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Impact of Bulbar Dysfunction on the Measurement of Vital Capacity in Amyotrophic Lateral Sclerosis

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A1767 - Impact of Bulbar Dysfunction on the Measurement of Vital Capacity in Amyotrophic Lateral Sclerosis
Author Block: S. Waheed1, S. Gogineni1, G. M. Pellegrino2, G. Sferrazza Papa2, D. Aleksonis1, D. Kvarnberg1, F. Di Marco2, M. J. Tobin1, F. Laghi1; 1Edward Hines Jr. VAH, Loyola University Medical Center, Hines, IL, United States, 2Università degli Studi di Milano, Milan, Italy.
Background: Deterioration of vital capacity (VC) in patients with amyotrophic lateral sclerosis (ALS) is a marker of disease progression and a criterion to start non-invasive ventilation. Yet, lack of mouth seal as a result of facial muscle weakness, particularly in patients with bulbar dysfunction, may bias VC measurements. Aim: To test whether different oral interfaces affect measurements of VC in ALS patients with and without bulbar dysfunction. Methods: Fourteen ALS patients, seven of whom with bulbar dysfunction, were studied. In random order, three different interfaces currently used in pulmonary function tests labs (rigid cylindrical mouthpiece, flanged mouthpiece, and oronasal mask) were tested. Patients performed 3 to 8 VC maneuvers with each interface. Results: In patients with bulbar dysfunction, VC was greater with the flanged mouthpiece (2.53 ± 0.30 L) than with oronasal mask (2.18 ± 0.35 L) or with the rigid cylindrical mouthpiece (2.10 ± 0.31 L) (p=0.03, ANOVA). In patients without bulbar dysfunction, VC was greater with the flanged mouthpiece (2.40 ± 0.37 L) than with oronasal mask (2.18 ± 0.36 L; p=0.04) and equivalent to VC with the rigid cylindrical mouthpiece (2.36 ± 0.36 L) (p=0.47). Conclusion: In patients with ALS, regardless of presence or absence of bulbar dysfunction, use of flanged mouthpiece may provide the ‘best fit’ for monitoring of VC. This finding has the potential to impact clinical decisions in patients with ALS.
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