{"title":"抗肌肉特异性激酶抗体阳性的女性广泛性重症肌无力患者对沙丁胺醇有反应而对吡哆斯的明无反应:1例报告","authors":"Konishi Tetsuro","doi":"10.23937/2378-3001/1410090","DOIUrl":null,"url":null,"abstract":"Anti-muscle-specific kinase (MuSK) antibody positive generalized myasthenia gravis (MuSK-MG) is an autoimmune disease, in which deterioration of myasthenic symptoms may occur by cholinesterase inhibitor (ChE-I) treatment. Salbutamol, a sympathetic β2 receptor agonist, was an effective therapeutic drug in congenital myasthenic syndrome patients with MuSK gene abnormalities and in MuSK-MG animal model. Salbutamol was attempted to treat a female MuSK-MG patient who did not respond to ChE-I. Any acute benefit of salbutamol was not observed but her myasthenic symptoms gradually improved over a year without adversive effects. Salbutamol may be a therapeutic drug to alleviate myasthenic symptoms in MuSK-MG patients.","PeriodicalId":14172,"journal":{"name":"International Journal of Neurology and Neurotherapy","volume":"7 1","pages":""},"PeriodicalIF":0.0000,"publicationDate":"2019-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"A Female Patient with Anti-Muscle-Specific Kinase Antibody Positive Generalized Myasthenia Gravis Responded to Salbutamol but not Pyridostigmine: A Case Report\",\"authors\":\"Konishi Tetsuro\",\"doi\":\"10.23937/2378-3001/1410090\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Anti-muscle-specific kinase (MuSK) antibody positive generalized myasthenia gravis (MuSK-MG) is an autoimmune disease, in which deterioration of myasthenic symptoms may occur by cholinesterase inhibitor (ChE-I) treatment. Salbutamol, a sympathetic β2 receptor agonist, was an effective therapeutic drug in congenital myasthenic syndrome patients with MuSK gene abnormalities and in MuSK-MG animal model. Salbutamol was attempted to treat a female MuSK-MG patient who did not respond to ChE-I. Any acute benefit of salbutamol was not observed but her myasthenic symptoms gradually improved over a year without adversive effects. Salbutamol may be a therapeutic drug to alleviate myasthenic symptoms in MuSK-MG patients.\",\"PeriodicalId\":14172,\"journal\":{\"name\":\"International Journal of Neurology and Neurotherapy\",\"volume\":\"7 1\",\"pages\":\"\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-09-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"International Journal of Neurology and Neurotherapy\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.23937/2378-3001/1410090\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"International Journal of Neurology and Neurotherapy","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.23937/2378-3001/1410090","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
A Female Patient with Anti-Muscle-Specific Kinase Antibody Positive Generalized Myasthenia Gravis Responded to Salbutamol but not Pyridostigmine: A Case Report
Anti-muscle-specific kinase (MuSK) antibody positive generalized myasthenia gravis (MuSK-MG) is an autoimmune disease, in which deterioration of myasthenic symptoms may occur by cholinesterase inhibitor (ChE-I) treatment. Salbutamol, a sympathetic β2 receptor agonist, was an effective therapeutic drug in congenital myasthenic syndrome patients with MuSK gene abnormalities and in MuSK-MG animal model. Salbutamol was attempted to treat a female MuSK-MG patient who did not respond to ChE-I. Any acute benefit of salbutamol was not observed but her myasthenic symptoms gradually improved over a year without adversive effects. Salbutamol may be a therapeutic drug to alleviate myasthenic symptoms in MuSK-MG patients.