title: Dysphagia in X-linked bulbospinal muscular atrophy (Kennedy disease).
abstract: Dysphagia in X-linked bulbospinal muscular atrophy (Kennedy disease) has never been characterized in detail by objective swallowing studies. We assessed the nature of swallowing impairment in Kennedy disease by undertaking fiberoptic endoscopic evaluation of swallowing examinations of 10 genetically confirmed patients with Kennedy disease who were scored according to an ordinal rating scale including 25 different items. The results were compared to an age-matched control group of 10 healthy volunteers. Swallowing dysfunction was found in 80% of patients with Kennedy disease. The main pattern of dysphagia was an incomplete food bolus clearance through the pharynx with residues left in the valleculae overflowing into the laryngeal vestibule after the swallow. Total duration of the pharyngeal swallow was significantly shorter in patients with Kennedy disease compared to the control group. These findings suggest that dysphagia in Kennedy disease is predominantly characterized by an impairment of the pharyngeal phase of swallowing resulting from reduced base-of-tongue movement and bilateral paresis of pharyngeal and laryngeal muscles.
authors.affiliation: Department of Neurology, University Hospital of Münster, Albert-Schweitzer-Str. 33, Münster D- 48129, Germany. Tobias.Warnecke@ukmuenster.de
authors.forename: Tobias
authors.initials: T
authors.lastname: Warnecke
authors.forename: Stephan
authors.initials: S
authors.lastname: Oelenberg
authors.forename: Inga
authors.initials: I
authors.lastname: Teismann
authors.forename: Sonja
authors.initials: S
authors.lastname: Suntrup
authors.forename: Christina
authors.initials: C
authors.lastname: Hamacher
authors.forename: Peter
authors.initials: P
authors.lastname: Young
authors.forename: E Bernd
authors.initials: EB
authors.lastname: Ringelstein
authors.forename: Rainer
authors.initials: R
authors.lastname: Dziewas
mesh_terms: D000328:Adult; D000368:Aged; D001696:Biomechanical Phenomena; D055534:Bulbo-Spinal Atrophy, X-Linked / Q000453:epidemiology* / Q000503:physiopathology; D003679:Deglutition / Q000502:physiology; D003680:Deglutition Disorders / Q000453:epidemiology* / Q000503:physiopathology; D006801:Humans; D007830:Larynx / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D010609:Pharyngeal Muscles / Q000502:physiology; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D013997:Time Factors; D014059:Tongue / Q000503:physiopathology