eprintid: 10059493 rev_number: 23 eprint_status: archive userid: 608 dir: disk0/10/05/94/93 datestamp: 2018-11-06 19:54:47 lastmod: 2021-10-23 01:43:08 status_changed: 2018-11-06 19:54:47 type: article metadata_visibility: show creators_name: Kouwenberg, CV creators_name: Voermans, NC creators_name: Quinlivan, R creators_name: van den Engel-Hoek, L title: Mastication and Oral Motor Function in McArdle Disease: Patient Reported Complaints ispublished: pub divisions: UCL divisions: B02 divisions: C07 divisions: D07 divisions: F85 keywords: McArdle disease, glycogen storage disease type V, mastication, oral motor function note: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. 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 date: 2018-08-02 date_type: published official_url: https://doi.org/10.3233/JND-180320 oa_status: green full_text_type: other language: eng primo: open primo_central: open_green verified: verified_manual elements_id: 1576611 doi: 10.3233/JND-180320 pii: JND180320 lyricists_name: Quinlivan, Rosaline lyricists_id: RCMQU67 actors_name: Jayawardana, Anusha actors_id: AJAYA51 actors_role: owner full_text_status: public publication: Journal of Neuromuscular Diseases volume: 5 number: 3 pagerange: 353-357 event_location: Netherlands issn: 2214-3599 citation: 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 <https://doi.org/10.3233/JND-180320>. Green open access document_url: https://discovery.ucl.ac.uk/id/eprint/10059493/1/Quinlivan_Mastication%20and%20oral%20motor%20function%20in%20Mc%20Ardle%20disease%2027-03-2018rq.pdf