[Pubmed] Unusual Presentation of Double-seronegative Myasthenia Gravis with Positive Anti-LRP4 Antibody: Diagnostic Util

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Intern Med. 2024 Jun 20. doi: 10.2169/internalmedicine.3348-23. Online ahead of print.

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.

PMID:38897960 | DOI:10.2169/internalmedicine.3348-23


Source: https://pubmed.ncbi.nlm.nih.gov/3889796 ... t9+e462414