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.

中文翻译：

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麻醉和神经肌肉疾病：神经科医生需要了解什么

神经科医生经常被要求提供有关需要全身麻醉的神经肌肉疾病患者的具体建议。然而，关于特定神经肌肉疾病的指南通常不包括有关（区域和/或全身）麻醉或程序镇静的具体指南或实用建议。此外，有关该主题的医学文献大多仅限于麻醉学期刊上的出版物。因此，我们总结了不同神经肌肉疾病麻醉的一般建议和具体建议，以提供对临床神经科医生至关重要的该主题知识的全面且易于理解的概述。包括麻醉师、心脏病专家、胸科医生、外科医生和神经科医生在内的术前多学科方法至关重要。应始终避免使用去极化肌肉松弛剂（琥珀胆碱）。必须减少非去极化肌肉松弛剂的剂量并监测其效果。RYR1（兰尼碱受体 1）具有特定突变且 CACNA1S（钙通道，电压依赖性，L 型，α1S 亚基）和 STAC3（SH3 和富含半胱氨酸结构域 3）突变较少的患者有发生危及生命的风险恶性高热反应。