Unusual Presentation of Double-seronegative Myasthenia Gravis with Positive Anti-LRP4 Antibody: Diagnostic Utility of a Videofluoroscopic Swallowing Study.

IF 1 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL Internal Medicine Pub Date : 2025-01-15 Epub Date: 2024-06-20 DOI:10.2169/internalmedicine.3348-23
Kensaku Yanai, Sunao Takahashi, Itsuki Soejima, Ayako Oniki, Toshiya Matsuda, Shoichiro Ishihara, Osamu Higuchi, Hiroyuki Tomimitsu
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Abstract

An 86-year-old woman was admitted to our hospital with cryptogenic progressive dyspnea and dysphagia following a tracheostomy procedure 4 months prior to presentation. She exhibited fluctuating diplopia, bilateral vocal fold paralysis, normal nerve test results, negative findings for serum anti-acetylcholine receptor and anti-muscle-specific kinase antibodies, and positive findings for anti-LDL receptor-related protein 4 (LRP4). A videofluoroscopic swallowing study (VFSS) with edrophonium revealed an improvement in bulbar paralysis. Consequently, the patient was diagnosed with double-seronegative myasthenia gravis (DSN-MG) and began immunomodulatory therapy. This case emphasizes the diagnostic challenges of bulbar-type DSN-MG and underscores the value of a VFSS with edrophonium for diagnosing this condition.

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抗 LRP4 抗体阳性的双酮阴性肌萎缩症的不寻常表现:视频荧光屏吞咽研究的诊断作用。
一名 86 岁的妇女因隐源性进行性呼吸困难和吞咽困难入院,她在入院前 4 个月接受了气管造口术。她表现为波动性复视、双侧声带麻痹、神经测试结果正常、血清抗乙酰胆碱受体抗体和抗肌肉特异性激酶抗体阴性、抗 LDL 受体相关蛋白 4(LRP4)阳性。使用依度铵进行的视频荧光吞咽检查(VFSS)显示,球麻痹有所改善。因此,患者被诊断为双阴性肌无力(DSN-MG),并开始接受免疫调节治疗。本病例强调了球部型 DSN-MG 的诊断难题,并强调了使用依度洛酮进行 VFSS 诊断的价值。
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来源期刊
Internal Medicine
Internal Medicine 医学-医学:内科
CiteScore
1.90
自引率
8.30%
发文量
0
审稿时长
2.2 months
期刊介绍: Internal Medicine is an open-access online only journal published monthly by the Japanese Society of Internal Medicine. Articles must be prepared in accordance with "The Uniform Requirements for Manuscripts Submitted to Biomedical Journals (see Annals of Internal Medicine 108: 258-265, 1988), must be contributed solely to the Internal Medicine, and become the property of the Japanese Society of Internal Medicine. Statements contained therein are the responsibility of the author(s). The Society reserves copyright and renewal on all published material and such material may not be reproduced in any form without the written permission of the Society.
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