{"title":"重症肌无力","authors":"Mamatha Pasnoor, Gil I Wolfe, Richard J Barohn","doi":"10.1016/B978-0-323-90820-7.00006-9","DOIUrl":null,"url":null,"abstract":"<p><p>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.</p>","PeriodicalId":12907,"journal":{"name":"Handbook of clinical neurology","volume":"203 ","pages":"185-203"},"PeriodicalIF":0.0000,"publicationDate":"2024-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Myasthenia gravis.\",\"authors\":\"Mamatha Pasnoor, Gil I Wolfe, Richard J Barohn\",\"doi\":\"10.1016/B978-0-323-90820-7.00006-9\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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.</p>\",\"PeriodicalId\":12907,\"journal\":{\"name\":\"Handbook of clinical neurology\",\"volume\":\"203 \",\"pages\":\"185-203\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Handbook of clinical neurology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1016/B978-0-323-90820-7.00006-9\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q2\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Handbook of clinical neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1016/B978-0-323-90820-7.00006-9","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"Medicine","Score":null,"Total":0}
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.
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.