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Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints

Kouwenberg, CV; Voermans, NC; Quinlivan, R; van den Engel-Hoek, L; (2018) Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints. Journal of Neuromuscular Diseases , 5 (3) pp. 353-357. 10.3233/JND-180320. Green open access

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Abstract

Background: Exertional myalgia and cramps of the limb and trunk muscles are typical in McArdle disease, but mastication and oral motor limitations have not been systematically investigated before. Objective: Determine the reported prevalence and characteristics of limitations on oral motor activities, mastication, swallowing, and other oral motor activities in patients with McArdle disease. Methods: An observational study was carried out in 28 patients using a standardised questionnaire on mastication and oral motor function. Results: 57% of the participants reported difficulties with mastication. Muscle cramps during mastication occurred in 57% of the patients. A feeling that food remains in the throat occurred in 32%. Adaptations during mastication were needed in 42% of the patients. Mastication difficulties, muscle cramps during mastication and mastication adjustments were inversely correlated with age (r2 = –0.445, p < 0.05; r2 = –0.509, p < 0.01; r2 = –0.456, p < 0.05). Feeling of food remaining in throat, cramps during mastication and during other oral motor muscle activities, were correlated with disease severity (r2 = 0.476, p < 0.01; r2 = 0.463, p < 0.01; r2 = 0.461, p < 0.01; r2 = 0.432, p < 0.05). Conclusions: In short, reported mastication difficulties are prevalent in people with McArdle disease, most frequently in younger people. Therefore, awareness of mastication and oral motor problems is required

Type: Article
Title: Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints
Location: Netherlands
Open access status: An open access version is available from UCL Discovery
DOI: 10.3233/JND-180320
Publisher version: https://doi.org/10.3233/JND-180320
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: McArdle disease, glycogen storage disease type V, mastication, oral motor function
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10059493
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