Measuring vital capacity in amyotrophic lateral sclerosis: Effects of interfaces and reproducibility

Deterioration of vital capacity (VC) in amyotrophic lateral sclerosis (ALS) signifies disease progression and indicates need for non-invasive ventilation. Weak facial muscles consequent to ALS, with resulting poor mouth seal, may interfere with the accuracy of VC measurements. To determine whether different interfaces affect VC measurements in ALS patients and whether the interface yielding the largest VC produces an even higher VC when re-measured after one week (learning effect). To explore the relationship between optimal interface VC and sniff nasal pressure (SNIP), a measurement of global inspiratory muscle strength. Thirty-five patients (17 bulbar and 18 spinal ALS) were studied. Three interfaces (rigid-cylindrical, flanged, oronasal mask) were tested. One week after the first visit, VC was recorded using the optimal interface. SNIP recordings were also obtained. In the bulbar ALS group, median (interquartile range) VC with the flanged mouthpiece was 8.4% (3.9–15.5) larger than with the cylindrical mouthpiece (p