Affiliations: [a] Centre for Neuroscience of Speech, The University of Melbourne, Parkville, Victoria, Australia
| [b] Calvary Health Care Bethlehem, Victoria, Australia
| [c] Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany
| [d] Center for Neurology, University Hospital Tübingen, Tübingen, Germany
| [e] Redenlab, Australia
Correspondence:
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Correspondence to: Megan Keage, PhD, Department of Audiology and Speech Pathology, The University of Melbourne, 550 Swanston Street, Carlton, Victoria, 3053, Australia. E-mail: megan.keage@unimelb.edu.au.
Abstract: Background:Dysphagia is common in Huntington’s disease (HD) affecting all phases of swallowing. Correlations exist between non-instrumental measures of dysphagia and clinical features of HD, including age, disease duration and degree of motor impairment. Lack of instrumental data limits our ability to wholly characterize HD-related dysphagia and prognosticate swallowing changes over time. Objective:To retrospectively describe a relatively large database of videofluoroscopic studies (VFSSs) and determine the relationships between dysphagia and HD clinical parameters, including disease duration and burden of pathology score. Methods:Medical and swallowing data of 49 individuals with HD and dysphagia were examined. VFSS data were interpreted using the Bethlehem Assessment Scale and Penetration-Aspiration Scale. Data from clinical bedside examination and social information were collated to describe the impact of dysphagia in HD. Repeated VFSS data were available for seven individuals. Results:Swallowing was characterized by lingual dysfunction, reduced soft palate elevation, delayed pharyngeal swallow initiation, and inability to clear matter from the pharynx. Two-thirds of cases presented with compromised airway protection with both liquid and solid consistencies. Tachyphagia and difficulty self-feeding were common. Dysphagia correlated with disease severity and duration. Longitudinal analysis revealed a mixed pattern of progression with some individuals presenting with worsening dysphagia whilst others appeared to remain stable or improved in function. Conclusions:Dysphagia in HD is exacerbated by difficulties with self-feeding and monitoring feeding rate. Burden of pathology relates to pharyngeal swallow initiation and penetration and aspiration of fluid. Dysphagia did not appear to worsen in a systematic way in a subset of participants.
