| Abstract: | Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease inwhich most patients die of respiratory failure. Although volume-targeted non-invasivebilevel positive airway pressure (BPAP) ventilation has been studied in patients withchronic respiratory failure of various etiologies, its use in ALS has not beenreported. We present the case of a 66-year-old woman with ALS and respiratory failuretreated with volume-targeted BPAP ventilation for 15 weeks. Weekly data downloadsshowed that disease progression was associated with increased respiratory muscleweakness, decreased spontaneous breathing, and increased use of non-invasive positivepressure ventilation, whereas tidal volume and minute ventilation remained relativelyconstant. |
