Dysphagia in Huntington's disease

Leiden Repository

Dysphagia in Huntington's disease

Title: Dysphagia in Huntington's disease
Author: Heemskerk-van den Berg, Willemien Antoinette
Publisher: Department of Neurology, Faculty of Medicine / Leiden University Medical Center (LUMC), Leiden University
Issue Date: 2015-04-15
Keywords: Huntington's disease
Dysphagia
Videofluoroscopy
Aspiration
Abstract: Huntington’s disease (HD) is a progressive neurodegenerative disease with an autosomal, dominant mode of inheritance. Patients with HD suffer from dysphagia which can have serious consequences, such as weight loss, dehydration, and pneumonia leading to death. Many patients with HD die of aspiration pneumonia. In order to start monitoring dysphagia in HD patients systematically during different stages of the disease, a new measurement instrument was needed. We developed the Huntington’s Disease Dysphagia Scale. The availability of this new scale now allows prospective monitoring of dysphagia in patients with HD. Another aim was to investigate specific dysphagia features in HD using videofluoroscopy. We found that 78% of our patients suffered from dysphagia. Swallowing difficulties start already in the first stage of HD, and become more severe as the disease progresses. The final aim was to initiate intervention studies on dysphagia in HD. We began by developing a guideline based on best practice. The guideline describes dysphagia problems per stage of HD, and makes recommendations for intervention and clinical advice. One of the recommendations in the European guideline is the chin tuck position while swallowing. As there is no evidence for improvement with this intervention for HD patients, we carried out a videofluoroscopic swallowing test with and without the chin tuck test procedure. No significant differences were found for spilling, aspiration or residue in swallowing with the head in normal upright position or the chin tuck posture. We therefore recommend that the chin tuck intervention should not be used in HD patients.
Description: Promotor: R.A.C. Roos, Co-promotores: J. Marinus, B.M. Verbist
With summary in Dutch
Faculty: LUMC
Citation: Heemsker-van den Berg, W.A., 2015, Doctoral Thesis, Leiden University
ISBN: 9789461089144
Sponsor: Topaz Vereniging van Huntington
Handle: http://hdl.handle.net/1887/32744
 

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