Objective: To describe the main findings of Fiberoptic endoscopic evaluation of swallowing (FEES) in patients with MJD.

Background: Machado-Joseph disease (MJD) is a degenerative process that occurs in different regions and/or functions of the central nervous system and/or peripheral, including areas responsible for motor control of speech articulation and swallowing. The applicant aspiration pneumonia due to dysphagia is one of the most common complications and causes of death of MJD.

Methods: 24 subjects with MJD, mean age 49.9(SD 10.44)years, mean disease duration 9.5(SD5.3)years. Patients were consecutively selected independently from voice complaints and swallowing. Patients underwent a questionnaire about quality of life in dysphagia (SWALQol) and FEES. During FEES, they were offered food edible cakes stained with aniline blue, the liquid consistency, thickened liquid, pasty and solid, simulating a meal. The anatomical structures and functional aspects of the swallowing events were assessed directly by fibronasopharyngolaryngoscopy. The tests were abnormal in the presence of one or more of the following dysphagic manifestations: early escape later, delayed swallowing, stasis in valleculae and/or piriform sinuses, laryngeal penetration and/or tracheal aspiration.

Results: The findings were, in decreasing order of occurrence: stasis in piriform recesses and the posterior wall of pharyngeal (20% respectively) and stasis in valleculae (13%) after ingesting the thick pasty consistency and residue on the tongue base and stasis in the posterior wall of the pharynx (7% respectively) after ingestion of thickened liquid consistency. Did not show penetration and laryngeal tracheal aspiration before or after swallowing to all consistencies. As regards the questionnaire Swal Qol the domains who presented with lower scores were fatigue 58.3(SD24.1)%, communications 63.3(SD21.6)%, afraid to eat 66.1(SD27.5)% and duration 72.5(SD33.8)%, the mean and SD of SwalQoL overall was 68.0(SD26.8)%, compared to the control group 94.5(SD4.2)% (p=0.0001).

Conclusions: There are alterations at the oral and pharyngeal stage of the swallowing in patients with MJD, by changing the oral motor control, which complicates the manipulation of high viscosity and volume in the oral cavity, increasing the occurrence of stasis. The endoscopy of swallowing shown to be an effective tool in the diagnosis of oropharyngeal dysphagia in patients with MJD.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/fiberoptic-endoscopic-evaluation-of-swallowing-findings-in-patients-with-machado-joseph-disease/