Background Huntington’s Disease (HD) is an autosomal dominant progressive neurodegenerative disease characterised by cognitive, motor and behavioural impairments. Corticobulbar symptoms (including speech and swallowing changes) have been reported in all stages of the disease, with aspiration pneumonia reported as the most common cause of death. There has been a recent shift to examine corticobulbar rehabilitation in other neurodegenerative conditions.
Aims This systematic review will determine if evidence exists to justify rehabilitation for corticobulbar symptoms in HD.
Methods Two investigators independently searched relevant electronic databases for literature related to corticobulbar rehabilitation in HD, published in English until April 2017. Included studies were critically appraised using the OCEBM Levels of Evidence, Cochrane Risk of Bias Tool and Scottish Intercollegiate Guidelines Network checklists. Primary outcomes included reported changes in function or neuromuscular physiology evidenced by validated measures.
Results Sixty-eight publications were screened. Three studies were excluded as they described compensatory management only. Eight studies matched the inclusion criteria. Two randomised control trials and six intervention studies evaluated rehabilitative approaches aiming to improve corticobulbar symptoms; however; there was limited use of validated or objective outcome measures.
Conclusions The few studies which focused on the effectiveness of rehabilitation programs in HD indicated no adverse effects and positive clinical outcomes were reported. As corticobulbar symptoms and associated pneumonia are among the most debilitating in terms of quality of life and caregiver burden, this review highlights the need for further research into the feasibility and potential of rehabilitation approaches for speech and swallowing changes in HD.
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