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Background Swallowing problems are common among people with Huntington’s disease (HD). Evidence suggests that death usually results from respiratory complications often caused by dysphagia. However, there are no studies which examine systematically the swallowing function through the successive stages of HD. Aims The purpose of this study is therefore to investigate the swallowing function and provide appropriate management of oral feeding difficulties in relation to disease progression. Methods Thirty-eight patients (mean age= 50.62, SD ± 12.7) with genetically confirmed HD underwent swallowing evaluation. Each subject was clinically assessed with the Unified Huntington’s Disease Rating Scale (UHDRS) and was classified in one of the three stages of the disease (early, middle and late) according to the guidelines recommendations. Results In the early stage of HD, swallowing alterations were observed in 11% of the patients and required the application of diet restrictions, compensatory strategies and postures. The prevalence of swallowing impairments at the middle stage of the disease had the same trend as the first stage. Severe dysphagia was mainly represented in the late stage (26%); in fact, the two subjects receiving nutrition through non-oral feeding were in this stage of HD. Conclusions Although, dysphagia is challenging in the last stage of the neurodegenerative disease, swallowing difficulties occur since the disease onset. The results confirm the importance of ongoing assessment and management of dysphagia in the early stage, as recommended by the EHDN Standards of Care Speech and Language Therapy Working Group, and provide directions to further improve the quality of care delivered to HD patients.

Huntington’s disease (HD) is an inherited neurodegenerative disorder characterised by motor impairment, cognitive decline and psychiatric disorders. Dysphagia is a pathologic condition that increases morbidity and mortality of the affected people because of its complications: malnutrition, dehydration, airways obstruction and aspiration pneumonia. Patients with HD may develop dysphagia with the progression of the disease. Our aim was to evaluate, in a group of HD patients, the prevalence of dysphagia, its correlation with motor and cognitive disorders and patient’s functionality. We recruited 37 patients with a genetically confirmed HD, attending the Ambulatory for Huntington’s chorea of the Neurological Science Department of Bari University, and evaluated them with Bedside Swallowing Assessment Scale (BSAS) of Smithard modified by Masotti for dysphagic symptoms, and with the Unified Huntington’s Disease Rating Scale (UHDRS) for HD. We considered both total score and partial scores of UHDRS-Motor. A group of 46 controls matched for sex and age, was recruited to normalise the scores of the BSAS on sex and age. According to the BSAS, the 36,6% of our HD patients was dysphagic. There was no correlation between dysphagia and motor, cognitive or functional impairment. The group of dysphagic patients did not present with more severe choreic movements in the Oro Buccal District, compared to the not dysphagic ones. In our HD patients, dysphagia seemed not to be correlated to movement impairment, including hypekinesias in Oro Buccal District. In addition, it did not imply more severe invalidity, according to Total Functional Capacity and Functional Assessment scales, which evaluate patient’s capacity in feeding in a single item. Severe dysphagia seems a not frequent symptom in HD. However, more focused evaluation is needed to confirm its impact on clinical outcome of the disease.