Respiratory muscle testing in amyotrophic lateral sclerosis: a practical approach

Giuseppe F Sferrazza Papa, Giulia M Pellegrino, Hameeda Shaikh, Agata Lax, Luca Lorini, Massimo Corbo

Research output: Contribution to journalReview articlepeer-review


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.

Original languageEnglish
Pages (from-to)11-19
Number of pages9
JournalMinerva Medica
Issue number6 Suppl 1
Publication statusPublished - Dec 2018


  • Amyotrophic Lateral Sclerosis/complications
  • Humans
  • Noninvasive Ventilation/methods
  • Positive-Pressure Respiration/methods
  • Respiratory Function Tests/methods
  • Respiratory Insufficiency/etiology
  • Respiratory Muscles/physiopathology
  • Spirometry


Dive into the research topics of 'Respiratory muscle testing in amyotrophic lateral sclerosis: a practical approach'. Together they form a unique fingerprint.

Cite this