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[灾难性神经肌肉疾病]

[Catastrophic neuromuscular diseases].

作者信息

Muñoz Blanco J L

机构信息

Unidad ELA-Neuromuscular, Servicio de Neurología, Hospital General Universitario Gregorio Marañón, Madrid, España.

出版信息

Neurologia. 2010 Oct;25 Suppl 1:37-45. doi: 10.1016/S0213-4853(10)70049-9.

Abstract

Neurologists should anticipate and recognize the onset of respiratory failure in patients with neuromuscular diseases. Symptoms vary according to the speed of onset of respiratory muscle weakness. Catastrophic situations usually occur in three clinical scenarios: 1) incorrect management of acute respiratory failure of neuromuscular origin, autonomic dysfunction or during general anaesthesia of patients with neuromuscular diseases ; 2) incorrect prognosis and treatment due to the lack of a correct diagnosis. This situation is more common in ventilated patients with associated muscular weakness, acute neuropathies or motor neuron disease, and 3) inappropriate medical intervention in patients with neuromuscular disease with a definitive diagnosis but longstanding disease (amyotrophic lateral sclerosis, spinal muscular atrophy, myotonic dystrophy and other muscular dystrophies).

摘要

神经科医生应预判并识别神经肌肉疾病患者呼吸衰竭的发作。症状因呼吸肌无力发作的速度而异。灾难性情况通常发生在三种临床场景中:1)神经肌肉源性急性呼吸衰竭、自主神经功能障碍或神经肌肉疾病患者全身麻醉期间的管理不当;2)由于诊断错误导致预后和治疗不当。这种情况在伴有肌无力、急性神经病或运动神经元病的通气患者中更为常见;3)对已明确诊断但患有长期疾病(肌萎缩侧索硬化、脊髓性肌萎缩、强直性肌营养不良和其他肌肉营养不良)的神经肌肉疾病患者进行不适当的医疗干预。

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