I05 Dysphagic Symptom'S in Huntington Disease Stages

Abstract

Purpose Instrumental assessments to visualize the structure and functions of the different stages of swallowing and to identify the risk of aspiration include Videofluoroscopic evaluation (VFSS) and Fiberoptic Endoscopic Evaluation of Swallowing (FEES). However, there are only a few studies on dysphagia in Huntington‘s Disease (HD). Recently, a guideline paper was published by the EHDN Standards of Care Speech and Language Therapy Working Group (Hamilton, Heemskerk et al. 2012), but there is no report on the evaluation of dysphagia by FEES so far. Therefore, the aim of the study was to determine the dysphagic symptoms spilling, residuals, penetration and aspiration of puree, liquids and solid food by FEES according to HD-stage. Furthermore, this study is a pilot project to establish predictors for dysphagia in clinical assessment in HD. Method This study investigated 29 HD patients with the Clinical Swallowing Assessment NOD-Stepwise Concept by GUT-Team 2009 (NOD) (Ickenstein, Hofmayer et al. 2009) followed by Fiberendoscopic Evaluation of Swallowing (FEES) (Langmore 2006) and compared morphological with functional data to determine the dysphagic symptoms spilling, residuals, penetration and aspiration in the different HD-Stages as well as predictors in clinical swallowing assessment for dysphagia (Daniels, McAdam et al. 1997) in HD. The consistencies used in FEES have been puree, water, thickened liquid, bread, apple and pill. Results There were no statistical significance in spilling between HD-stages. Residuals of puree, penetration and aspiration as well as PEG-Recommendation showed significant differences between HD-stages. Conclusions FEES allows clinicians not only to characterise dysphagia precisely but also to find out an appropriate compensatory method and verifies its effectiveness by correlating morphological with functional data in HD-Patients. There is a higher risk for penetration and aspiration in later stages of HD inducing recommendation for Percutaneous Endoscopic Gastrostomy (PEG). Dysarthria, dysphonia and abnormal gag reflex show a high sensitivity to predict dysphagia. Dysarthria, dysphonia, gag refelx and voluntary cough distinguished Dysphagia in sence of a worst PAS-Score of all tested consistencies in FEES higher than 2 (p < 0.05). The 90 ml water test is not a sufficient diagnostic instrument to exclude dysphagia in HD. Due to lack of specificity of dysphagia, it is useful to initiate an imaging diagnosis of dysphagia. This study is a pilot project and further studies with larger groups of HD patients must be performed.

Management of dysphagia in Huntington’s disease: a descriptive review

“…In the same study by Dello Monaco and colleagues [13], the prevalence of dysphagia was found to be stable between the early and the middle stage of the disease, while it increased only in the advanced stage. Conversely, other studies reported a progression in the severity of the signs of dysphagia with significant differences also between the early and the moderate stage [12,14].…”

“…old age [9,15] high Unified Huntington's Disease Rating Scale (UHDRS) motor score [9,15] poor cognitive status [9,16] dysphonia [6,14] dysarthria [6,[14][15]17] tongue movements alterations [15,17].…”

“…Their sensitivity depends on the disease of the population being tested because of the different rate of silent aspiration [27]. The only study addressing this issue in patients with HD shows that the 90ml Water Swallow Test does not have a sufficient diagnostic accuracy to exclude dysphagia in HD when compared to FEES [14]. Concerning clinical assessment tools, only de Tommaso and colleagues [15] applied a standardized bedside swallowing assessment checklist [28] in patients with HD.…”