Abstract

ObjectiveDysphagia is common in Amyotrophic lateral sclerosis (ALS). ALS shows significant phenotypic variability. It is characterized by progressive weakness and/or spasticity of muscles. Dysphagia symptoms vary. Aspiration is often silent and cognitive dysfunction is common. The purpose of the study was to evaluate tongue strength measurements, dysphagia questionnaire, the presence of pharyngeal secretions, and FEES findings in dysphagia management in ALS. MethodsTwenty-five patients completed the Eating Assessment Tool-10 (EAT-10), had their Maximum Isometric Tongue Pressure (MITP), and endurance measured and underwent Flexible Endoscopic Evaluation of Swallowing (FEES) providing 31 examinations. ResultsOut of 25 patients, 76% were self-reported as dysphagic (EAT-10≥3) with a mean EAT-10 at 14.95 (±7.96). ALS patients had significantly decreased tongue strength (mean MITPanterior: 31.69 ± 17.32kPa). Comparing examinations of dysphagic and non-dysphagic status the mean MITPa of non-dysphagic was significantly greater (52.33 ± 10.97 kPa versus 20.6 ± 12.67 kPa), p<0.001. FEES detected aspiration in 10 out of 31 examinations [Penetration Aspiration Scale(PAS) ≥6]. Aspirator status examinations showed statistically significantly worse cough (p = 0.001), tongue strength (p = 0.001) and endurance (p = 0.003), pharyngeal secretions (p<0.001), velopharyngeal sufficiency (p = 0.006), pharyngeal squeeze (p = 0.009), vocal cords’ movements (p = 0.001), pharyngeal pooling (p<0.001), EAT-10 (p = 0.001) and bulbar subscale of ALS Functioning Rating Scale-Revised (b-ALSFRS-R) scores (p = 0.014) compared to non-aspirator status. Correlation analysis indicated that the feeding status had strong statistically significant correlations with the EAT-10 score (rho = −0.816), anterior tongue strength (rho = 0.735), secretions (rho = −0.811), pharyngeal pooling (rho = −0.712) and PAS (rho = −0.676) at FEES, and b-ALSFRS-R score (rho = 0.791), all p<0.001. The EAT-10 had strong statistically significant correlations with the MITPa (r = −0.794, p<0.001), secretions (rho = 0.668, p<0.001), and b-ALSFRS-R score (rho = -0.766, p = 0.001). The FEES findings had strong statistically significant correlations with the anterior tongue strength (pooling: rho = −0.784), and secretions (PAS: rho = 0.723; pooling: rho = 0.671), all p<0.001. For the questionnaire, tongue strength and pharyngeal secretions, ROC analysis assessed cut-off points and discriminating ability to predict aspiration status (Area Under the Curve: 0.838; 0.845 and 0.93, respectively). EAT-10 with a cut-off at 8 was able to predict aspirator status with a sensitivity of 100% and a specificity of 42.9% (negative predictive value-NPV = 100%). A cut-off value of 22KPa for the MITPa discriminated aspirator status (sensitivity = 80%, specificity = 89.5%, NPV = 89.5%). The quantity of secretions observed upon endoscopy with a cut-off value at 1 was able to predict aspirator status (sensitivity = 90%, specificity = 80%, NPV = 94.1%). ConclusionReduced tongue strength, questionnaire-reported symptoms, pharyngeal secretions, and FESS findings can guide identification of patients with ALS at risk of inefficient and unsafe swallowing.

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