Affiliations: [a]
Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands
| [b]
MRC Centre for Neuromuscular Diseases and Department of Molecular Neuroscience, University College London Institute of Neurology and National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
| [c]
Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands
Correspondence:
[*]
Correspondence to: Dr. L. van den Engel-Hoek, Speech Language Pathologist, Department of Neurology, Radboud University Medical Centre, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. Tel.: +31 243615187; E-mail:Lenie.vandenEngel-Hoek@radboudumc.nl.
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.
Keywords: McArdle disease, glycogen storage disease type V, mastication, oral motor
function
