PT - JOURNAL ARTICLE AU - Tiina Andersen AU - Astrid Sandnes AU - Anne Kristine Brekka AU - Magnus Hilland AU - Hege Clemm AU - Ove Fondenes AU - Ole-Bjørn Tysnes AU - John-Helge Heimdal AU - Thomas Halvorsen AU - Maria Vollsæter AU - Ola Drange Røksund TI - Laryngeal response patterns influence the efficacy of mechanical assisted cough in amyotrophic lateral sclerosis AID - 10.1136/thoraxjnl-2015-207555 DP - 2017 Mar 01 TA - Thorax PG - 221--229 VI - 72 IP - 3 4099 - http://thorax.bmj.com/content/72/3/221.short 4100 - http://thorax.bmj.com/content/72/3/221.full SO - Thorax2017 Mar 01; 72 AB - Background Most patients with amyotrophic lateral sclerosis (ALS) are treated with mechanical insufflation–exsufflation (MI-E) in order to improve cough. This method often fails in ALS with bulbar involvement, allegedly due to upper-airway malfunction. We have studied this phenomenon in detail with laryngoscopy to unravel information that could lead to better treatment.Methods We conducted a cross-sectional study of 20 patients with ALS and 20 healthy age-matched and sex-matched volunteers. We used video-recorded flexible transnasal fibre-optic laryngoscopy during MI-E undertaken according to a standardised protocol, applying pressures of ±20 to ±50 cm H2O. Laryngeal movements were assessed from video files. ALS type and characteristics of upper and lower motor neuron symptoms were determined.Results At the supraglottic level, all patients with ALS and bulbar symptoms (n=14) adducted their laryngeal structures during insufflation. At the glottic level, initial abduction followed by subsequent adduction was observed in all patients with ALS during insufflation and exsufflation. Hypopharyngeal constriction during exsufflation was observed in all subjects, most prominently in patients with ALS and bulbar symptoms. Healthy subjects and patients with ALS and no bulbar symptoms (n=6) coordinated their cough well during MI-E.Conclusions Laryngoscopy during ongoing MI-E in patients with ALS and bulbar symptoms revealed laryngeal adduction especially during insufflation but also during exsufflation, thereby severely compromising the size of the laryngeal inlet in some patients. Individually customised settings can prevent this and thereby improve and extend the use of non-invasive MI-E.