Anaesthesia and neuromuscular disorders: what a neurologist needs to know.
Anaesthesia and neuromuscular disorders: what a neurologist needs to know.
复制标题
麻醉和神经肌肉疾病：神经科医生需要了解什么。
DOI：
10.1136/practneurol-2020-002633
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发表时间：
2020
影响因子：
2.8
通讯作者：
Voermans,NicolC
中科院分区：
文献类型：
--
作者：
vandenBersselaar,LuukR;Snoeck,MarcMJ;Gubbels,Madelief;Riazi,Sheila;Kamsteeg,Erik-Jan;Jungbluth,Heinz;Voermans,NicolC
神经科医生经常被要求对需要全身麻醉的神经肌肉疾病患者提出具体建议。然而，关于特定神经肌肉疾病的指南通常不包括关于（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，关于这一主题的医学文献大多局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，为临床神经科医生提供了关于这一主题的知识的全面和可访问的概述。术前多学科的方法，包括麻醉师，心脏病学家，胸科医生，外科医生和神经科医生是至关重要的。应始终避免使用去极化肌松药（琥珀胆碱）。必须减少非去极化肌松药的剂量，并监测其效果。RYR 1（ryanodine receptor 1）发生特异性突变的患者，以及CACNA 1 S（钙通道，电压依赖性，L型，α 1 S亚基）和STAC 3（SH 3和富含半胱氨酸的结构域3）发生突变的患者，有发生危及生命的恶性高热反应的风险。
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 inRYR1(ryanodine receptor 1) and less frequently inCACNA1S(calcium channel, voltage-dependent, L type, alpha 1S subunit) andSTAC3(SH3 and cysteine rich domain 3) are at risk of developing a life-threatening malignant hyperthermia reaction.