REVIEW  XIII PNEUMOLAB PROCEEDINGS Free access

Minerva Medica 2018 December;109(6 Suppl 1):11-9

DOI: 10.23736/S0026-4806.18.05920-7

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Respiratory muscle testing in amyotrophic lateral sclerosis: a practical approach

Giuseppe F. SFERRAZZA PAPA ^{1, 2} ✉, Giulia M. PELLEGRINO ^{1, 2}, Hameeda SHAIKH ^{3, 4}, Agata LAX ⁵, Luca LORINI ⁶, Massimo CORBO ¹

¹ Department of Neurorehabilitation Sciences, Casa di Cura Privata del Policlinico, Milan, Italy; ² Respiratory Unit, Department of Health Sciences, ASST Santi Paolo e Carlo, Università degli Studi di Milano, Milan, Italy; ³ Division of Pulmonary and Critical Care Medicine, Loyola University of Chicago Stritch School of Medicine, Maywood, IL, USA; ⁴ Edward Hines Jr. Veterans Administration Hospital Hines, Chicago, IL, USA; ⁵ IRCCS Don Carlo Gnocchi Foundation, Milan, Italy; ⁶ Unit of Neurosurgical Intensive Care, Department of Anesthesia and Critical Care Medicine, Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy

In amyotrophic lateral sclerosis (ALS), respiratory muscle weakness leads to respiratory failure and death. Non-invasive positive pressure ventilation (NIPPV) appears to reduce lung function decline, thus improving survival and quality-of-life of patients affected by the disease. Unfortunately, clinical features and timing to start NIPPV are not well defined. Starting from recent findings, we examine established and novel tests of respiratory muscle function that could help clinicians decide whether and when to start NIPPV in ALS. Non-invasive tests estimate the function of inspiratory, expiratory, and bulbar muscles, whereas clinical examination allows to assess the overall neurologic and respiratory symptoms and general conditions. Most of the studies recommend that together with a thorough clinical evaluation of the patient according to current guidelines, vital capacity, maximal static and sniff nasal inspiratory pressures, maximal static expiratory pressures and peak cough expiratory flow, and nocturnal pulse oximetry be measured. A sound understanding of physiology can guide the physician also through the current armamentarium for additional supportive treatments for ALS, such as symptomatic drugs and new treatments to manage sialorrhea and thickened saliva, cough assistance, air stacking, and physiotherapy. In conclusion, careful clinical and functional evaluation of respiratory function and patient’s preference are key determinants to decide “when” and “to whom” respiratory treatments can be provided.

KEY WORDS: Amyotrophic lateral sclerosis - Respiration disorders - Respiratory muscles - Spirometry