@article{discovery10059493,
           month = {August},
          number = {3},
            year = {2018},
            note = {This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.},
           pages = {353--357},
           title = {Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints},
          volume = {5},
         journal = {Journal of Neuromuscular Diseases},
          author = {Kouwenberg, CV and Voermans, NC and Quinlivan, R and van den Engel-Hoek, L},
        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 {\ensuremath{<}} 0.05; r2 = -0.509, p {\ensuremath{<}} 0.01; r2 = -0.456, p {\ensuremath{<}} 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 {\ensuremath{<}} 0.01; r2 = 0.463, p {\ensuremath{<}} 0.01; r2 = 0.461, p {\ensuremath{<}} 0.01; r2 = 0.432, p {\ensuremath{<}} 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},
        keywords = {McArdle disease, glycogen storage disease type V, mastication, oral motor function},
            issn = {2214-3599},
             url = {https://doi.org/10.3233/JND-180320}
}