{"title":"抗 MuSK 抗体阳性的重症肌无力患者的垂头综合征可能并发轴性肌病","authors":"So Okubo, Mitsuhiro Kainaga, Shin-ichi Tokushige, Ayumi Uchibori, Chizuko Oishi, Teruyuki Hirano, Yaeko Ichikawa","doi":"10.1111/cen3.12782","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Background</h3>\n \n <p>Dropped head syndrome (DHS) is a group of disorders that result in anterior neck flexion. Myasthenia gravis (MG) is a common cause of DHS. Previous reports have suggested that concurrent myopathy involving the paraspinal musculature may underlie DHS in patients with anti-acetylcholine receptor antibody-positive or thymoma-associated MG.</p>\n </section>\n \n <section>\n \n <h3> Case Presentation</h3>\n \n <p>A 64-year-old woman presented with a 3-month history of head drop and lordosis. Neurological examination revealed bilateral ptosis and weakness of the posterior neck extensors. Electrophysiology suggested neuromuscular junction involvement. Serum anti-MuSK antibodies were positive, and generalized anti-MuSK antibody-positive myasthenia gravis (MuSK-MG) was diagnosed. Needle electromyography (nEMG) of the splenius capitus and paraspinal muscles revealed acute and chronic myogenic changes. Imaging suggested paraspinal muscle atrophy. nEMG and magnetic resonance imaging (MRI) of both extremities were normal, and autoimmune myopathy-related antibody testing was negative.</p>\n </section>\n \n <section>\n \n <h3> Conclusion</h3>\n \n <p>Thymoma-negative MuSK-MG, demonstrating treatment-refractory DHS, may present with concurrent axial myopathy.</p>\n </section>\n </div>","PeriodicalId":10193,"journal":{"name":"Clinical and Experimental Neuroimmunology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2024-02-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Dropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy\",\"authors\":\"So Okubo, Mitsuhiro Kainaga, Shin-ichi Tokushige, Ayumi Uchibori, Chizuko Oishi, Teruyuki Hirano, Yaeko Ichikawa\",\"doi\":\"10.1111/cen3.12782\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Background</h3>\\n \\n <p>Dropped head syndrome (DHS) is a group of disorders that result in anterior neck flexion. Myasthenia gravis (MG) is a common cause of DHS. Previous reports have suggested that concurrent myopathy involving the paraspinal musculature may underlie DHS in patients with anti-acetylcholine receptor antibody-positive or thymoma-associated MG.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Case Presentation</h3>\\n \\n <p>A 64-year-old woman presented with a 3-month history of head drop and lordosis. Neurological examination revealed bilateral ptosis and weakness of the posterior neck extensors. Electrophysiology suggested neuromuscular junction involvement. Serum anti-MuSK antibodies were positive, and generalized anti-MuSK antibody-positive myasthenia gravis (MuSK-MG) was diagnosed. Needle electromyography (nEMG) of the splenius capitus and paraspinal muscles revealed acute and chronic myogenic changes. Imaging suggested paraspinal muscle atrophy. nEMG and magnetic resonance imaging (MRI) of both extremities were normal, and autoimmune myopathy-related antibody testing was negative.</p>\\n </section>\\n \\n <section>\\n \\n <h3> Conclusion</h3>\\n \\n <p>Thymoma-negative MuSK-MG, demonstrating treatment-refractory DHS, may present with concurrent axial myopathy.</p>\\n </section>\\n </div>\",\"PeriodicalId\":10193,\"journal\":{\"name\":\"Clinical and Experimental Neuroimmunology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-02-18\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical and Experimental Neuroimmunology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://onlinelibrary.wiley.com/doi/10.1111/cen3.12782\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Immunology and Microbiology\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical and Experimental Neuroimmunology","FirstCategoryId":"1085","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/cen3.12782","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Immunology and Microbiology","Score":null,"Total":0}
Dropped head syndrome in anti-MuSK antibody-positive myasthenia gravis with possible concurrent axial myopathy
Background
Dropped head syndrome (DHS) is a group of disorders that result in anterior neck flexion. Myasthenia gravis (MG) is a common cause of DHS. Previous reports have suggested that concurrent myopathy involving the paraspinal musculature may underlie DHS in patients with anti-acetylcholine receptor antibody-positive or thymoma-associated MG.
Case Presentation
A 64-year-old woman presented with a 3-month history of head drop and lordosis. Neurological examination revealed bilateral ptosis and weakness of the posterior neck extensors. Electrophysiology suggested neuromuscular junction involvement. Serum anti-MuSK antibodies were positive, and generalized anti-MuSK antibody-positive myasthenia gravis (MuSK-MG) was diagnosed. Needle electromyography (nEMG) of the splenius capitus and paraspinal muscles revealed acute and chronic myogenic changes. Imaging suggested paraspinal muscle atrophy. nEMG and magnetic resonance imaging (MRI) of both extremities were normal, and autoimmune myopathy-related antibody testing was negative.
Conclusion
Thymoma-negative MuSK-MG, demonstrating treatment-refractory DHS, may present with concurrent axial myopathy.
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