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Chapter 41 - Neuromuscular Physiology and Disorders

Published online by Cambridge University Press:  21 February 2025

By
Jacqueline Donovan
Edited by
Dharti Patel ,
Sang J. Kim ,
Himani V. Bhatt  and
Alopi M. Patel
Dharti Patel
Affiliation:
Mount Sinai West and Morningside Hospitals, New York
Sang J. Kim
Affiliation:
Hospital for Special Surgery, New York
Himani V. Bhatt
Affiliation:
Mount Sinai West and Morningside Hospitals, New York
Alopi M. Patel
Affiliation:
Rutgers Robert Wood Johnson Medical School, New Jersey
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Summary

Disorders of the neuromuscular system have significant consequences for the anesthetic provider. A thorough knowledge of inheritance patterns, diagnosis, symptomatology, treatment, and pathophysiology is paramount in providing safe anesthetic care. Here we provide a review of the commonly encountered neuromuscular disorders and highlight the most salient features of each. The broad range of demyelinating diseases, myotonias, channelopathies, myasthenic syndromes, and mitochondrial diseases that affect the neuromuscular system will be presented.

Keywords

AnesthesiaMultiple SclerosisAmyotrophic Lateral SclerosisDuchenne’s Muscular DystrophyGuillain–Barré SyndromeMyastenia GravisLambert–Eaton SyndromeHyperkalemic Periodic Paralysis
Type
Chapter
Information
BASIC Essentials
A Comprehensive Review for the Anesthesiology BASIC Exam
 , pp. 226 - 230
Publisher: Cambridge University Press
Print publication year: 2025

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References

Further Reading

Dunø, M, Colding-Jørgensen, E. Myotonia congenita. 2005 Aug 3 [Updated 2015 Aug 6]. In Pagon, RA, Adam, MP, Ardinger, HH, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2017.Google Scholar
Fink, J. Heredeitary spastic paraplegia. Neurologic Clinics 2002;20:711–726.CrossRefGoogle ScholarPubMed
Jeevendra Martyn, JA. Neuromuscular physiology and pharmacology. In Miller, RD, Eriksson, LI, Fleischer, LA, et al., editors. Miller’s Anesthesia, 8th ed. Elsevier, 2015; chapter 22, pp 423–443.Google Scholar
Makris, A, Piperopoulos, A, Karmaniolou, I. Multiple sclerosis: basic knowledge and new insights in perioperative management. J Anesth 2014;28:267.CrossRefGoogle ScholarPubMed
Marchant, CL, Ellis, FR, Halsall, PJ, et al. Mutation analysis of two patients with hypokalemic periodic paralysis and suspected malignant hyperthermia. Muscle Nerve 2004;30:114–117.CrossRefGoogle ScholarPubMed
Neuromuscular blocking agents. In Butterworth, JF IV, Mackey, DC, Wasnick, JD, editors. Morgan & Mikhail’s Clinical Anesthesiology, 5th ed. McGraw-Hill, 2013; chapter 11, pp 199–220.Google Scholar
Nozari, A, Bagchi, A, Saxena, R, Bateman, BT. Neuromuscular disorders and other genetic disorders. In Miller, RD, Eriksson, LI, Fleischer, LA, et al., editors. Miller’s Anesthesia, 8th ed. Elsevier, 2015; chapter 42, pp 1266–1286.Google Scholar
Raja Rayan, DL, Hanna, MG. Skeletal muscle channelopathies: nondystrophic myotonias and periodic paralysis. Curr Opin Neurol 2010;23:466–476.CrossRefGoogle ScholarPubMed
Segura, L, Lorenz, J, Weingarten, T, et al. Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures. Pediatr Anaesth 2013;23:855–864.CrossRefGoogle ScholarPubMed
Shaw, PJ, Cudkowicz, ME. Amyotrophic lateral sclerosis and other motor neuron diseases. In Goldman, L, Schaffer, AI, editors. Goldman-Cecil Medicine, 26th ed. Elsevier, 2020; pp 2522–2526.Google Scholar
Sinclair, JL, Reed, PW. Risk factors for perioperative adverse events in children with myotonic dystrophy. Paediatr Anaesth 2009;19:740–747.CrossRefGoogle ScholarPubMed
Südhof, TC. The molecular machinery of neurotransmitter release (Nobel lecture). Angew Chem Int Ed Engl 2014 Nov 17;53(47):12696–12717.CrossRefGoogle ScholarPubMed
Thomas, M, Scrutton, M. Spinal anaesthesia in a patient with hereditary spastic paraplegia: case report and literature review. Int J of Obst Anesth 2006;15:254.CrossRefGoogle Scholar
Xiao, W, Zhao, L, Wang, F, et al. Total intravenous anesthesia without muscle relaxant in a parturient with amyotrophic lateral sclerosis undergoing cesarean section: a case report. J Clin Anesth 2017;36:107–109.CrossRefGoogle Scholar

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