Anaesthesia and neuromuscular disorders


Number 259
Date 11 December 2020

Location: Virtual meeting

Title: 259th ENMC International Workshop: Anaesthesia and neuromuscular disorders

Date: December 11th 2020; PART 1, PART 2 will take place in May 2021.

Organisers: Dr Nicol Voermans (Nijmegen, The Netherlands), Dr Marc Snoeck (Nijmegen, The Netherlands), Prof. Sheila Riazi (Toronto, Canada) and Prof. Heinz Jungbluth (London, UK).

Participants: Oscar Díaz Cambronero (Valencia, Spain), Martina Cornel (Amsterdam, The Netherlands), Klaus Dietrich (Grenoble, France), Francesca Forzana (London, UK), Luc Heytens (Antwerp, Belgium), Phil Hopkins (Leeds, UK), Anna Hellblom (Lund, Sweden), Erik Jan Kamsteeg (Nijmegen, The Netherlands), Andrea Klein (Bern, Switzerland), Ron Litman (Philadelphia, USA), Mette Ørngreen/Nicoline Løkken (Kopenhagen, Denmark), John Rendu (Grenoble, France), Jens Reinmann (Bonn, Germany), Hendrik Rueffert (Leipzig, Germany), Helga Silva (Sao Paulo, Brasil), Maria Soller (Stockholm, Sweden), Giorgio Tasca (Rome, Italy), Juan Jesus Vilchez (Valencia, Spain), Stephan Johanssen, (Wurzburg, Germany), Luuk van der Bersselaar (Nijmegen, the Netherlands)

Regrets: Robyn Gillies, (Melbourne, Australia) and Kathryn Stowell (Palmerston North, New Zealand)

Because of the COVID-19 pandemic, the 259th ENMC workshop on anaesthesia and neuromuscular disorders initially scheduled in Nijmegen, the Netherlands was changed to a virtual meeting. The organizers split the workshop into two sessions; a virtual meeting in December 2020 and a hybrid meeting (virtual and face-to-face if the conditions allow it) in May 2021 following the European Malignant Hyperthermia Group (EMHG) Annual Meeting in Nijmegen.

The first virtual conference gathered 26 participants from Europe and North America. Participants were medical doctors and/or researchers in the field of (paediatric) neurology, genetics and anaesthesia.

The meeting was opened by Nicol Voermans. She welcomed the participants and presented an overview of the objectives and schedule of the workshop. Main objectives were to increase and share knowledge on the anaesthetic management of patients with neuromuscular disorders and provide a consensus statement and a practical peri-operative risk tool for neuromuscular disorders and anaesthesia. Nicol Voermans and Marc Snoeck presented an introduction on the basics of “anaesthesia for the neurologist and neurology for the anaesthetists”.
Currently, the EMHG is preparing a guideline on anaesthesia in myopathic patients using a Delphi process. This guideline’s focus is mainly on anaesthesiology aspects, while the ENMC guideline will address neuromuscular neurologists and geneticists. Luc Heytens presented the current status of the EMHG guideline. Luuk van den Bersselaar presented the results of a literature review on anaesthesia and myopathies. The anaesthetic management of patients and perioperative complications reported in medical literature was reviewed. The next part of the meeting was performed in two virtual rooms with audience split in two.

In the first parallel session, Jens Reinmann presented the anaesthetic management of myotonic dystrophy. Giorgio Tasca talked about the anaesthetic management in the muscular dystrophies (e.g. Duchenne, Becker, Limb Girdle Dystrophy and Facio Scapulo Humeral Dystrophy). Oscar Díaz Cambronero’s presentation was on anaesthetic management of (acquired and congenital) neuromuscular junction disorders such as myasthenia gravis. This parallel session was chaired by Marc Snoeck and Heinz Jungbluth.

The second parallel session started with a presentation by Nicoline Løkken (with support of Mette Ørngreen) on mitochondrial and metabolic myopathies. This was followed by a presentation from Juan Jesus Vilchez about channelopathies, such as periodic paralysis. The last presentation was given by Andrea Klein about congenital myopathies and congenital muscular dystrophies. This session was chaired by Nicol Voermans and Sheila Riazi.

The last part of this meeting was a plenary session started by Marc Snoeck and Nicol Voermans. They presented the framework of the risk assessment tool. All presenters were asked to complete this tool for the specific neuromuscular disorders. Heinz Jungbluth ended the meeting by a preview about the second meeting in May. He presented four cases of patients with various neuromuscular disorders and mutations in the RYR1 gene. These mutations are associated with malignant hyperthermia, a life threatening anaesthesia complication. Heinz Jungbluth presented the difficulties with the RYR1 variants identified during diagnostic investigations in the neuromuscular clinic. This topic will be addressed next meeting.

This virtual meeting has been a great opportunity to exchange knowledge about the anaesthetic management of patients with neuromuscular disorders. The participants will work on the practical risk assessment tool for anaesthesia and neuromuscular disorders. A full report will be published after the final meeting in Neuromuscular Disorders.

References:
- M Schieren, J Defosse, A Böhmer, F Wappler, MU Gerbershagen. Anaesthetic management of patients with myopathies. Eur J Anaesthesiol. 2017 Oct;34(10):641-649.
- LR van den Bersselaar, MMJ Snoeck, M Gubbels, S Riazi, EJ Kamsteeg, H Jungbluth, NC Voermans. Anaesthesia and neuromuscular disorders: what a neurologist needs to know. Pract Neurol. 2020 Oct 27;practneurol-2020-002633.