Article Text

Download PDFPDF
Anaesthesia and neuromuscular disorders: what a neurologist needs to know
  1. Luuk R van den Bersselaar1,2,
  2. Marc M J Snoeck1,
  3. Madelief Gubbels2,
  4. Sheila Riazi3,
  5. Erik-Jan Kamsteeg4,
  6. Heinz Jungbluth5,6,7,
  7. Nicol C Voermans2
  1. 1 Anaesthesiology, Malignant Hyperthermia Investigation Unit, Canisius Wilhelmina Hospital, Nijmegen, Netherlands
  2. 2 Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboudumc, Nijmegen, Netherlands
  3. 3 Anesthesiology and Pain Medicine, Malignant Hyperthermia Investigation Unit, University of Toronto, Toronto, Canada
  4. 4 Genetics, Radboudumc, Nijmegen, Netherlands
  5. 5 Paediatric Neurology, Neuromuscular Service, Evelina’s Children Hospital, Guy’s and St Thomas’ Hospitals NHS Foundation Trust, London, UK
  6. 6 Department of Basic and Clinical Neuroscience, IoPPN, King’s College, London, UK
  7. 7 Randall Division for Cell and Molecular Biophysics, Muscle Signalling Section, King’s College, London, UK
  1. Correspondence to Nicol Voermans, Neuromuscular Centre Nijmegen, Department of Neurology, 910, Radboud University Nijmegen Medical Centre, P. O. Box 9101, Nijmegen 6500 HB, The Netherlands; nicol.voermans{at}radboudumc.nl

Abstract

Neurologists are often asked for specific advice regarding patients with neuromuscular disease who require general anaesthesia. However, guidelines on specific neuromuscular disorders do not usually include specific guidelines or pragmatic advice regarding (regional and/or general) anaesthesia or procedural sedation. Furthermore, the medical literature on this subject is mostly limited to publications in anaesthesiology journals. We therefore summarise general recommendations and specific advice for anaesthesia in different neuromuscular disorders to provide a comprehensive and accessible overview of the knowledge on this topic essential for clinical neurologists. A preoperative multidisciplinary approach involving anaesthesiologists, cardiologists, chest physicians, surgeons and neurologists is crucial. Depolarising muscle relaxants (succinylcholine) should be avoided at all times. The dose of non-depolarising muscle relaxants must be reduced and their effect monitored. Patients with specific mutations in RYR1 (ryanodine receptor 1) and less frequently in CACNA1S (calcium channel, voltage-dependent, L type, alpha 1S subunit) and STAC3 (SH3 and cysteine rich domain 3) are at risk of developing a life-threatening malignant hyperthermia reaction.

  • Anaesthetics
  • muscle disease
  • muscular dystrophy
  • myasthenia

Statistics from Altmetric.com

Footnotes

  • HJ and NV contributed equally

  • Contributors All authors have contributed significantly to the writing and editing of this manuscript and to the figures added.

  • Funding LRvdB received a Radboudumc Regional Junior Researcher Grant, in collaboration with the Canisius Wilhelmina Hospital.

  • Competing interests None declared.

  • Patient consent for publication Not required.

  • Ethics approval No ethical approval was required for this review.

  • Provenance and peer review Commissioned. Externally peer reviewed by Jon Walters, Swansea, UK.

  • Data availability statement A data sharing agreement was not applicable to this paper.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

Linked Articles

  • Editors’ commentary
    Phil E M Smith Geraint N Fuller

Other content recommended for you