Laryngeal function in amyotrophic lateral sclerosis during breathing and mechanical insufflation - exsufflation.

Project summary

Amyotrophic lateral sclerosis (ALS) is a disease of the brain and spinal cord that affects respiration and ability to cough because of muscle weakness of chest, tongue and larynx. Effective airway clearance is central and many patients are helped using mechanical cough device for home treatment avoiding hospital admissions. Experience has shown that disease progression lapses this effect, ending in choking sensation, tracheostomy and death. This study aimed, by video-laryngoscopy to visualize changes in laryngeal response during use of mechanical cough device to enable a more individualized home treatment, delaying discomfort and need for tracheostomy and hospitalizations. The study is threefold, involving use of the cough device; a) method-study to map the laryngeal movements by various negative and positive pressures in healthy control subjects. b)  cross-sectional study of 20 ALS patients and c) longitudinal study where changes in laryngeal response in 20 ALS patients are screened throughout  the disease course