caa a22 4500 445808829 CHVBK 20180317145205.0 cr unu---uuuuu 170323e20110301xx s 000 0 eng 10.1007/s00415-010-5830-y doi (NATIONALLICENCE)springer-10.1007/s00415-010-5830-y Hardiman Orla HRB Clinician Scientist, Trinity College and Beaumont Hospital, Dublin, Ireland aut Management of respiratory symptoms in ALS [Elektronische Daten] [Orla Hardiman] Respiratory insufficiency is a frequent feature of ALS and is present in almost all cases at some stage of the illness. It is the commonest cause of death in ALS. FVC is used as important endpoint in many clinical trials, and in decision-making events for patients with ALS, although there are limitations to its predictive utility. There are multiple causes of respiratory muscle failure, all of which act to produce a progressive decline in pulmonary function. Diaphragmatic fatigue and weakness, coupled with respiratory muscle weakness, lead to reduced lung compliance and atelectasis. Increased secretions increase the risk of aspiration pneumonia, which further compromises respiratory function. Bulbar dysfunction can lead to nutritional deficiency, which in turn increases the fatigue of respiratory muscles. Early recognition of respiratory decline and symptomatic intervention, including non-invasive ventilation can significantly enhance both quality of life and life expectancy in ALS. Patients with respiratory failure should be advised to consider an advance directive to avoid emergency mechanical ventilation. Springer-Verlag, 2010 ALS nationallicence Respiratory function nationallicence Non-invasive ventilation nationallicence Journal of Neurology Springer-Verlag 258/3(2011-03-01), 359-365 0340-5354 258:3<359 2011 258 415 https://doi.org/10.1007/s00415-010-5830-y text/html Onlinezugriff via DOI 1 review-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00415-010-5830-y text/html Onlinezugriff via DOI NATIONALLICENCE 100 1- Hardiman Orla HRB Clinician Scientist, Trinity College and Beaumont Hospital, Dublin, Ireland aut NATIONALLICENCE 773 0- Journal of Neurology Springer-Verlag 258/3(2011-03-01), 359-365 0340-5354 258:3<359 2011 258 415 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer