Ventilation treatment in patients with amyotrophic lateral sclerosis

Authors: S. Dostálová;  D. Kemlink;  I. Příhodová;  M. Srp;  M. Miler;  K. Šonka
Authors‘ workplace: Neurologická klinika a centrum klinických, neurověd 1. LF UK a VFN v Praze
Published in: Cesk Slov Neurol N 2021; 84/117(3): 232-236
Category: Review Article


Amyotrophic lateral sclerosis is a neurodegenerative disease affecting both upper and lower motoneurones, which leads to progressive weakness of muscles. Respiratory failure caused by muscle weakness is the major cause of death in this disease. As respiratory complications can be improved by treatment, it is necessary to pay signifi cant attention to respiratory functions at each patient review. Symptoms related to respiratory muscle weakness (dyspnea, tachypnea, orthopnea, use of auxiliary respiratory muscles at rest, paradoxical respiration, disturbed sleep, morning headache, daytime fatigue, excessive daytime sleepiness) should be observed, and respiratory functions require monitoring, especially forced vital capacity and sniff nasal pressure. An important dia gnostic feature is the early detection of nocturnal hypoventilation with the help of polysomnography or polygraphy, which are supplemented with blood gas analysis, ideally using transcutaneous capnography. Timely use of carefully monitored ventilation therapy improves the health status, quality of life, and survival of patients with amyotrophic lateral sclerosis. The essential treatment modality is non-invasive ventilation through a nasal or full-face mask allowing the achievement of the target tidal volume, backup respiratory rate, and automatic pressure adjustment. In order to ensure sufficient cough eff ectiveness cough-assisting devices are applied.


Amyotrophic lateral sclerosis – hypoventilation – capnography – noninvasive ventilation


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