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

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-acet...

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Bibliographic Details
Published in:Internal Medicine 2024, pp.3348-23
Main Authors: Yanai, Kensaku, Takahashi, Sunao, Soejima, Itsuki, Oniki, Ayako, Matsuda, Toshiya, Ishihara, Shoichiro, Higuchi, Osamu, Tomimitsu, Hiroyuki
Format: Article
Language:English
Subjects:
anti-LDL receptor-related protein 4
Bulbar paralysis
Double seronegative myasthenia gravis
Dysphagia
videofluoroscopic swallowing study
vocal fold paralysis
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Summary: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.
ISSN:0918-2918
1349-7235
1349-7235
DOI:10.2169/internalmedicine.3348-23