{"title":"重症肌无力患者抗肌肉特异性酪氨酸激酶抗体的临床特征和预后：一项回顾性研究。","authors":"Yi-Chun Chung, Jiann-Horng Yeh, Hou-Chang Chiu, Li-Ming Lien, Wen-Hung Chen","doi":"10.4103/ANT.ANT_112_0081","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Myasthenia gravis (MG) patients with anti-MuSK (muscle-specific tyrosine kinase) antibody (MuSK-MG) are uncommon. Compare with MG patient with anti-ACh (acetylcholine) receptor antibody (AChR-MG), MuSK-MG exhibit unique features.</p><p><strong>Objectives: </strong>The aim of this study is to analyze the difference of the clinical characteristics and electrophysiological features between MuSK-MG and AChR-MG.</p><p><strong>Materials and methods: </strong>We retrospectively review the medical records of generalized MG patients from 2004 to 2019 at Shin Kong Memorial Wu Ho-Su Hospital. We enrolled 47 MuSK-MG patients (33 females and 14 males), and 48 AChR-MG patient (23 females and 25 males).</p><p><strong>Results: </strong>MuSK-MG patients have a female predominance (70.2% vs. 47.9%, P = 0.027) and no significant difference to AChR-MG in onset age (44.02 ± 15.06 vs. 47.52 ± 15.85, P = 0.273). MuSK-MG are more likely to involve facial (76.6% vs. 16.7%, P < 0.001), bulbar (100% vs. 50%, P < 0.001), neck (55.3% vs. 22.9%, P = 0.001), and respiratory muscles (61.7% vs. 10.4%, P < 0.001). MuSK-MG tent to experience more severe symptoms with MGFA Class III or greater (72.3% vs. 50.0%, P = 0.032). The thymic pathology in MuSK-MG is likely to be normal (55.3% vs. 18.8%) or thymic hyperplasia (44.7% vs. 25%). The overall positive rate for repetitive nerve stimulation (RNS) in MuSK-MG patients is less than AChR-MG patients (66.0% vs. 85.4%, P = 0.027). Further analysis shows the difference is mainly by recording from trapezius muscles (51.1% vs. 79.2%, P = 0.004), whereas there is no difference in nasalis muscles (60% vs. 67.6%, P = 0.544) and abductor digiti minimi muscles (13% vs. 18.4%, P = 0.582). The positive rate for single fiber electromyography (SFEMG) recording from orbicularis oculi is very high in both MuSK-MG and AChR-MG (100% vs. 97.2%, P = 0.289). Both MuSK-MG and AChR-MG patients achieved good outcome after proper treatment (68.1% vs. 75%, P = 0.4455).</p><p><strong>Conclusions: </strong>MuSK-MG patients have a female predominance and more facial, bulbar, neck, and respiratory muscles involvements. The electrodiagnostic features of MuSK-MG are lower positive rate of RNS at trapezius and high positive rate of SFEMG at facial muscles. Although MuSK-MG patients are associated with a more progressive course, proper diagnosis and treatment still can lead to a favorable outcome.</p>","PeriodicalId":93852,"journal":{"name":"Acta neurologica Taiwanica","volume":"34 3","pages":"150-156"},"PeriodicalIF":0.0000,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Clinical Features and Outcome in Myasthenia Gravis Patients with Antimuscle-specific Tyrosine Kinase Antibody: A Retrospective Study.\",\"authors\":\"Yi-Chun Chung, Jiann-Horng Yeh, Hou-Chang Chiu, Li-Ming Lien, Wen-Hung Chen\",\"doi\":\"10.4103/ANT.ANT_112_0081\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Background: </strong>Myasthenia gravis (MG) patients with anti-MuSK (muscle-specific tyrosine kinase) antibody (MuSK-MG) are uncommon. Compare with MG patient with anti-ACh (acetylcholine) receptor antibody (AChR-MG), MuSK-MG exhibit unique features.</p><p><strong>Objectives: </strong>The aim of this study is to analyze the difference of the clinical characteristics and electrophysiological features between MuSK-MG and AChR-MG.</p><p><strong>Materials and methods: </strong>We retrospectively review the medical records of generalized MG patients from 2004 to 2019 at Shin Kong Memorial Wu Ho-Su Hospital. We enrolled 47 MuSK-MG patients (33 females and 14 males), and 48 AChR-MG patient (23 females and 25 males).</p><p><strong>Results: </strong>MuSK-MG patients have a female predominance (70.2% vs. 47.9%, P = 0.027) and no significant difference to AChR-MG in onset age (44.02 ± 15.06 vs. 47.52 ± 15.85, P = 0.273). MuSK-MG are more likely to involve facial (76.6% vs. 16.7%, P < 0.001), bulbar (100% vs. 50%, P < 0.001), neck (55.3% vs. 22.9%, P = 0.001), and respiratory muscles (61.7% vs. 10.4%, P < 0.001). MuSK-MG tent to experience more severe symptoms with MGFA Class III or greater (72.3% vs. 50.0%, P = 0.032). The thymic pathology in MuSK-MG is likely to be normal (55.3% vs. 18.8%) or thymic hyperplasia (44.7% vs. 25%). The overall positive rate for repetitive nerve stimulation (RNS) in MuSK-MG patients is less than AChR-MG patients (66.0% vs. 85.4%, P = 0.027). Further analysis shows the difference is mainly by recording from trapezius muscles (51.1% vs. 79.2%, P = 0.004), whereas there is no difference in nasalis muscles (60% vs. 67.6%, P = 0.544) and abductor digiti minimi muscles (13% vs. 18.4%, P = 0.582). The positive rate for single fiber electromyography (SFEMG) recording from orbicularis oculi is very high in both MuSK-MG and AChR-MG (100% vs. 97.2%, P = 0.289). Both MuSK-MG and AChR-MG patients achieved good outcome after proper treatment (68.1% vs. 75%, P = 0.4455).</p><p><strong>Conclusions: </strong>MuSK-MG patients have a female predominance and more facial, bulbar, neck, and respiratory muscles involvements. The electrodiagnostic features of MuSK-MG are lower positive rate of RNS at trapezius and high positive rate of SFEMG at facial muscles. Although MuSK-MG patients are associated with a more progressive course, proper diagnosis and treatment still can lead to a favorable outcome.</p>\",\"PeriodicalId\":93852,\"journal\":{\"name\":\"Acta neurologica Taiwanica\",\"volume\":\"34 3\",\"pages\":\"150-156\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2025-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Acta neurologica Taiwanica\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.4103/ANT.ANT_112_0081\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2025/9/29 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Acta neurologica Taiwanica","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.4103/ANT.ANT_112_0081","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/9/29 0:00:00","PubModel":"Epub","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
背景：重症肌无力（MG）患者携带抗麝香（肌肉特异性酪氨酸激酶）抗体（麝香-MG）并不常见。与抗乙酰胆碱受体抗体（AChR-MG）的MG患者相比，麝香-MG表现出独特的特点。目的：分析麝香- mg与AChR-MG的临床特征及电生理特征的差异。材料与方法：回顾性分析2004 - 2019年新光纪念吴浩秀医院广泛性MG患者的病历。我们招募了47例MuSK-MG患者（女性33例，男性14例）和48例AChR-MG患者（女性23例，男性25例）。结果：MuSK-MG患者以女性为主（70.2%∶47.9%,P = 0.027），与AChR-MG在发病年龄上差异无统计学意义（44.02±15.06∶47.52±15.85,P = 0.273）。MuSK-MG更可能累及面部（76.6%比16.7%,P < 0.001）、球部（100%比50%，P < 0.001）、颈部（55.3%比22.9%,P = 0.001）和呼吸肌（61.7%比10.4%,P < 0.001）。麝香- mg可能会出现更严重的MGFA III级或更高的症状（72.3%对50.0%,P = 0.032）。麝香- mg的胸腺病理可能是正常的（55.3%比18.8%）或胸腺增生（44.7%比25%）。MuSK-MG患者重复性神经刺激（RNS）总阳性率低于AChR-MG患者（66.0% vs. 85.4%, P = 0.027）。进一步分析显示，差异主要来自斜方肌（51.1%比79.2%,P = 0.004），而鼻肌（60%比67.6%,P = 0.544）和指外展肌（13%比18.4%,P = 0.582）无差异。眼轮匝肌单纤维肌电图（SFEMG）记录的MuSK-MG和AChR-MG阳性率均很高（100% vs. 97.2%, P = 0.289）。MuSK-MG和AChR-MG患者经适当治疗后均获得良好的预后（68.1% vs. 75%, P = 0.4455）。结论：麝香- mg患者以女性为主，且更多累及面部、球、颈部和呼吸肌。麝香- mg的电诊断特点是斜方肌RNS阳性率低，面肌SFEMG阳性率高。尽管MuSK-MG患者的病程更为进展，但正确的诊断和治疗仍然可以导致良好的结果。
Clinical Features and Outcome in Myasthenia Gravis Patients with Antimuscle-specific Tyrosine Kinase Antibody: A Retrospective Study.
Background: Myasthenia gravis (MG) patients with anti-MuSK (muscle-specific tyrosine kinase) antibody (MuSK-MG) are uncommon. Compare with MG patient with anti-ACh (acetylcholine) receptor antibody (AChR-MG), MuSK-MG exhibit unique features.
Objectives: The aim of this study is to analyze the difference of the clinical characteristics and electrophysiological features between MuSK-MG and AChR-MG.
Materials and methods: We retrospectively review the medical records of generalized MG patients from 2004 to 2019 at Shin Kong Memorial Wu Ho-Su Hospital. We enrolled 47 MuSK-MG patients (33 females and 14 males), and 48 AChR-MG patient (23 females and 25 males).
Results: MuSK-MG patients have a female predominance (70.2% vs. 47.9%, P = 0.027) and no significant difference to AChR-MG in onset age (44.02 ± 15.06 vs. 47.52 ± 15.85, P = 0.273). MuSK-MG are more likely to involve facial (76.6% vs. 16.7%, P < 0.001), bulbar (100% vs. 50%, P < 0.001), neck (55.3% vs. 22.9%, P = 0.001), and respiratory muscles (61.7% vs. 10.4%, P < 0.001). MuSK-MG tent to experience more severe symptoms with MGFA Class III or greater (72.3% vs. 50.0%, P = 0.032). The thymic pathology in MuSK-MG is likely to be normal (55.3% vs. 18.8%) or thymic hyperplasia (44.7% vs. 25%). The overall positive rate for repetitive nerve stimulation (RNS) in MuSK-MG patients is less than AChR-MG patients (66.0% vs. 85.4%, P = 0.027). Further analysis shows the difference is mainly by recording from trapezius muscles (51.1% vs. 79.2%, P = 0.004), whereas there is no difference in nasalis muscles (60% vs. 67.6%, P = 0.544) and abductor digiti minimi muscles (13% vs. 18.4%, P = 0.582). The positive rate for single fiber electromyography (SFEMG) recording from orbicularis oculi is very high in both MuSK-MG and AChR-MG (100% vs. 97.2%, P = 0.289). Both MuSK-MG and AChR-MG patients achieved good outcome after proper treatment (68.1% vs. 75%, P = 0.4455).
Conclusions: MuSK-MG patients have a female predominance and more facial, bulbar, neck, and respiratory muscles involvements. The electrodiagnostic features of MuSK-MG are lower positive rate of RNS at trapezius and high positive rate of SFEMG at facial muscles. Although MuSK-MG patients are associated with a more progressive course, proper diagnosis and treatment still can lead to a favorable outcome.
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