, Kim YS, Kwon WJ et al. Peripheral nerve blocks as the sole anesthetic technique in a patient with severe Duchenne muscular dystrophy. J Anesth 2016; 30(2): 320– 323. doi: 10.1007/ s00540-015-2127-4.

63. Muenster T, Mueller C, Forst J et al. Anaesthetic management in patients with Duchenne muscular dystrophy undergoing orthopaedic surgery: a review of 232 cases. Eur J Anaesthesiol 2012; 29(10): 489– 494. doi: 10.1097/ EJA.0b013e3283566789.

64. Miles F, Dare T. Scoliosis repair in a teenager with Duchenne’s muscular dystrophy: who calls the shots? Paediatr Anaesth 2009; 19(10): 1022– 1024. doi: 10.1111/ j.1460-9592.2009.03071.x

65. Meola G, Cardani R. Myotonic dystrophies: An update on clinical aspects, genetic, pathology, and molecular pathomechanisms. Biochim Biophys Acta 2015; 1852(4): 594– 606. doi: 10.1016/ j.bbadis.2014.05.019.

66. Meola G, Cardani R. Myotonic dystrophy type 2: an update on clinical aspects, genetic and pathomolecular mechanism. J Neuromuscul Dis 2015; 2(s2): S59– S71. doi: 10.3233/ JND-150088.

67. Veyckemans F, Scholtes JL. Myotonic dystrophies type 1 and 2: anesthetic care. Paediatr Anaesth 2013; 23(9): 794– 803. doi: 10.1111/ pan.12120.

68. Bisinotto FMB, Fabri DC, Calçado MS et al. Anesthesia for videolaparoscopic cholecystectomy in a patient with Steinert disease. Case report and review of the literature. Rev Bras Anestesiol 2010; 60(2): 181– 191.

69. Schoser BG, Ricker K, Schneider-Gold C et al. Sudden cardiac death in myotonic dystrophy type 2. Neurology 2004; 63(12): 2402– 2404.

70. Sansone VA, Brigonzi E, Schoser B et al. The frequency and severity of cardiac involvement in myotonic dystrophy type 2 (DM2): long-term outcomes. Int J Cardiol 2013; 168(2): 1147– 1153. doi: 10.1016/ j.ijcard.2012.11.076.

71. Groh WJ, Lowe MR, Zipes DP. Severity of cardiac conduction involvement and arrhythmias in myotonic dystrophy type 1 correlates with age and CTG repeat length. J Cardiovasc 2002; 13(5): 444– 448.

72. Mathieu J, Allard P, Gobeil G et al. Anesthetic and surgical complications in 219 cases of myotonic dystrophy. Neurology 1997; 49(6): 1646– 1650.

73. Sinclair JL, Reed PW. Risk factors for perioperative adverse events in children with myotonic dystrophy. Paediatr Anaesth 2009; 19(8): 740– 747. doi: 10.1111/ j.1460-9592.2009.03079.x.

74. Weingarten TN, Hofer RE, Milone M et al. Anesthesia and myotonic dystrophy type 2: a case series. Can J Anesth 2010; 57(3): 248– 255. doi: 10.1007/ s12630-009-9244-1.

75. Wahbi K, Bellino A, Duboc D et al. Venous thromboembolism in myotonic dystrophy type 1. [online]. Available from URL: https:/ / clinicaltrials.gov/ ct2/ show/ NCT03424460.

76. Veyckemans F. Can inhalation agents be used in the presence of a child with myopathy? Curr Opin Anaesthesiol 2010; 23(3): 348– 355. doi: 10.1097/ ACO.0b013e3283393977.

77. Driessen JJ. Neuromuscular and mitochondrial disorders: what is relevant to the anaesthesiologist? Curr Opin Anaesthesiol 2008; 21(3): 350– 355. doi: 10.1097/ ACO.0b013e3282f82bcc.

78. Bembi B, Cerini E, Danesino C et al. Management and treatment of glycogenosis type II. Neurology 2008; 71 (23 Suppl 2): S12– S36. doi: 10.1212/ WNL.0b013e31818da93f.

79. Kishnani PS, Steiner RD, Bali D et al. Pompe disease diagnosis and management guideline. Genet Med 2006; 8(5): 267– 288. doi: 10.1097/ 01.gim.0000218152.87434.f3.

80. Ploeg AT van der. Monitoring of pulmonary function in Pompe disease: a muscle disease with new therapeutic perspectives. Eur Respir J 2005; 26(6): 984– 985. doi: 10.1183/ 09031936.05.00112005.

81. Pfeiffer G, Schiller B, Kruse J et al. Indicators of dysautonomia in severe Guillain-Barré syndrome. J Neurol 1999; 246(11): 1015– 1022.

82. Kotani N, Hirota K, Anzawa N et al. Motor and sensory disability has a strong relationship to induction dose of thiopental in patients with the hypertropic variety of Charcot-Marie-Tooth syndrome. Anesth Analg 1996; 82(1): 182– 186.

83. Orphananesthesia: a project of the German Society of Anesthesiology and Intensive Care Medicine. [online]. Available from URL: https:/ / www.orphananesthesia.eu/ .