{"title":"[肌肉特异性受体酪氨酸激酶抗体阳性肌萎缩症]。","authors":"Masakatsu Motomura, Hiroko Kitanosono, Shunsuke Yoshimura, Hirokazu Shiraishi","doi":"10.11477/mf.1416202652","DOIUrl":null,"url":null,"abstract":"<p><p>Reportedly, patients with muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MG) account for approximately 3.0% of all patients with MG in Japan. Compared with patients who have acetylcholine receptor antibody-positive MG, those with MuSK antibody-positive MG show young-onset disease with female predominance, a low rate of ocular involvement (5.9%), and greater severity of dysphagia. The aforementioned types of MG are indistinguishable based on clinical symptoms and electrophysiological tests, and measurement of MuSK antibodies is essential for diagnosis. Thymectomy and complement inhibitors are not indicated for treatment, and acetylcholinesterase inhibitors, steroids, immunosuppressants, plasma exchange, intravenous immunoglobulin therapy, and neonatal Fc receptor inhibitors are used.</p>","PeriodicalId":52507,"journal":{"name":"Brain and Nerve","volume":"76 5","pages":"623-629"},"PeriodicalIF":0.0000,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Muscle-Specific Receptor Tyrosine Kinase Antibody Positive Myasthenia Gravis].\",\"authors\":\"Masakatsu Motomura, Hiroko Kitanosono, Shunsuke Yoshimura, Hirokazu Shiraishi\",\"doi\":\"10.11477/mf.1416202652\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>Reportedly, patients with muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MG) account for approximately 3.0% of all patients with MG in Japan. Compared with patients who have acetylcholine receptor antibody-positive MG, those with MuSK antibody-positive MG show young-onset disease with female predominance, a low rate of ocular involvement (5.9%), and greater severity of dysphagia. The aforementioned types of MG are indistinguishable based on clinical symptoms and electrophysiological tests, and measurement of MuSK antibodies is essential for diagnosis. Thymectomy and complement inhibitors are not indicated for treatment, and acetylcholinesterase inhibitors, steroids, immunosuppressants, plasma exchange, intravenous immunoglobulin therapy, and neonatal Fc receptor inhibitors are used.</p>\",\"PeriodicalId\":52507,\"journal\":{\"name\":\"Brain and Nerve\",\"volume\":\"76 5\",\"pages\":\"623-629\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-05-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Brain and Nerve\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.11477/mf.1416202652\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Brain and Nerve","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.11477/mf.1416202652","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
据报道,在日本,肌肉特异性激酶(MuSK)抗体阳性的重症肌无力患者约占重症肌无力患者总数的 3.0%。与乙酰胆碱受体抗体阳性的重症肌无力患者相比,肌肉特异性激酶(MuSK)抗体阳性的重症肌无力患者发病年轻,女性居多,眼部受累率低(5.9%),吞咽困难的严重程度更高。根据临床症状和电生理测试,上述类型的 MG 难以区分,因此测量 MuSK 抗体是诊断的关键。胸腺切除术和补体抑制剂不适用于治疗,可使用乙酰胆碱酯酶抑制剂、类固醇、免疫抑制剂、血浆置换、静脉注射免疫球蛋白疗法和新生儿 Fc 受体抑制剂。
Reportedly, patients with muscle-specific kinase (MuSK) antibody-positive myasthenia gravis (MG) account for approximately 3.0% of all patients with MG in Japan. Compared with patients who have acetylcholine receptor antibody-positive MG, those with MuSK antibody-positive MG show young-onset disease with female predominance, a low rate of ocular involvement (5.9%), and greater severity of dysphagia. The aforementioned types of MG are indistinguishable based on clinical symptoms and electrophysiological tests, and measurement of MuSK antibodies is essential for diagnosis. Thymectomy and complement inhibitors are not indicated for treatment, and acetylcholinesterase inhibitors, steroids, immunosuppressants, plasma exchange, intravenous immunoglobulin therapy, and neonatal Fc receptor inhibitors are used.