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Heterogeneous Presentations and Serologies in Myasthenia Gravis Patients Presenting with Dysphagia.
Moffatt, Clare; Pillutla, Pranati; Soltanzadeh, Payam; Chhetri, Dinesh K.
Afiliación
  • Moffatt C; Department of Head & Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A.
  • Pillutla P; Department of Head & Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A.
  • Soltanzadeh P; Department of Neurology, David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A.
  • Chhetri DK; Department of Head & Neck Surgery, David Geffen School of Medicine at UCLA, Los Angeles, California, U.S.A.
Laryngoscope ; 2024 Jul 01.
Article en En | MEDLINE | ID: mdl-38949061
ABSTRACT

INTRODUCTION:

Myasthenia gravis (MG) is an autoimmune disease that affects the neuromuscular junction. MG patients may present de novo with primary otolaryngology complaints, including swallowing dysfunction. This study describes a range of unique presentations and rare diagnostic serologies, which have not previously been fully described.

METHODS:

A retrospective review was performed of all patients presenting with primary symptom of dysphagia and subsequently diagnosed with MG. Data collected included demographics, clinical presentation, swallow studies, serology, imaging, treatment, and response.

RESULTS:

Five patients met the inclusion criteria. Four endorsed dysphagia as primary complaint and one endorsed dysphagia and dysphonia. All patients underwent in-office swallow evaluations that showed vallecular or pyriform sinus residue. Three patients completed modified barium swallow studies that showed pharyngeal weakness and epiglottic dysfunction in all, and upper esophageal sphincter dysfunction in two. One patient with additional symptom of dyspnea was admitted and found to be in myasthenic crisis. Upon serologic evaluation, three patients were positive for acetylcholine receptor (AChR) antibodies only, one for muscle-specific-kinase (MuSK) antibodies only, and one for low density lipoprotein receptor-related protein 4 (LRP4) antibodies only. All patients received neurology evaluation and were treated with steroids, pyridostigmine, plasma exchange, or rituximab. In three patients with over 1 year follow-up, symptoms were significantly improved or resolved.

CONCLUSION:

MG is an important differential diagnosis in patients with unexplained pharyngeal dysphagia. While workup can include AChR antibody screening, in seronegative patients with persistent symptoms, additional testing for MuSK and LRP4 may lead to diagnosis and effective treatment. LEVEL OF EVIDENCE Level 4 Laryngoscope, 2024.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Laryngoscope Asunto de la revista: OTORRINOLARINGOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Laryngoscope Asunto de la revista: OTORRINOLARINGOLOGIA Año: 2024 Tipo del documento: Article País de afiliación: Estados Unidos Pais de publicación: Estados Unidos