Dysphagia and swallowing-related quality of life in Friedreich ataxia

Vogel, Adam P., Brown, Sophie E., Folker, Joanne E., Corben, Louise A. and Delatycki, Martin B. (2013) Dysphagia and swallowing-related quality of life in Friedreich ataxia. Journal of Neurology, 261 2: 392-399. doi:10.1007/s00415-013-7208-4

Author Vogel, Adam P.
Brown, Sophie E.
Folker, Joanne E.
Corben, Louise A.
Delatycki, Martin B.
Title Dysphagia and swallowing-related quality of life in Friedreich ataxia
Journal name Journal of Neurology   Check publisher's open access policy
ISSN 0340-5354
Publication date 2013-01-01
Year available 2013
Sub-type Article (original research)
DOI 10.1007/s00415-013-7208-4
Open Access Status Not Open Access
Volume 261
Issue 2
Start page 392
End page 399
Total pages 8
Place of publication Heidelberg, Germany
Publisher Springer Medizin
Language eng
Subject 2808 Neurology
2728 Clinical Neurology
Abstract Dysphagia in Friedreich ataxia (FRDA) and its impact on quality of life is not adequately understood. The objective of this study was to characterise dysphagia in FRDA and to determine the impact of swallowing dysfunction on activities, participation, and sense of well-being. Thirty-six individuals with a confirmed diagnosis of FRDA were assessed via a clinical bedside examination (CBE), the Royal Brisbane Hospital outcome measure for swallowing, an oral-motor examination and the Australian therapy outcome measures for speech and swallowing (AusTOMS). Data on swallowing function, diet modification and swallowing strategies were collated. Thirty-three (91.67 %) participants exhibited clinical signs of dysphagia according to the CBE, and all participants received ratings indicating swallowing difficulties on at least one other measure. Dysphagia in FRDA is characterised by oral and pharyngeal stage impairment relating to incoordination, weakness and spasticity. A significant positive correlation was found between the severity of impairment, activity, participation and distress/well-being on the AusTOMS, suggesting that swallowing function decreases with overall reductions in quality of life. A significant correlation was found between activity on the AusTOMS and disease duration (r = -0.283, p = 0.012). No significant correlations were found between dysphagia severity and GAA repeat length, age of onset or disease severity. Participants employing diet modification and swallowing strategies demonstrated higher dysphagia severity, activity limitations and participation restrictions. These data advocate a holistic approach to dysphagia management in FRDA. Early detection of swallowing impairment and consideration of the potential impact dysphagia has on quality of life should be key aspects in disease management.
Keyword Ataxia
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2014 Collection
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