La disfagia en la enfermedad de Huntington: propuesta de intervención logopédica

Abstract

IntroductionHuntington disease (HD) is a progressive neurodegenerative autosomal dominant disease characterized by motor, behavioral and cognitive disturbances. Dysphagia is a common and life-threatening symptom of the disease leading to nutritional deficiencies and a high risk of bronchoaspiration. Our objective was to study swallowing dysfunction and to present a specific intervention to improve or maintain swallowing efficiency. Materials and methodsWe recruited 10ambulatory HD patients aged between 46 and 79years old, with a moderate stage of the disease, who were compared with 10 age- and gender-matched healthy controls. The safety and effectiveness of the oral and pharyngeal phases was assessed qualitatively and quantitatively through the volume-viscosity swallow test. ResultsWe observed ineffective lip closure in 6patients, oral residue in 4, repetitive swallows in 10, pharyngeal residue in 7, cough in 3, voice changes in 6, and a drop of 2points in blood oxygen saturation in a further 3. Controls scored between 60 and 63points, while patients scored between 19 and 53points. ConclusionsThe oral and pharyngeal swallowing phases are already compromised in moderate stages of HD. We hypothesize that a specific rehabilitation program for dysphagia could improve swallowing difficulties and contribute to maintaining nutritional status in affected individuals. Consequently, we propose a rehabilitation program including orolingual praxis and specific muscular postural strategies.