259th ENMC international workshop: Anaesthesia and neuromuscular disorders 11 December, 2020 and 28–29 May, 2021

Bersselaar, Luuk R. van den; Riazi, Sheila; Snoeck, Mmj; Jungbluth, Heinz; Voermans, N.C.

doi:10.1016/j.nmd.2021.11.005

Cited by 7 publications

(4 citation statements)

References 70 publications

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“…In individuals suffering from junctional disorders, such as MG, LEMS, and MD, the evaluation of serum electrolyte levels, such as calcium, magnesium, potassium, and phosphate, is recommended ( 2 ). It is also required to keep in mind the difficulties in temperature control and blood glucose regulation due to reduced muscle mass ( 7 , 8 ).…”

Section: Resultsmentioning

confidence: 99%

A Review on the Anesthetic Management of Patients with Neuromuscular Diseases

et al. 2023

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Context: Neuromuscular diseases (NMDs) are regarded as a clinically and genetically heterogeneous group of diseases characterized by weakening muscle strength and dystrophic changes in the muscle. Due to the nature of these diseases, it can be challenging for anesthesiologists to provide appropriate pain medications, symptom management, and other necessary techniques that are implemented to anesthetize the patient properly. Evidence Acquisition: This study was based on the available literature and the authors’ experience. The current study aimed to review the available anesthesia for patients suffering from NMDs. The search process resulted in the detection of relevant articles using valid keywords on electronic databases, including Embase, PubMed, Scopus, Web of Science, and Cochrane Library. Subsequently, 24 articles published between 2009 to 2022 were identified as eligible for this review. Results: When anesthetizing a patient with NMD, special attention should be paid to preoperative evaluation, medical-history taking, risk of difficult intubation or cardiac incidents, respiratory insufficiency, and frequent pulmonary infections. It is also necessary to keep in mind that these patients are at risk of prolonged paralysis, hyperkalemia, rigidity, malignant hyperthermia, cardiac arrest, rhabdomyolysis, or even death. Conclusions: Problems of anesthesia in patients with NMDs arise from the nature of the condition itself and the interaction of anesthetics and muscle relaxants with anticholinesterase drugs used in therapy. Each patient’s individual risk should be assessed before anesthesia. Therefore, it is important (and even necessary before major surgery) to perform a thorough preoperative examination to not only determine perioperative risk but also to ensure optimal perioperative follow-up.

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Section: Resultsmentioning

confidence: 99%

A Review on the Anesthetic Management of Patients with Neuromuscular Diseases

et al. 2023

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“…The comprehensive workshop report has been published separately [ 6 ] but did not allow for the inclusion of the full anaesthesia considerations included in this paper.…”

Section: Methodsmentioning

confidence: 99%

“…The ENMC research board approved the participants list. The virtual workshop consisted of three parts: The comprehensive workshop report has been published separately [6] but did not allow for the inclusion of the full anaesthesia considerations included in this paper.…”

Section: Me Thods Enmc Workhopmentioning

confidence: 99%

“…We therefore present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with NMDs as discussed and formulated during the 259th ENMC Workshop on Anaesthesia in Neuromuscular Disorders [ 6 ]. This consensus statement provides a comprehensive and accessible overview with the aim of guiding neurologists and anaesthesiologists, improving perioperative safety, and optimizing risk assessment and appropriate management of these patients.…”

Section: Introductionmentioning

confidence: 99%

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European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

Bersselaar

Heytens

Silva

et al. 2022

Euro J of Neurology

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Background and purpose Patients with neuromuscular conditions are at increased risk of suffering perioperative complications related to anaesthesia. There is currently little specific anaesthetic guidance concerning these patients. Here, we present the European Neuromuscular Centre (ENMC) consensus statement on anaesthesia in patients with neuromuscular disorders as formulated during the 259th ENMC Workshop on Anaesthesia in Neuromuscular Disorders. Methods International experts in the field of (paediatric) anaesthesia, neurology, and genetics were invited to participate in the ENMC workshop. A literature search was conducted in PubMed and Embase, the main findings of which were disseminated to the participants and presented during the workshop. Depending on specific expertise, participants presented the existing evidence and their expert opinion concerning anaesthetic management in six specific groups of myopathies and neuromuscular junction disorders. The consensus statement was prepared according to the AGREE II (Appraisal of Guidelines for Research & Evaluation) reporting checklist. The level of evidence has been adapted according to the SIGN (Scottish Intercollegiate Guidelines Network) grading system. The final consensus statement was subjected to a modified Delphi process. Results A set of general recommendations valid for the anaesthetic management of patients with neuromuscular disorders in general have been formulated. Specific recommendations were formulated for (i) neuromuscular junction disorders, (ii) muscle channelopathies (nondystrophic myotonia and periodic paralysis), (iii) myotonic dystrophy (types 1 and 2), (iv) muscular dystrophies, (v) congenital myopathies and congenital dystrophies, and (vi) mitochondrial and metabolic myopathies. Conclusions This ENMC consensus statement summarizes the most important considerations for planning and performing anaesthesia in patients with neuromuscular disorders.

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Cellular mechanisms of acute rhabdomyolysis in inherited metabolic diseases

de Calbiac,

Imbard,

de Lonlay

2024

J of Inher Metab Disea

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Acute rhabdomyolysis (RM) constitutes a life‐threatening emergency resulting from the (acute) breakdown of skeletal myofibers, characterized by a plasma creatine kinase (CK) level exceeding 1000 IU/L in response to a precipitating factor. Genetic predisposition, particularly inherited metabolic diseases, often underlie RM, contributing to recurrent episodes. Both sporadic and congenital forms of RM share common triggers. Considering the skeletal muscle's urgent need to rapidly adjust to environmental cues, sustaining sufficient energy levels and functional autophagy and mitophagy processes are vital for its preservation and response to stressors. Crucially, the composition of membrane lipids, along with lipid and calcium transport, and the availability of adenosine triphosphate (ATP), influence membrane biophysical properties, membrane curvature in skeletal muscle, calcium channel signaling regulation, and determine the characteristics of autophagic organelles. Consequently, a genetic defect involving ATP depletion, aberrant calcium release, abnormal lipid metabolism and/or lipid or calcium transport, and/or impaired anterograde trafficking may disrupt autophagy resulting in RM. The complex composition of lipid membranes also alters Toll‐like receptor signaling and viral replication. In response, infections, recognized triggers of RM, stimulate increased levels of inflammatory cytokines, affecting skeletal muscle integrity, energy metabolism, and cellular trafficking, while elevated temperatures can reduce the activity of thermolabile enzymes. Overall, several mechanisms can account for RMs and may be associated in the same disease‐causing RM.

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259th ENMC international workshop: Anaesthesia and neuromuscular disorders 11 December, 2020 and 28–29 May, 2021

Cited by 7 publications

References 70 publications

A Review on the Anesthetic Management of Patients with Neuromuscular Diseases

A Review on the Anesthetic Management of Patients with Neuromuscular Diseases

European Neuromuscular Centre consensus statement on anaesthesia in patients with neuromuscular disorders

Cellular mechanisms of acute rhabdomyolysis in inherited metabolic diseases

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