Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands
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
Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Centre, Nijmegen, The Netherlands
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:[email protected].
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