Greene-Chandos Diana, Torbey Michel
Continuum (Minneap Minn). 2018 Dec;24(6):1753-1775. doi: 10.1212/CON.0000000000000682.
Weakness is a common reason patients are seen in neurologic consultation. This article reviews the differential diagnosis of neuromuscular disorders in the intensive care unit (ICU), discusses the intensive care needs and evaluation of respiratory failure in patients with neuromuscular disorders, and provides a practical guide for management.
Although primary neuromuscular disorders used to be the most common cause for weakness from peripheral nervous system disease in the ICU, a shift toward ICU-acquired weakness is observed in today's clinical practice. Therefore, determining the cause of weakness is important and may have significant prognostic implications. Guillain-Barré syndrome and myasthenia gravis remain the most common primary neuromuscular disorders in the ICU. In patients with myasthenia gravis, it is important to be vigilant with the airway and institute noninvasive ventilation early in the course of the disease to attempt to avoid the need for intubation. On the other hand, patients with Guillain-Barré syndrome should be intubated without delay if the airway is at risk to avoid further complications. In patients with ICU-acquired weakness, failure to wean from the ventilator is usually the challenge. Early mobility, glucose control, minimizing sedation, and avoiding neuromuscular blocking agents remain the only therapeutic regimen available for ICU-acquired weakness.
Critical care management of neuromuscular disorders requires a multidisciplinary approach engaging members of the ICU and consultative teams. Developing an airway management protocol could have implications on outcome and length of stay for patients with neuromuscular disorders in the ICU. Tending to the appropriate nuances of each patient who is critically ill with a neuromuscular disorder through evidence-based medicine can also have implications on length of stay and outcome.
肌无力是患者寻求神经科会诊的常见原因。本文综述了重症监护病房（ICU）中神经肌肉疾病的鉴别诊断，讨论了神经肌肉疾病患者呼吸衰竭的重症监护需求及评估，并提供了实用的管理指南。
尽管原发性神经肌肉疾病曾是ICU中周围神经系统疾病导致肌无力的最常见原因，但在当今临床实践中，已观察到向ICU获得性肌无力的转变。因此，确定肌无力的病因很重要，可能具有重大的预后意义。吉兰-巴雷综合征和重症肌无力仍然是ICU中最常见的原发性神经肌肉疾病。对于重症肌无力患者，在疾病过程中要警惕气道情况，并尽早进行无创通气，以尽量避免插管的需要。另一方面，如果气道有风险，吉兰-巴雷综合征患者应立即插管，以避免进一步的并发症。对于ICU获得性肌无力患者，脱机困难通常是一个挑战。早期活动、控制血糖、尽量减少镇静以及避免使用神经肌肉阻滞剂仍然是治疗ICU获得性肌无力的唯一可用治疗方案。
神经肌肉疾病的重症监护管理需要ICU团队和会诊团队成员采取多学科方法。制定气道管理方案可能会对ICU中神经肌肉疾病患者的预后和住院时间产生影响。通过循证医学关注每一位患有神经肌肉疾病的重症患者的适当细微差别，也可能会对住院时间和预后产生影响。
