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.
- dysphagia; Huntington’s disease
- Bedside Swallowing Assessment Scale
Statistics from Altmetric.com
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.