title: Oral muscles are progressively affected in Duchenne muscular dystrophy: implications for dysphagia treatment.
abstract: Dysphagia is reported in advanced stages of Duchenne muscular dystrophy (DMD). The population of DMD is changing due to an increasing survival. We aimed to describe the dysphagia in consecutive stages and to assess the underlying mechanisms of dysphagia in DMD, in order to develop mechanism based recommendations for safe swallowing. In this cross-sectional study, participants were divided into: early and late ambulatory stage (AS, n = 6), early non-ambulatory stage (ENAS, n = 7), and late non-ambulatory stage (LNAS, n = 11). Quantitative oral muscle ultrasound was performed to quantify echo intensity. Swallowing was assessed with a video fluoroscopic swallow study, surface electromyography (sEMG) of the submental muscle group and tongue pressure. Differences in outcome parameters among the three DMD stages were tested with analysis of variance. Oral muscles related to swallowing were progressively affected, starting in the AS with the geniohyoid muscle. Tongue (pseudo) hypertrophy was found in 70 % of patients in the ENAS and LNAS. Oral phase problems and post-swallow residue were observed, mostly in the LNAS with solid food. sEMG and tongue pressure data of swallowing solid food revealed the lowest sEMG amplitude, the longest duration and lowest tongue pressure in the LNAS. In case of swallowing problems in DMD, based on the disturbed mechanisms of swallowing, it is suggested to (1) adjust meals in terms of less solid food, and (2) drink water after meals to clear the oropharyngeal area.
authors.affiliation: Department of Rehabilitation, Nijmegen Centre for Evidence Based Practice, Radboud University Medical Centre, Nijmegen, Geert Grooteplein 10, internal post 818, 6525 GA Nijmegen, The Netherlands. L.vandenEngel@reval.umcn.nl
authors.forename: Lenie
authors.initials: L
authors.lastname: van den Engel-Hoek
authors.forename: Corrie E
authors.initials: CE
authors.lastname: Erasmus
authors.forename: Jan C M
authors.initials: JC
authors.lastname: Hendriks
authors.forename: Alexander C H
authors.initials: AC
authors.lastname: Geurts
authors.forename: Willemijn M
authors.initials: WM
authors.lastname: Klein
authors.forename: Sigrid
authors.initials: S
authors.lastname: Pillen
authors.forename: Lilian T
authors.initials: LT
authors.lastname: Sie
authors.forename: Bert J M
authors.initials: BJ
authors.lastname: de Swart
authors.forename: Imelda J M
authors.initials: IJ
authors.lastname: de Groot
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D003679:Deglutition / Q000502:physiology; D003680:Deglutition Disorders / Q000209:etiology* / Q000473:pathology*; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006801:Humans; D008297:Male; D008410:Masticatory Muscles / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D014463:Ultrasonography; D014741:Video Recording; D055815:Young Adult