Hutchinson David, Whyte Ken
Department of Neurology, Auckland City Hospital, Auckland, New Zealand.
Pract Neurol. 2008 Aug;8(4):229-37. doi: 10.1136/pn.2008.152611.
Neurologists should be able to anticipate and recognise the onset of respiratory failure in patients with neuromuscular disorders. Symptoms will differ depending on the speed of onset of the respiratory muscle weakness. Careful monitoring of respiratory function is particularly important in acute disorders such as Guillain-Barré syndrome. Patients with an unrecognised neuromuscular disorder may occasionally present with respiratory failure. Important investigations include vital capacity, mouth pressures, arterial blood gases, chest x ray and sometimes overnight respiratory monitoring. Patients with Guillain-Barré and other acute conditions may require short-term ventilatory support in the intensive care unit. Patients with some chronic disorders, such as motor neuron disease and Duchenne dystrophy, can be successfully treated with non-invasive ventilation, usually in collaboration with a respiratory physician. New-onset weakness of limb and respiratory muscles in the intensive care unit is usually due to critical illness myopathy or critical illness polyneuropathy, and treatment is supportive.
神经科医生应能够预见并识别神经肌肉疾病患者呼吸衰竭的发作。症状会因呼吸肌无力发作的速度而有所不同。在诸如吉兰 - 巴雷综合征等急性疾病中，仔细监测呼吸功能尤为重要。患有未被识别的神经肌肉疾病的患者偶尔可能会出现呼吸衰竭。重要的检查包括肺活量、口腔压力、动脉血气、胸部X光，有时还包括夜间呼吸监测。患有吉兰 - 巴雷综合征和其他急性疾病的患者可能需要在重症监护病房接受短期通气支持。患有一些慢性疾病的患者，如运动神经元病和杜氏肌营养不良症，通常可在呼吸科医生的协作下通过无创通气成功治疗。重症监护病房中肢体和呼吸肌无力的新发情况通常是由危重病性肌病或危重病性多发性神经病引起的，治疗以支持治疗为主。
