Swallowing, Chewing and Speaking: Frequently Impaired in Oculopharyngeal Muscular Dystrophy
- PMID: 32804098
- PMCID: PMC7592669
- DOI: 10.3233/JAD-200511
Swallowing, Chewing and Speaking: Frequently Impaired in Oculopharyngeal Muscular Dystrophy
Abstract
Background: Oculopharyngeal muscular dystrophy (OPMD) is a late onset progressive neuromuscular disorder. Although dysphagia is a pivotal sign in OPMD it is still not completely understood.
Objective: The aim of this study was to systematically investigate oropharyngeal functioning in a large OPMD population.
Methods: Forty-eight genetically confirmed OPMD patients completed questionnaires, performed clinical tests on swallowing, chewing, speaking, tongue strength and bite force, and underwent videofluoroscopy of swallowing. Descriptive statistics was used for all outcomes and logistic regression to investigate predictors of abnormal swallowing.
Results: Eighty-two percent reported difficulties with swallowing, 27% with chewing and 67% with speaking. Patients performed significantly worse on all oropharyngeal tests compared to age-matched controls except for bite force. Also asymptomatic carriers performed worse than controls: on chewing time, swallowing speed and articulation rate. During videofluoroscopy, all patients (except one asymptomatic) had abnormal residue and 19% aspirated. Independent predictors of abnormal residue were reduced swallowing capacity for thin liquids (OR 10 mL = 0.93; 20 mL = 0.95) and reduced tongue strength for thick liquids (OR 10 mL = 0.95); 20 mL = 0.90). Aspiration of thin liquids was predicted by disease duration (OR = 1.11) and post-swallow residue with 20 mL (OR = 4.03).
Conclusion: Next to pharyngeal dysphagia, chewing and speaking are also frequently affected in OPMD patients, even in asymptomatic carriers. Residue after swallowing is a very early sign, while aspiration is a later sign in OPMD. For clinical follow-up monitoring of subjective complaints, swallowing capacity and tongue strength seems relevant.
Keywords: Oculopharyngeal muscular dystrophy; cohort studies; dysarthria; dysphagia; neuromuscular diseases.
Conflict of interest statement
The authors have no conflict of interest to report.
Figures


Similar articles
-
A Preliminary Videofluoroscopic Investigation of Swallowing Physiology and Function in Individuals with Oculopharyngeal Muscular Dystrophy (OPMD).Dysphagia. 2018 Dec;33(6):789-802. doi: 10.1007/s00455-018-9904-9. Epub 2018 May 3. Dysphagia. 2018. PMID: 29725764
-
Oropharyngeal dysphagia profiles in individuals with oculopharyngeal muscular dystrophy.Neurogastroenterol Motil. 2018 Apr;30(4):e13251. doi: 10.1111/nmo.13251. Epub 2017 Nov 16. Neurogastroenterol Motil. 2018. PMID: 29144056 Free PMC article.
-
Intranuclear Aggregates Precede Clinical Onset in Oculopharyngeal Muscular Dystrophy.J Neuromuscul Dis. 2016 Mar 3;3(1):101-109. doi: 10.3233/JND-150118. J Neuromuscul Dis. 2016. PMID: 27854203
-
Textural Changes by Mastication and Proper Food Texture for Patients with Oropharyngeal Dysphagia.Nutrients. 2020 May 30;12(6):1613. doi: 10.3390/nu12061613. Nutrients. 2020. PMID: 32486264 Free PMC article. Review.
-
Emerging and established biomarkers of oculopharyngeal muscular dystrophy.Neuromuscul Disord. 2023 Nov;33(11):824-834. doi: 10.1016/j.nmd.2023.09.010. Epub 2023 Oct 6. Neuromuscul Disord. 2023. PMID: 37926637 Review.
Cited by
-
Dynamic Reconstruction Using Bilateral Lengthening Temporalis Myoplasty for Facial Palsies in Patients with Hereditary Skin Laxity.Plast Reconstr Surg Glob Open. 2024 Feb 19;12(2):e5618. doi: 10.1097/GOX.0000000000005618. eCollection 2024 Feb. Plast Reconstr Surg Glob Open. 2024. PMID: 38375370 Free PMC article.
-
Living with Dysphagia: A Survey Exploring the Experiences of Adults Living with Neuromuscular Disease and their Caregivers in the United Kingdom.J Neuromuscul Dis. 2024;11(2):389-410. doi: 10.3233/JND-230002. J Neuromuscul Dis. 2024. PMID: 38250781 Free PMC article.
-
Practical recommendations for swallowing and speaking during NIV in people with neuromuscular disorders.Acta Myol. 2024 Jun;43(2):62-70. doi: 10.36185/2532-1900-417. Acta Myol. 2024. PMID: 39082323 Free PMC article. Review.
-
Indications for Tube Feeding in Adults with Muscular Disorders: A Scoping Review.J Neuromuscul Dis. 2023;10(5):777-785. doi: 10.3233/JND-230014. J Neuromuscul Dis. 2023. PMID: 37483025 Free PMC article.
References
-
- Brais B. Oculopharyngeal muscular dystrophy: A late-onset polyalanine disease. Cytogenetic and genome research. 2003;100(1-4):252–60. - PubMed
-
- Brais B, Bouchard JP, Xie YG, Rochefort DL, Chretien N, Tome FM, et al. Short GCG expansions in the PABP2 gene cause oculopharyngeal muscular dystrophy. Nat Genet. 1998;18(2):164–7. - PubMed
-
- van der Sluijs BM, Raz V, Lammens M, van den Heuvel LP, Voermans NC, van Engelen BG. Intranuclear Aggregates Precede Clinical Onset in Oculopharyngeal Muscular Dystrophy. J Neuromuscul Dis. 2016;3(1):101–9. - PubMed
-
- Waito AA, Steele CM, Peladeau-Pigeon M, Genge A, Argov Z. A Preliminary Videofluoroscopic Investigation of Swallowing Physiology and Function in Individuals with Oculopharyngeal Muscular Dystrophy (OPMD). Dysphagia, 2018. - PubMed
MeSH terms
LinkOut - more resources
Full Text Sources
Medical