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4. Laryngoscope, 128:1335-1339, 2018.

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Background: Corticobulbar symptoms have been reported in all stages of Huntington's disease (HD); aspiration pneumonia associated with swallowing impairment has been identified as the most common cause of death. Whilst recent research has described positive effects of corticobulbar rehabilitation in other neurodegenerative conditions, it is unclear if this is similarly effective in HD. Preliminary evidence in corticospinal rehabilitation has revealed physical therapy and exercise could be beneficial for individuals with HD. Objective: This systematic review will explore the literature relative to rehabilitation of corticobulbar symptoms in adults with HD. Methods: Two investigators independently searched relevant electronic databases for literature related to corticobulbar rehabilitation in HD, published in English until October 2019. Included studies were critically appraised using the Oxford Centre for Evidence-based Medicine Levels of Evidence, Cochrane Risk of Bias Tool and Scottish Intercollegiate Guidelines Network checklists. Study outcomes included measurements of function, quality of life or neuromuscular physiology. Results: Seventy-seven publications were screened with eight studies meeting the inclusion criteria-two randomised control trials and six intervention studies. Validated and objective outcome measures of corticobulbar symptoms were infrequently used. There was a high risk of bias identified in 7/8 studies. The data suggested positive clinical outcomes, no adverse effects and no deterioration observed across longitudinal studies. Conclusions: This systematic review documented a lack of high-quality evidence to support the use of rehabilitation to treat corticobulbar symptoms in HD. However, the suggestion of potential positive effects based on available, albeit limited, studies provides justification for further research in this area.

Purpose Ultrasound imaging offers a noninvasive adjunct to clinical swallowing assessment. Published reliability of sophisticated ultrasound systems is promising; however, no data exist for reliability using more affordable, pocket-sized devices. This study explored intrarater, interrater, and test–retest reliability of swallowing measures acquired with pocket-sized ultrasound technology. Method Five participants collected measures of swallowing from 20 healthy individuals using the Clarius ultrasound. Hyoid excursion and thyrohyoid approximation were derived during saliva, liquid, and puree swallowing. The cross-sectional area of the floor of mouth muscles and tongue thickness were obtained at rest. Measures were collected at two occasions minimum 11 days apart. Reliability was assessed for the entire process of data acquisition including scanning and online measurement, and for offline measurement of saved images. Results For most measures, reliability was poor (ICC [intraclass correlation coefficient] < .50) to moderate (ICC = .50–.75) for the entire process of data acquisition and poor to good (ICC > .75) when measuring saved images. Conclusion Further work is needed to elucidate whether our study findings apply to the Clarius system only or the data suggest a general limitation of pocket-sized ultrasound technology.

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