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改善重症肌无力和吉兰-巴雷综合征的治疗效果。

Improving Outcome in Severe Myasthenia Gravis and Guillain-Barré Syndrome.

作者信息

Hawkes Maximiliano A, Wijdicks Eelco F M

机构信息

Department of Neurology, Neurocritical Care Services, Mayo Clinic, Rochester, Minnesota.

出版信息

Semin Neurol. 2024 Jun;44(3):263-270. doi: 10.1055/s-0044-1785509. Epub 2024 Apr 1.

DOI:10.1055/s-0044-1785509
PMID:38560985
Abstract

When progressive and severe, myasthenia gravis and Guillain-Barré syndrome may have the potential for fatal and unfavorable clinical outcomes. Regardless of important differences in their clinical course, the development of weakness of oropharyngeal muscles and respiratory failure with requirement of mechanical ventilation is the main driver of poor prognosis in both conditions. The need for prolonged mechanical ventilation is particularly relevant because it immobilizes the patient and care becomes extraordinarily complex due to daily risks of systemic complications. Additionally, patients with myasthenia gravis often require long-term immunosuppressive treatments with associated toxicity and infectious risks. Unlike myasthenia gravis, the recovery period is prolonged in Guillain-Barré syndrome, but often favorable, even in the more severely affected patients. Outcome, for a large part, is determined by expert neurocritical care.

摘要

重症肌无力和吉兰 - 巴雷综合征病情进展严重时,可能会导致致命和不良的临床结局。尽管它们在临床病程上存在重要差异,但口咽肌无力和呼吸衰竭并需要机械通气是这两种疾病预后不良的主要驱动因素。需要长时间机械通气尤为关键,因为这会使患者活动受限,且由于全身并发症的日常风险,护理变得异常复杂。此外,重症肌无力患者通常需要长期免疫抑制治疗,存在相关毒性和感染风险。与重症肌无力不同,吉兰 - 巴雷综合征的恢复期较长,但即便在病情严重的患者中,恢复情况通常也较好。在很大程度上,结局取决于专业的神经重症护理。

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