Patients with Chronic Neuromuscular Diseases

  • Valentine Léopold
  • Alice Blet
  • Kathleen McGee
  • Benoît Plaud
Chapter
First Online:

Abstract

Neuromuscular diseases are a group of acquired or congenital conditions, characterized by the impairment of either neuromuscular transmission or the muscle itself (myopathies). Myasthenia gravis is characterized by a defect in the transmission of the nervous signal due to a quantitative reduction of the acetylcholine postsynaptic receptor. These diseases are rare and in nonspecialized centres, clinicians and anaesthetists, in particular, are not often confronted with such patients. Respiratory and cardiac involvement are the main factors determining the prognosis. These diseases require careful preoperative screening. The use of some specific anaesthetic agents may be contraindicated or modified as they increase the risk of perioperative complications, mainly respiratory and cardiac. These patients undergo surgical procedures, which may be (1) related to the treatment of the aetiology of the disease (thymectomy in myasthenia), (2) functional surgery (correction of a spinal deformation in the case of myopathy) and (3) treatment of complications of the disease (cataract or cholecystectomy in Steinert myotonic dystrophy).

Keywords

Anaesthesia Neuromuscular disease Perioperative complication 
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Notes

Conflict of Interest

Benoît Plaud is consultant and lecturer for MSD™ France.

Alice Blet, Valentine Léopold and Kathleen McGee have no current conflict of interest.

Key Points

  • Neuromuscular diseases are divided into disorders of (1) the nicotinic acetylcholine receptor (nAchR), (2) the motor endplate (myasthenia gravis) and (3) the muscle fibre (myopathy).

  • These are rare diseases. However, complications occurring in the perioperative period are potentially life-threatening.

  • These patients undergo surgical procedures, which may be (1) related to the treatment of the aetiology of the disease (thymectomy in myasthenia), (2) functional surgery (correction of a spinal deformation in the case of myopathy) and (3) treatment complications of the disease (cataract or cholecystectomy during Steinert myotonic dystrophy). The preoperative screening of these patients is mainly oriented towards respiratory and cardiac function.

  • Myopathies are a subgroup of inherited diseases characterized by primary muscle damage. The two most frequent types are Duchenne muscular dystrophy and myotonic dystrophy known as Steinert’s disease.

  • Anaesthetic management of patients with myopathies has to take into consideration impaired respiratory and cardiac and smooth muscle function, which worsen progressively over time.

  • Intravenous anaesthetics should be preferred to inhalational anaesthetics as halogenated agents impair neuromuscular transmission.

  • The risk of MH is strongly associated with the central core myopathy, these two diseases sharing the same genetic anomaly of the ryanodine receptor.

  • Local anaesthesia is a good alternative when possible and is useful for postoperative pain control.

  • Non-depolarizing muscle relaxants can be used, but the doses needed should be drastically reduced in myasthenia gravis and in myopathies. Monitoring of neuromuscular function is thus imperative in this context. Succinylcholine is strictly contraindicated in muscle diseases (generalized contracture, rhabdomyolysis).

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Copyright information

© Springer International Publishing AG 2018

Authors and Affiliations

  • Valentine Léopold
    • 1
  • Alice Blet
    • 1
    • 2
  • Kathleen McGee
    • 1
  • Benoît Plaud
    • 1
  1. 1.Anesthesia and Critical Care Department, Paris-Diderot University and Assistance Publique—Hôpitaux de ParisSaint-Louis Hospital, Sorbonne Paris Cité UniversityParis Cedex 10France
  2. 2.Inserm UMR S942Lariboisière HospitalParisFrance

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