Nutritional Risk in Oculopharyngeal Muscular Dystrophy: Beyond Dysphagia

Publication: Canadian Journal of Dietetic Practice and Research
20 April 2021

Abstract

Purpose: To document the nutritional risk in adults with oculopharyngeal muscular dystrophy (OPMD) and its association with oropharyngeal dysphagia.
Methods: In this cross-sectional study, 33 adults with molecular confirmation of OPMD between 50 and 75 years old were recruited from the registry of a university-affiliated neuromuscular clinic. Nutritional risk was assessed with the French version of Seniors in the Community: Risk Evaluation for Eating and Nutrition II (SCREEN II), whereas the severity of dysphagia was assessed using the French-Canadian version of the Sydney Swallow Questionnaire. Anthropometric measurements were performed with standardized procedures.
Results: SCREEN II scores showed high nutritional risk for 81.8% of OPMD participants with 6 factors contributing to nutritional risk in at least 50% of the sample. Pearson’s correlational analysis showed a significant moderate relationship between dysphagia and nutritional risk (r = −0.470; P = 0.006).
Conclusion: To our knowledge, this study is the first to investigate the nutritional risk of adults with OPMD. Our results indicate that individuals with OPMD may be at high nutritional risk mostly associated with swallowing difficulty, in the absence of a low body mass index. The present study highlights the need for dietary counseling in OPMD.

Résumé

Objectif. Documenter le risque nutritionnel chez les adultes atteints de dystrophie musculaire oculopharyngée (DMOP) et son association avec la dysphagie oropharyngée.
Méthodes. Dans le cadre de cette étude transversale, 33 adultes avec confirmation moléculaire de DMOP et âgés de 50 à 75 ans ont été recrutés par l’entremise du registre d’une clinique neuromusculaire affiliée à une université. Le risque nutritionnel a été évalué à partir de la version française de Seniors in the Community: Risk Evaluation for Eating and Nutrition [Aînés dans la communauté : évaluation du risque nutritionnel], version II (SCREEN II), tandis que la gravité de la dysphagie a été évaluée à l’aide de la version canadienne-française du questionnaire Sydney Swallow. Des mesures anthropométriques ont été effectuées en suivant des procédures normalisées.
Résultats. Les résultats de SCREEN II montraient un risque nutritionnel élevé chez 81,8 % des participants atteints de DMOP, et 6 facteurs contribuaient au risque nutritionnel chez au moins 50 % des adultes échantillonnés. L’analyse du coefficient de corrélation de Pearson révélait une relation modérée significative entre la dysphagie et le risque nutritionnel (r = −0,470; P = 0,006).
Conclusions. À notre connaissance, cette étude est la première à explorer le risque nutritionnel des adultes atteints de DMOP. Nos résultats indiquent que les personnes atteintes de DMOP pourraient présenter un risque nutritionnel élevé, principalement associé à des difficultés de déglutition, en l’absence d’un faible indice de masse corporelle. La présente étude souligne la nécessité de counseling diététique dans les cas de DMOP.

Get full access to this article

View all available purchase options and get full access to this article.

References

1
Tomé F, Fardeau M. Oculopharyngeal muscular dystrophy. In: Engel AG, Franzini-Armstrong C, editors. Myology. Vol. 2. New York, NY: McGraw-Hill; 1994. p. 1233–45.
2
Fanelli Kuczmarski M, Pohlig RT, Stave Shupe E, Zonderman AB, and Evans MK. Dietary protein intake and overall diet quality are associated with handgrip strength in African American and White adults. J Nutr Health Aging. 2018;22(6):700–9.
3
Keller HH, Goy R, and Kane SL. Validity and reliability of SCREEN II (Seniors in the Community: Risk Evaluation for Eating and Nutrition, Version II). Eur J Clin Nutr. 2005;59(10):1149–57.
4
Wallace KL, Middleton S, and Cook IJ. Development and validation of a self-report symptom inventory to assess the severity of oral-pharyngeal dysphagia. Gastroenterology. 2000;118(4):678–87.
5
Côté C, Fortin J, Brais B, Youssof S, Gagnon C. French Canadian cross-cultural adaptation of the SWAL-QOL and the SSQ and a preliminary psychometric assessment for their use in an oculopharyngeal muscular dystrophy (OPMD) population. Qual Life Res. 2021.
6
Wild D, Grove A, Martin M, Eremenco S, McElroy S, Verjee-Lorenz A, et al. Principles of good practice for the translation and cultural adaptation process for patient-reported outcomes (PRO) measures: report of the ISPOR Task Force for Translation and Cultural Adaptation. Value Health. 2005;8(2):94–104.
7
Cote C, Gagnon C, Youssof S, sKurtz N, and Brais B. The requirement for a disease-specific patient-reported outcome measure of dysphagia in oculopharyngeal muscular dystrophy. Muscle Nerve. 2019;59(4):445–50.
8
Cederholm T, Jensen GL, Correia M, Gonzalez MC, Fukushima R, Higashiguchi T, et al. GLIM criteria for the diagnosis of malnutrition—a consensus report from the global clinical nutrition community. Clin Nutr. 2019;38(1):1–9.
9
Mori H and Tokuda Y. Differences and overlap between sarcopenia and physical frailty in older community-dwelling Japanese. Asia Pac J Clin Nutr. 2019;28(1):157–65.
10
Krok-Schoen JL, Archdeacon Price A, Luo M, Kelly OJ, and Taylor CA. Low dietary protein intakes and associated dietary patterns and functional limitations in an aging population: a NHANES analysis. J Nutr Health Aging. 2019;23(4):338–47.
11
Coelho-Júnior H, Milano-Teixeira L, Rodrigues B, Bacurau R, Marzetti E, and Uchida M. Relative protein intake and physical function in older adults: a systematic review and meta-analysis of observational studies. Nutrients. 2018;10(9):1330.
12
Ellegård L, Aldenbratt A, Svensson MK, and Lindberg C. Body composition in patients with primary neuromuscular disease assessed by dual energy X-ray absorptiometry (DXA) and three different bioimpedance devices. Clin Nutr ESPEN. 2019;29:142–8.
13
van der Sluijs BM, Lassche S, Knuiman GJ, Kusters B, Heerschap A, Hopman M, et al. Involvement of pelvic girdle and proximal leg muscles in early oculopharyngeal muscular dystrophy. Clin Neuromusc. Disord. 2017;27(12):1099–105.

Information & Authors

Information

Published In

cover image Canadian Journal of Dietetic Practice and Research
Canadian Journal of Dietetic Practice and Research
Volume 82Number 2June 2021
Pages: 95 - 97
Editor: Naomi Cahill Ph.D RD

History

Version of record online: 20 April 2021

Authors

Affiliations

Catherine Forgues RD, MSc
Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, QC
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC
Julie Fortin BA
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC
Cynthia Gagnon OT, PhD
Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, QC
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC
Jean-Denis Brisson MD
Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, QC
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC
Jean Mathieu MD, MSc
Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, QC
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC
Bernard Brais MD, PhD
Department of Neurology and Neurosurgery, McGill University, Montreal, QC
Claudia Côté RD, MSc
Faculty of Medicine and Health Sciences, University of Sherbrooke, Sherbrooke, QC
Groupe de recherche interdisciplinaire sur les maladies neuromusculaires, CIUSSS Saguenay-Lac-St-Jean/Neuromuscular Clinic, Saguenay, QC
Centre de recherche Charles-Le Moyne—Saguenay-Lac-Saint-Jean sur les innovations en santé, Longueuil, QC

Metrics & Citations

Metrics

Other Metrics

Citations

Cite As

Export Citations

If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. Simply select your manager software from the list below and click Download.

There are no citations for this item

View Options

Login options

Check if you access through your login credentials or your institution to get full access on this article.

Subscribe

Click on the button below to subscribe to Canadian Journal of Dietetic Practice and Research

Purchase options

Purchase this article to get full access to it.

Restore your content access

Enter your email address to restore your content access:

Note: This functionality works only for purchases done as a guest. If you already have an account, log in to access the content to which you are entitled.

View options

PDF

View PDF

Full Text

View Full Text

Media

Media

Other

Tables

Share Options

Share

Share the article link

Share on social media