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新型冠状病毒大流行时期神经肌肉疾病患者的管理。

Management of patients with neuromuscular disorders at the time of the SARS-CoV-2 pandemic.

机构信息

Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), Dino Ferrari Centre, University of Milan, Via Francesco Sforza 35, 20122, Milan, Italy.

Neurology Unit, Foundation IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, Italy.

出版信息

J Neurol. 2021 May;268(5):1580-1591. doi: 10.1007/s00415-020-10149-2. Epub 2020 Aug 17.

Abstract

The novel Coronavirus disease-19 (COVID-19) pandemic has posed several challenges for neuromuscular disorder (NMD) patients. The risk of a severe course of SARS-CoV-2 infection is increased in all but the mildest forms of NMDs. High-risk conditions include reduced airway clearance due to oropharyngeal weakness and risk of worsening with fever, fasting or infection Isolation requirements may have an impact on treatment regimens administered in hospital settings, such as nusinersen, glucosidase alfa, intravenous immunoglobulin, and rituximab infusions. In addition, specific drugs for SARS-CoV2 infection under investigation impair neuromuscular function significantly; chloroquine and azithromycin are not recommended in myasthenia gravis without available ventilatory support and prolonged prone positioning may influence options for treatment. Other therapeutics may affect specific NMDs (metabolic, mitochondrial, myotonic diseases) and experimental approaches for Coronavirus disease 2019 may be offered "compassionately" only after consulting the patient's NMD specialist. In parallel, the reorganization of hospital and outpatient services may change the management of non-infected NMD patients and their caregivers, favouring at-distance approaches. However, the literature on the validation of telehealth in this subgroup of patients is scant. Thus, as the first wave of the pandemic is progressing, clinicians and researchers should address these crucial open issues to ensure adequate caring for NMD patients. This manuscript summarizes available evidence so far and provides guidance for both general neurologists and NMD specialists dealing with NMD patients in the time of COVID-19.

摘要

新型冠状病毒病-19 (COVID-19) 大流行给神经肌肉疾病 (NMD) 患者带来了诸多挑战。除了最轻微的 NMD 形式外,所有形式的 NMD 患者都有感染严重 SARS-CoV-2 疾病的风险。高危情况包括因口咽肌无力而导致的气道清除能力降低,以及因发热、禁食或感染而使病情恶化的风险。隔离要求可能会对医院环境中实施的治疗方案产生影响,例如 nusinersen、葡萄糖脑苷脂酶、静脉注射免疫球蛋白和利妥昔单抗输注。此外,正在研究用于 SARS-CoV2 感染的特定药物会显著影响神经肌肉功能;氯喹和阿奇霉素在没有呼吸机支持的情况下不建议用于重症肌无力,长时间俯卧位可能会影响治疗选择。其他治疗方法可能会影响特定的 NMD(代谢性、线粒体性、肌强直性疾病),且只有在咨询患者的 NMD 专家后,才会提供针对 2019 年冠状病毒病的实验性治疗方法。与此同时,医院和门诊服务的重组可能会改变非感染性 NMD 患者及其照顾者的管理方式,有利于远程方法。然而,关于远程医疗在这组患者中验证的文献却很少。因此,随着大流行第一波的发展,临床医生和研究人员应解决这些关键的未决问题,以确保为 NMD 患者提供足够的护理。本文总结了迄今为止的可用证据,并为处理 COVID-19 期间 NMD 患者的一般神经科医生和 NMD 专家提供了指导。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b63a/8068659/de3d8b72aeb8/415_2020_10149_Fig1_HTML.jpg

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