H31 Dysphagia in huntington´s disease – an observational study

Background In Huntington´s Disease (HD) swallowingfunctions become progressively impaired, increasing the risk of aspiration and broncho-pulmonary infections. Currently the widely held belief that swallowtraining (SLT) is beneficial in HD is not supported by adequate evidence. As a first step towards establishing at what disease stage SLT interventions are most beneficial we describe onset and characteristics of the swallowingdisorder in HD. Purpose and methods We studied 73 consecutive HD patients attending the HD center of UUlm using the Clinical SwallowingExamination (CSE) & Fiberendoscopic Evaluation of Swallowing(FEES) in different stages of HD. Dysphagiawas operationally defined as a score >2 on the Penetration-Aspiration-Scale (PAS). It was explored whether an increased risk of aspiration can be predicted by applying predictors established by Daniels for stroke patients and whether compensatory and adaptive methods are helpful interventions in HD. Results As expected, patients in later stages of HD displayed a more severe dysphagiaand precautions for oral intake were recommended; insertion of a Percutaneous Feeding Tube(PFT) was recommended more frequently in more advanced stages of HD (Chi-Quadrat Pearson p=0.001). In stages 3 & 4, 21 subjects exhibited dysarthria(>95%) and voice-changeafter swallow(>86%) as sensitive, but not very specific ( 2 (sensitivity>86%). To prevent aspiration, chintuck swallowingas well as individual diet adaptation were effective in all stages of HD. There is a tendency for lower BMI in later stages. However, there was no significant correlation between the extent of dysphagiaand BMI. Conclusions Most patients with dysphagiain FEES exhibited dysarthriaand tongue movement disorder. However, to exclude dysphagiawith confidence, FEES should be employed; so far no reliable clinical predictor for penetration and aspiration in HD has been established. Chintuck in combination with an appropriate diet adjustment allow HD patients a safe oral feeding.