Article Text
Abstract
Background Oropharyngeal dysphagia (OD) is common in Huntington’s disease (HD) and aspiration pneumonia is the leading cause of death in this population. Nevertheless, OD pathophysiology in HD is not well understood.
Aims To investigate OD pathophysiology in patients with HD and its association with neurological data in different stages of the disease.
Methods Twenty-four patients with HD (6 in stage I, 6 in stage II, 6 in stage III and 6 in stage IV) were recruited. Neurological examination was performed with the Unified Huntington’s Disease Rating Scale (UHDRS). Patient underwent a Fiberoptic Endoscopic Evaluation of Swallowing (FEES) and data on penetration/aspiration, residue and OD pathophysiology were gained. Maximum tongue pressure (MTP) and tongue endurance were measured.
Results Regardless of HD stage, the most common pathophysiological mechanisms of OD were oropharyngeal dyspraxia (91.7%), posterior oral incontinence (87.5%), and delayed pharyngeal phase (87.5%). As the disease stage progresses, protective and propulsion deficits became more prevalent (Figure 1). Propulsion deficit was associated with disease stage and total motor function (UHDRS I) (p<0.05). Propulsion (p=0.010) and protective deficits (p<0.001) were significantly associated with penetration and aspiration. Propulsion deficit, resistive issue, posterior oral incontinence, and delayed pharyngeal phase were significantly associated with pharyngeal residues (p<0.05). MTP and tongue endurance significantly reduces in the more advanced stages of the disease (p<0.05) and were lower in patients with propulsion deficit (p<0.05).
Conclusions OD in HD results from a combination of different pathophysiological mechanisms whose prevalence changes over the course of the disease.