Myasthenia gravis.

Q2 Medicine
Mamatha Pasnoor, Gil I Wolfe, Richard J Barohn
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引用次数: 0

Abstract

Myasthenia gravis (MG) is a rare neuromuscular junction disorder that is characterized by fatigable weakness of muscles. People with MG experience various clinical manifestations based on the muscles involved. MG can be autoimmune, paraneoplastic, congenital, medication-related, or transient in the neonatal period due to the passive placental transfer of antibodies from mothers with MG. Acetylcholine receptor antibodies are seen in the majority of patients with MG. However, other antibodies have been discovered in the last 20 years, including muscle-specific tyrosine kinase (MuSK) and lipoprotein-related peptide 4 (LRP4), and are now available through commercial testing. More recently, a handful of other antibodies have been associated with MG; however, they are not presently available for routine testing. A disease classification system has been developed by the Myasthenia Gravis Foundation of America (MGFA) and is commonly used worldwide. A number of objective and subjective outcome measures have been developed and validated over the years and have been proven useful for both clinical and research purposes, serving as primary and secondary outcome measures in most clinical trials. A growing number of therapies are available for both acute and chronic management of MG, with several new mechanistic approaches under investigation. An international consensus guidance for the management of MG was first published in 2016 and updated in 2020.

重症肌无力
重症肌无力(MG)是一种罕见的神经肌肉接头疾病,其特征是肌肉疲劳性无力。肌无力症患者的临床表现因涉及的肌肉不同而各异。肌肉萎缩症可能是自身免疫性疾病、副肿瘤性疾病、先天性疾病、药物相关疾病,也可能是由于患有肌肉萎缩症的母亲将抗体从胎盘被动转移到新生儿期而导致的一过性疾病。乙酰胆碱受体抗体可见于大多数 MG 患者。然而,在过去的 20 年中还发现了其他抗体,包括肌肉特异性酪氨酸激酶(MuSK)和脂蛋白相关肽 4(LRP4),这些抗体现在都可以通过商业检测获得。最近,少数其他抗体也与 MG 有关,但目前还不能用于常规检测。美国重症肌无力基金会(MGFA)制定了一套疾病分类系统,并在全球范围内普遍使用。多年来,一些客观和主观的结果测量方法已被开发和验证,并被证明可用于临床和研究目的,可作为大多数临床试验的主要和次要结果测量方法。目前有越来越多的疗法可用于 MG 的急性和慢性治疗,还有几种新的机理方法正在研究中。2016年首次发布了MG治疗的国际共识指南,并于2020年进行了更新。
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来源期刊
Handbook of clinical neurology
Handbook of clinical neurology Medicine-Neurology (clinical)
CiteScore
4.10
自引率
0.00%
发文量
302
期刊介绍: The Handbook of Clinical Neurology (HCN) was originally conceived and edited by Pierre Vinken and George Bruyn as a prestigious, multivolume reference work that would cover all the disorders encountered by clinicians and researchers engaged in neurology and allied fields. The first series of the Handbook (Volumes 1-44) was published between 1968 and 1982 and was followed by a second series (Volumes 45-78), guided by the same editors, which concluded in 2002. By that time, the Handbook had come to represent one of the largest scientific works ever published. In 2002, Professors Michael J. Aminoff, François Boller, and Dick F. Swaab took on the responsibility of supervising the third (current) series, the first volumes of which published in 2003. They have designed this series to encompass both clinical neurology and also the basic and clinical neurosciences that are its underpinning. Given the enormity and complexity of the accumulating literature, it is almost impossible to keep abreast of developments in the field, thus providing the raison d''être for the series. The series will thus appeal to clinicians and investigators alike, providing to each an added dimension. Now, more than 140 volumes after it began, the Handbook of Clinical Neurology series has an unparalleled reputation for providing the latest information on fundamental research on the operation of the nervous system in health and disease, comprehensive clinical information on neurological and related disorders, and up-to-date treatment protocols.
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