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急性神经肌肉无力患者的实用诊疗方法

Practical approach to the patient with acute neuromuscular weakness.

作者信息

Nayak Rajeev

机构信息

Rajeev Nayak, Department of Neurology, Jabalpur Hospital and Research Centre, Jabalpur 482002, Madhyapradesh, India.

出版信息

World J Clin Cases. 2017 Jul 16;5(7):270-279. doi: 10.12998/wjcc.v5.i7.270.

Abstract

Acute neuromuscular paralysis (ANMP) is a clinical syndrome characterized by rapid onset muscle weakness progressing to maximum severity within several days to weeks (less than 4 wk). Bulbar and respiratory muscle weakness may or may not be present. It is a common neurological emergency which requires immediate and careful investigations to determine the etiology because accurate diagnosis has significant impact on therapy and prognosis. Respiratory failure caused by neuromuscular weakness is considered as more critical than lung disease because its development may be insidious or subtle until sudden decompensation leads to life threatening hypoxia. Also, the arterial blood gas finding of severe hypoxemia, hypercapnia, and acidosis may not be apparent until respiratory failure is profound. Hence, the requirement for respiratory assistance should also be intensively and promptly investigated in all patients with neuromuscular disease. The disorder is classified based on the site of defect in motor unit pathway, ., anterior horn cells, nerve root, peripheral nerve, neuromuscular junction or muscle. Identification of the cause is primarily based on a good medical history and detailed clinical examination supplemented with neurophysiologic investigations and sometimes few specific laboratory tests. Medical history and neurological examination should be focused on the onset, progression, pattern and severity of muscle weakness as well as cranial nerves testing and tests for autonomic dysfunction. Associated non neurological features like fever, rash or other skin lesions etc. should also be noted. Globally, Guillain-Barré syndrome is the most frequent cause of ANMP and accounts for the majority of cases of respiratory muscles weakness associated with neuromuscular disorders. Newly acquired neuromuscular weakness in intensive care unit patients consist of critical illness polyneuropathy, critical illness myopathy and drug induced neuromuscular weakness which may arise as a consequence of sepsis, multi-organ failure, and exposure to certain medications like intravenous corticosteroids and neuromuscular blocking agents.

摘要

急性神经肌肉麻痹(ANMP)是一种临床综合征,其特征为肌肉无力迅速发作,在数天至数周内(少于4周)发展至最严重程度。延髓和呼吸肌无力可能存在,也可能不存在。这是一种常见的神经科急症,需要立即进行仔细检查以确定病因,因为准确诊断对治疗和预后有重大影响。由神经肌肉无力引起的呼吸衰竭被认为比肺部疾病更危急,因为其发展可能隐匿或不明显,直到突然失代偿导致危及生命的低氧血症。此外,严重低氧血症、高碳酸血症和酸中毒的动脉血气结果可能直到呼吸衰竭严重时才会明显。因此,对于所有神经肌肉疾病患者,也应积极、及时地调查其呼吸辅助需求。该疾病根据运动单位通路中的缺陷部位进行分类,即前角细胞、神经根、周围神经、神经肌肉接头或肌肉。病因的确定主要基于良好的病史和详细的临床检查,并辅以神经生理学检查,有时还需要一些特定的实验室检查。病史和神经系统检查应关注肌肉无力的发作、进展、模式和严重程度,以及颅神经检查和自主神经功能障碍检查。还应注意相关的非神经学特征,如发热、皮疹或其他皮肤病变等。在全球范围内,吉兰-巴雷综合征是ANMP最常见的病因,也是与神经肌肉疾病相关的呼吸肌无力病例中的大多数病因。重症监护病房患者新出现的神经肌肉无力包括危重病性多发性神经病、危重病性肌病和药物性神经肌肉无力,这些可能是由败血症、多器官衰竭以及接触某些药物(如静脉注射皮质类固醇和神经肌肉阻滞剂)引起的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/001e/5535318/f43ab9447775/WJCC-5-270-g001.jpg

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