Noah Lechtzin1, Merit E Cudkowicz2, Mamede de Carvalho3, Angela Genge4, Orla Hardiman5, Hiroshi Mitsumoto6, Jesus S Mora7, Jeremy Shefner8, Leonard H Van den Berg9, Jinsy A Andrews6. 1. a Johns Hopkins University School of Medicine , Baltimore , MD , USA. 2. b Massachusetts General Hospital , Boston , MA , USA. 3. c Faculty of Medicine, IMM, University of Lisbon , Department of Neurosciences-CHLN , Lisbon , Portugal. 4. d Montreal Neurological Institute , Montreal , Canada. 5. e Trinity Biomedical Sciences Institute, Trinity College , Dublin , Ireland. 6. f Eleanor and Lou Gehrig ALS Center, The Neurological Institute, Columbia University , New York , NY , USA. 7. g Unidad de ELA-Hospital Universitario La Paz-Hospital Carlos III , Madrid , Spain. 8. h Department of Neurology , Barrow Neurological Institute , Phoenix , AZ , USA. 9. i Department of Neurology , Brain Centre Rudolf Magnus, University Medical Centre Utrecht , Utrecht , The Netherlands.
Abstract
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.
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.
Authors: Lauren Tabor Gray; Kasey L McElheny; Terrie Vasilopoulos; James Wymer; Barbara K Smith; Emily K Plowman Journal: Dysphagia Date: 2022-08-05 Impact factor: 2.733
Authors: Benjamin Rix Brooks; Terry Heiman-Patterson; Martina Wiedau-Pazos; Shawn Liu; Jeffrey Zhang; Stephen Apple Journal: PLoS One Date: 2022-06-14 Impact factor: 3.752