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Laryngeal Myasthenia Gravis in Voice Patients: Clinical, Serologic, and Neuromuscular Characterization of Seronegative Patients for Antibodies Against the Acetylcholine Receptor and Muscle-Specific Kinase
Journal article   Open access   Peer reviewed

Laryngeal Myasthenia Gravis in Voice Patients: Clinical, Serologic, and Neuromuscular Characterization of Seronegative Patients for Antibodies Against the Acetylcholine Receptor and Muscle-Specific Kinase

Katherine D. Mullen, Kathryn A. Mozzochi, Mary J. Hawkshaw and Robert T. Sataloff MD
Journal of voice
16 Oct 2024
DOI: https://doi.org/10.1016/j.jvoice.2024.09.038
PMID: 39419706
Featured in Collection :   Research Supported by Drexel Libraries' OA Programs
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https://doi.org/10.1016/j.jvoice.2024.09.038View
Published, Version of Record (VoR)Open Access via Drexel Libraries Read and Publish Program 2024CC BY-NC-ND V4.0,  Open

Abstract

Dysphonia Laryngeal Myasthenia Gravis Professional voice use Myasthenia Gravis
Laryngeal myasthenia gravis (MG) is a focal manifestation of MG, and most patients are seronegative for antibodies against the acetylcholine receptor (AChR) and muscle specific kinase (MuSK). The purpose of this study was to determine the incidence of anti-AChR and anti-Musk antibodies in voice patients and to characterize the clinical and neuromuscular profiles of these patients in order to guide the diagnosis of laryngeal MG. This was a retrospective case-control study that included patients over the age of 18 who underwent laryngeal electromyography as part of their evaluation for neuromuscular junction dysfunction. Cases and controls were evaluated serologically, for the anti-AChR, anti-MuSK, and anti-striational muscle antibodies, and neuromuscularly using the Tensilon test in some patients, repetitive nerve stimulation (RNS) test, and a treatment trial of pyridostigmine bromide. Cases were defined as either (1) positive anti-AChR or anti-MuSK, or (2) a positive Tensilon test or positive pyridostigmine bromide trial. Two hundred and eleven patients were screened; 61 (29%) patients were identified with laryngeal MG, and 77 (36%) patients were selected as controls. All case and control patients were seronegative for the anti-AChR and anti-MuSK antibodies with no significant difference between case and control status for seropositivity for anti-striational muscle antibodies. Of the case patients with an electrically positive Tensilon test (80.6%) who completed a treatment trial, 100% had symptom improvement. Of the case patients with a symptomatically positive Tensilon test (16.1%), only 60% of patients had improvement with a treatment trial. The RNS was more likely to be positive in case patients than control patients, and cases had a higher severity of paresis in all laryngeal muscles with LEMG evaluation. Laryngeal MG is an underrecognized condition in the otolaryngology community owing in part to its seronegative presentation. Electrical improvement with a Tensilon test, or electrical or substantial symptomatic improvement with pyridostigmine bromide represent the most robust diagnostic criteria in these patients.

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