How to increase noninvasive ventilation effectiveness in bulbar amyotrophic lateral sclerosis patients

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease in which the respiratory muscles are also affected, leading to respiratory failure. Bulbar impairment develops in almost all cases during the course of the disease, becoming severe in the late stages of disease. Noninvasive ventilation (NIV) has been shown to increase survival in ALS; however, severe bulbar dysfunction has a negative impact on NIV tolerance and effectiveness. Therefore, certain steps should be taken to improve NIV outcomes in these patients including optimal ventilatory parameters, adequate interface selection, effective respiratory secretion management and control of bulbar symptoms.

FIGURE 1

Polysomnography recording of an obstructive event in the upper airway with decreased central drive in a patient with amyotrophic lateral sclerosis under noninvasive ventilation in volume assisted/control mode (increase in peak inspiratory pressure, abolition of thoraco-abdominal movements, oxygen desaturation).