Rabinstein Alejandro A
Continuum (Minneap Minn). 2015 Oct;21(5 Neurocritical Care):1324-45. doi: 10.1212/CON.0000000000000218.
Neurologists working in the hospital are often called to evaluate patients with severe muscle weakness. Some of these patients can develop ventilatory compromise and require admission to the intensive care unit (ICU). This article reviews the general evaluation of neuromuscular respiratory failure, discusses its differential diagnosis, and provides practical advice on the management of its most common causes.
Determining the cause of acute neuromuscular respiratory failure is crucial because functional prognosis is poor in patients for whom the cause cannot be defined. The differential diagnosis is extensive, but the first step is to discriminate between cases related to a primary neurologic disease (primary neuromuscular respiratory failure) and those provoked by systemic disease, most often critical illness from sepsis and multiorgan failure (secondary neuromuscular respiratory failure). Guillain-Barré syndrome (GBS) and myasthenic crisis are the two most frequent causes of primary neuromuscular respiratory failure. Although they are both autoimmune conditions that benefit from the administration of plasma exchange or IV immunoglobulin (IVIg), they are otherwise very different disorders with unique features and distinct complications. Optimal strategies for mechanical ventilation also differ between these two conditions; while myasthenic crisis is ideally managed with noninvasive bilevel positive airway pressure (BiPAP) ventilation, GBS demands early intubation.
Prompt recognition of neuromuscular respiratory failure can be lifesaving, and identification of its cause has substantial prognostic implications. Adequate management of these patients requires a multidisciplinary team with the neurologist at its center, not only to guide the diagnostic evaluation but often also to prescribe the optimal management.
在医院工作的神经科医生经常被要求评估患有严重肌肉无力的患者。其中一些患者可能会出现通气功能受损，需要入住重症监护病房（ICU）。本文综述了神经肌肉性呼吸衰竭的一般评估，讨论了其鉴别诊断，并为其最常见病因的管理提供实用建议。
确定急性神经肌肉性呼吸衰竭的病因至关重要，因为病因不明的患者功能预后较差。鉴别诊断范围广泛，但第一步是区分与原发性神经系统疾病相关的病例（原发性神经肌肉性呼吸衰竭）和由全身性疾病引发的病例，最常见的是败血症和多器官功能衰竭导致的危重病（继发性神经肌肉性呼吸衰竭）。格林-巴利综合征（GBS）和重症肌无力危象是原发性神经肌肉性呼吸衰竭最常见的两个病因。尽管它们都是自身免疫性疾病，都受益于血浆置换或静脉注射免疫球蛋白（IVIg）治疗，但它们是非常不同的疾病，具有独特的特征和不同的并发症。这两种疾病的最佳机械通气策略也有所不同；重症肌无力危象理想的治疗方法是无创双水平气道正压（BiPAP）通气，而GBS则需要早期插管。
迅速识别神经肌肉性呼吸衰竭可能挽救生命，确定其病因具有重要的预后意义。对这些患者进行充分管理需要一个以神经科医生为核心的多学科团队，不仅要指导诊断评估，而且通常还要制定最佳治疗方案。
