Tiina M Andersen1,2,3, Astrid Sandnes4,5,3, Ove Fondenes6, Roy M Nilsen7, Ole-Bjørn Tysnes8,9, John-Helge Heimdal10,9, Hege H Clemm4,3, Thomas Halvorsen4,3, Maria Vollsæter6,4,3, Ola D Røksund4,7. 1. Norwegian Centre of Excellence for Home Mechanical Ventilation, Thoracic Department, Haukeland University Hospital, Bergen, Norway. tiina.andersen@helse-bergen.no. 2. Department of Physiotherapy, Haukeland University Hospital, Bergen, Norway. 3. The Faculty of Medicine, Department of Clinical Science, University of Bergen, Bergen, Norway. 4. Department of Pediatrics, Haukeland University Hospital, Bergen, Norway. 5. Department of Medicine, Innlandet Hospital Trust, Gjøvik, Norway. 6. Norwegian Centre of Excellence for Home Mechanical Ventilation, Thoracic Department, Haukeland University Hospital, Bergen, Norway. 7. The Faculty of Health and Social Sciences, Western Norway University of Applied Sciences, Bergen, Norway. 8. Department of Neurology, Haukeland University Hospital, Bergen, Norway. 9. The Faculty of Medicine, Department of Clinical Medicine, University of Bergen, Bergen, Norway. 10. Department of Otolaryngology/Head and Neck Surgery, Haukeland University Hospital, Bergen, Norway.
Abstract
BACKGROUND: Respiratory complications represent the major cause of death in amyotrophic lateral sclerosis (ALS). Noninvasive respiratory support is the mainstay therapy, but treatment becomes challenging as the disease progresses, possibly due to a malfunctioning larynx, which is the entrance to the airways. We studied laryngeal response patterns to mechanically assisted cough (mechanical insufflation-exsufflation) as ALS progresses. METHODS: This prospective longitudinal study of 13 consecutively included subjects with ALS were followed up during 2011-2016 with repeated tests of lung function, neurological status, and laryngeal responses to mechanical insufflation-exsufflation using video-recorded flexible transnasal fiberoptic laryngoscopy. RESULTS: Follow-up time was median 17 (range 6-59) months. In total, 751 laryngoscopy recordings from 67 individual examinations (median 4 per subject, range 2-11 per subject) were analyzed. Adverse laryngeal events that developed with disease progression during insufflation included adduction of true vocal folds in 8 of 9 spinal-onset subjects and adduction of aryepiglottic folds in all subjects, initially at the highest positive pressure and prior to onset of other bulbar symptoms in spinal-onset subjects. As cough became less expulsive with disease progression, laryngeal adduction occurred at lower insufflation pressures. Retroflex movement of the epiglottis was observed in 7 of 13 subjects regardless of insufflation pressures and independent of bulbar involvements. Backward movement of the tongue base occurred regardless of insufflation pressures in all but 1 subject. During exsufflation, constriction of the hypopharynx was observed in all subjects regardless of the presence of bulbar symptoms, after the adverse events that occurred during insufflation. CONCLUSIONS: Applying high insufflation pressures during mechanically assisted cough in ALS can become counterproductive as the disease progresses as well as prior to the onset of bulbar symptoms. The application of positive inspiratory pressures should be tailored to the individual patient, and laryngoscopy during ongoing treatment appears to be a feasible tool.
BACKGROUND: Respiratory complications represent the major cause of death in amyotrophic lateral sclerosis (ALS). Noninvasive respiratory support is the mainstay therapy, but treatment becomes challenging as the disease progresses, possibly due to a malfunctioning larynx, which is the entrance to the airways. We studied laryngeal response patterns to mechanically assisted cough (mechanical insufflation-exsufflation) as ALS progresses. METHODS: This prospective longitudinal study of 13 consecutively included subjects with ALS were followed up during 2011-2016 with repeated tests of lung function, neurological status, and laryngeal responses to mechanical insufflation-exsufflation using video-recorded flexible transnasal fiberoptic laryngoscopy. RESULTS: Follow-up time was median 17 (range 6-59) months. In total, 751 laryngoscopy recordings from 67 individual examinations (median 4 per subject, range 2-11 per subject) were analyzed. Adverse laryngeal events that developed with disease progression during insufflation included adduction of true vocal folds in 8 of 9 spinal-onset subjects and adduction of aryepiglottic folds in all subjects, initially at the highest positive pressure and prior to onset of other bulbar symptoms in spinal-onset subjects. As cough became less expulsive with disease progression, laryngeal adduction occurred at lower insufflation pressures. Retroflex movement of the epiglottis was observed in 7 of 13 subjects regardless of insufflation pressures and independent of bulbar involvements. Backward movement of the tongue base occurred regardless of insufflation pressures in all but 1 subject. During exsufflation, constriction of the hypopharynx was observed in all subjects regardless of the presence of bulbar symptoms, after the adverse events that occurred during insufflation. CONCLUSIONS: Applying high insufflation pressures during mechanically assisted cough in ALS can become counterproductive as the disease progresses as well as prior to the onset of bulbar symptoms. The application of positive inspiratory pressures should be tailored to the individual patient, and laryngoscopy during ongoing treatment appears to be a feasible tool.
Authors: Anne Kristine Brekka; Maria Vollsæter; George Ntoumenopoulos; Hege Havstad Clemm; Thomas Halvorsen; Ola Drange Røksund; Tiina Maarit Andersen Journal: BMJ Open Date: 2022-05-25 Impact factor: 3.006