重症肌无力
医学
临床试验
神经肌肉接头
乙酰胆碱受体
抗体
弱点
神经肌肉传递
儿科
免疫学
内科学
外科
神经科学
受体
生物
作者
Mamatha Pasnoor,Gil I. Wolfe,Richard J. Barohn
出处
期刊:Handbook of Clinical Neurology
日期:2024-01-01
卷期号:: 185-203
被引量:1
标识
DOI:10.1016/b978-0-323-90820-7.00006-9
摘要
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.
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