TY - JOUR
T1 - Respiratory measures in amyotrophic lateral sclerosis
AU - Lechtzin, Noah
AU - Cudkowicz, Merit E.
AU - de Carvalho, Mamede
AU - Genge, Angela
AU - Hardiman, Orla
AU - Mitsumoto, Hiroshi
AU - Mora, Jesus S.
AU - Shefner, Jeremy
AU - Van den Berg, Leonard H.
AU - Andrews, Jinsy A.
N1 - Funding Information:
NL serves as a consultant/advisor for Cytokinetics, Hill-Rom, Vertex, and PMD Healthcare and lectured and participated at meetings on behalf of Cytokinetics and Hill-Rom; MEC serves as a consultant/advisor to Biogen, Biohaven, Cytokinetics, Lilly, Karyopharm, Denali, Wave, and Mitsubishi; MdC serves as a consultant/advisor for Biogen, Cytokinetics, and Kedrion, is an investigator at the Institute of Molecular Medicine, is involved with scientific studies/trials sponsored by AB Science and Cytokinetics, and serves as a board member/officer/trustee for Amyotrophic Lateral Sclerosis & Frontotemporal Degeneration, Clinical Neurophysiology-Neurophysiologie Clinique, and Neurology Research International; AG is an investigator for AB Sciences, Alexion, AL-S Pharma, Baxter, Bioblast, Biogen, CSL Behring, Cytokinetics, Genzyme, Grifols, Ionis, Novartis, Roche, Sanofi, and UCB; OH is an investigator for Cytokinetics and has served as a consultant for Biogen, Cytokinetics, Novartis, Roche, Merck and Mitsubishi. She is editor in chief of the journal Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration; HM is an advisory board member for Cytokinetics, Mitsubishi-Tanabe, and Sunovion and is an investigator for CDC, Cytokinetics, NIH, and Tsumura; JSM serves as a consultant/advisor for AB Science, Biogen, and Cytokinetics and is involved with scientific studies/trials sponsored by AB Science, Biogen, and Cytokinetics; JS is a consultant to Biogen, Inc., Cytokinetics, Inc., Mitsubishi Tanabe Pharma, Denali, Neuraltus Pharmaceuticals, Inc, and Biohaven and has received grant funding from Cytokinetics, Neuraltus, Biogen, ALS Association, Muscular Dystrophy Association, and the ALS Finding a Cure Foundation. LHvdB serves as an advisory board member for Biogen, Cytokinetics, and Orion; and JAA is a consultant/advisor for Cytokinetics and is an investigator for Neuraltus and Roche. This work was supported by Cytokinetics, Inc., which provided funding for writing and editorial support provided by Deb Stull, PhD, on behalf of Evidence Scientific Solutions, Philadelphia, PA, USA.
Publisher Copyright:
© 2018, © 2018 The Author(s). Published by Informa UK Limited, trading as Taylor & Francis Group.
PY - 2018/7/3
Y1 - 2018/7/3
N2 - Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3–5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable. Methods: This review describes the tests used to assess respiratory muscle and pulmonary function in patients with ALS and the correlations between different respiratory measures and clinical outcomes measures. Results: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece. Other tests such as slow VC, sniff inspiratory pressure, or transdiaphragmatic pressure with magnetic stimulation are also associated with distinct advantages and disadvantages. Conclusions: Therefore, how and when to use different tests remains unclear. Understanding how each test relates to disease progression and survival may help determine which is best suited for specific clinical decisions.
AB - Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3–5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable. Methods: This review describes the tests used to assess respiratory muscle and pulmonary function in patients with ALS and the correlations between different respiratory measures and clinical outcomes measures. Results: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece. Other tests such as slow VC, sniff inspiratory pressure, or transdiaphragmatic pressure with magnetic stimulation are also associated with distinct advantages and disadvantages. Conclusions: Therefore, how and when to use different tests remains unclear. Understanding how each test relates to disease progression and survival may help determine which is best suited for specific clinical decisions.
KW - Non-invasive ventilation
KW - amyotrophic lateral sclerosis
KW - pulmonary function tests
KW - slow vital capacity
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U2 - 10.1080/21678421.2018.1452945
DO - 10.1080/21678421.2018.1452945
M3 - Review article
C2 - 29566571
AN - SCOPUS:85044358784
SN - 2167-8421
VL - 19
SP - 321
EP - 330
JO - Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
JF - Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
IS - 5-6
ER -