Myopathies featuring early or prominent dysphagia

James D. Triplett, Marcus V. Pinto, Emily A. Hosfield, Margherita Milone, Teerin Liewluck

Research output: Contribution to journalArticle

Abstract

Background: Limited data exist regarding myopathies with early or prominent dysphagia. Methods: A retrospective study was performed (January 2003 to August 2019) to identify myopathy patients in whom dysphagia was the initial symptom or was disproportionately severe compared with limb weakness. Results: Thirty-two patients were identified. The median age at diagnosis was 65 y (range, 36–80 y). Inclusion body myositis (IBM) (n = 15), immune-mediated necrotizing myopathy (IMNM) (n = 5), and oculopharyngeal muscular dystrophy (n = 4), were the most common diagnoses. In 4 patients (3 IMNM and 1 nonspecific myositis) dysphagia evolved rapidly. At evaluation, 21 patients required diet alterations, 5 required feeding tubes, and 8 had aspiration pneumonia. Follow-up data were available for 20 patients (median, 24 mo). Eight patients received immunosuppressive therapies with improvement in 7, including 3 of 4 with rapidly progressive dysphagia. Conclusions: IBM and IMNM accounted for approximately two-thirds of patients with early or prominent dysphagia at our institution. Rapidly progressive dysphagia may predict immunotherapy responsiveness.

Original languageEnglish (US)
Pages (from-to)344-350
Number of pages7
JournalMuscle and Nerve
Volume62
Issue number3
DOIs
StatePublished - Sep 1 2020

Keywords

  • dysphagia
  • inclusion body myositis
  • muscular dystrophy
  • myopathy
  • myositis
  • videofluoroscopy

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)

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  • Cite this

    Triplett, J. D., Pinto, M. V., Hosfield, E. A., Milone, M., & Liewluck, T. (2020). Myopathies featuring early or prominent dysphagia. Muscle and Nerve, 62(3), 344-350. https://doi.org/10.1002/mus.26996