Dysphagia in Indivuduals with Huntington's Disease: A Narrative Review

Abstract

Huntington's disease (HD) is a neurodegenerative autosomal dominant condition characterized by motor, behavioral, and cognitive symptoms. Aspiration pneumonia stands out as a leading cause of death in HD, primarily attributed to dysphagia, which gets more noticeable as the disease progresses. Dysphagia symptoms in individuals with HD are compounded by noticeable movement problems, including Chorean or rigid-bradykinetic patterns. These symptoms manifest in every phase of swallowing and fluctuate with the progression of HD. Lingual chorea, delayed swallowing initiation, and impaired swallowing-respiratory coordination are key indicators of dysphagia in HD individuals. The negative impact on eating behaviors is further exacerbated by concurrent cognitive and sensory deficits. Consequently, dysphagia leads to social isolation, restrictions on activities and involvement, and a diminished quality of life for individuals with HD. To minimize these adverse effects, a referral to a speech-language therapist (SLT) for swallowing assessment should be initiated immediately upon the diagnosis of HD by a neurologist. Starting from the earliest stages of the disease, both clinical and instrumental swallowing assessments should be employed to minimize the detrimental consequences of dysphagia. Depending on the assessment results, compensatory and/or rehabilitative (restitutive) strategies can be recommended for treatment. Furthermore, the SLT actively collaborates with other team members, including individuals with HD, caregivers, neurologists, otolaryngologists, gastroenterologists, and others, contributing collectively to the decision-making process regarding both oral and non-oral feeding considerations. Despite negative impact of dysphagia on individuals with HD and its significant role in individuals’ deterioration, the evidence for specific dysphagia interventions remains limited. Clinicians, therefore, rely on well-established general swallowing therapy practices. There is a pressing need for evidence-based research on dysphagia in HD. In this study, the literature on dysphagia in HD will be examined, with a focus on its pathophysiology and the role of SLT in diagnostic and intervention techniques.