Introduction
The Swallowing Quality of Life instrument (SWAL-QOL) is a patient-reported outcome (PRO) measure of swallowing-related quality of life (SR-QoL). Its psychometric properties in oculopharyngeal muscular dystrophy (OPMD) are not known.
Methods
We administered the SWAL-QOL to U.S. OPMD Registry participants. We described SR-QoL profiles and assessed reliability and validity.
Results
Mean composite score in 113 individuals with OPMD was 54.4 ± 20.7, indicating moderate impairment. Severe impairments were observed in eating duration, burden, and fatigue scales. Internal consistency reliability of all scales was found to be satisfactory, and 9 of 10 scales demonstrated adequate test-retest reliability. Data confirmed 86% of hypotheses, supporting construct validity. The SWAL-QOL limitations in OPMD include: floor/ceiling effects in 7 of 10 scales and low specificity of sleep, fatigue, and communication scales for dysphagia.
Discussion
SR-QoL is reduced in OPMD. Given several limitations of the SWAL-QOL, development of an improved dysphagia-specific QoL instrument for OPMD is warranted.
These findings highlight the high prevalence of oropharyngeal swallowing impairments in both swallowing efficiency and safety. A high proportion of physiologic impairments in epiglottic inversion and laryngeal vestibule closure were noted that related to functional impairments in swallow safety and inefficiency.
Introduction
Despite multiple studies reporting marked benefit of botulinum toxin (BTX) for treatment of cricopharyngeal dysphagia, little is known about its safety for this indication. We examined the safety of cricopharyngeal BTX for dysphagia in oculopharyngeal muscular dystrophy (OPMD).
Methods
We reviewed records of patients with OPMD who received cricopharyngeal BTX.
Results
Twenty-four patients underwent 66 procedures. Overall adverse event frequency was 44%. The most common adverse events were dysphonia (24%) and worsened dysphagia (14%). Logistic regression demonstrated that dose was a significant predictor of worsened dysphagia (P = 0.036) and of the composite event of dysphonia or worsened dysphagia (P = 0.009). There was a nonsignificant trend for dose as a predictor of dysphonia (P = 0.073). 59% of procedures were associated with symptomatic improvement.
Conclusions
While BTX appears to be beneficial for treatment of dysphagia in OPMD, caution is warranted when injecting the cricopharyngeus muscle due to dose-related risk of dysphonia or worsened dysphagia.
including left atrial volume index (R = − 0.798, p < 0.001), and the ratio of early transmitral pulse-wave Doppler flow velocity (E) to early mitral annulus tissue Doppler velocity E' (E/E') (R = − 0.608, p = 0.036), suggesting a role of diastolic dysfunction in patients with NAFLD with exercise intolerance. DISCUSSION/ SIGNIFICANCE OF IMPACT: Cardiac abnormalities drive cardiorespiratory fitness and exercise intolerance in patients with NAFLD. These findings are exaggerated in patients with NASH suggesting a link between disease severity in NAFLD, exercise intolerance and diastolic dysfunction.
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