Pulmonary Function at Diagnosis of Amyotrophic Lateral Sclerosis

Schiffman, Philip L.; Belsh, Jerry M.

doi:10.1378/chest.103.2.508

Cited by 163 publications

(112 citation statements)

References 8 publications

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“…As respiratory dysfunction remains the usual cause of death in ALS, measurement and quantification of respiratory function in these patients is, on the face of it, one of the most important measures to obtain [36,37]. As respiratory dysfunction is mainly caused by muscle weakness and produces a restrictive pattern, one of the most utilized measures in clinical trials is the forced vital capacity (FVC) [22,27,38].…”

Section: Respiratory Functionmentioning

confidence: 99%

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Rutkove

2015

Neurotherapeutics

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Progressive weakness remains the clinical hallmark of amyotrophic lateral sclerosis (ALS). Accordingly, a variety of tools has been developed to capture this disease feature, including questionnaires, such as the ALS-functional rating scale, strength testing, pulmonary function tests, electrophysiologic measures, including motor unit number estimation, and imaging techniques. Despite this plethora of approaches, there is little agreement as to what measures to use in a given clinical trial or in the clinic during routine patient care. Part of the reason for this uncertainty is that ALS is a remarkably protean disease. Some individuals progress rapidly, others slowly; some patients have considerable upper motor neuron dysfunction, whereas others have little; and there is considerable variation in the sequence of body regions affected, in some the disease beginning in the bulbar musculature and in others in one arm or one leg. Here, I present a variety of basic and more complex clinical measures for potential use in therapeutic trials with the aim of offering a balanced and practical set of recommendations, as well as considerations for future studies.

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Section: Respiratory Functionmentioning

confidence: 99%

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Rutkove

2015

Neurotherapeutics

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“…Training and improving the force‐generating ability of the muscles involved in expiration is a direct physiologic goal of targeted expiratory training; however, the impact of targeting both inspiratory and expiratory pressure generation may yield improved outcomes related to both airway protection and ventilator dependence and has yet to be investigated in individuals with ALS. Further, early intervention for respiratory insufficiency in ALS may be beneficial in managing respiratory function and tracking disease progression 20. It is important to note potential limitations of these findings, including the possibility that JR could distinguish the sham from the active treatment trainer and concern for testing effect of cough spirometry measurements on cough volume outcomes.…”

Section: Discussionmentioning

confidence: 99%

Respiratory training in an individual with amyotrophic lateral sclerosis

Tabor

Rosado

Robison

et al. 2016

Ann Clin Transl Neurol

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We examined the impact of expiratory muscle strength training on maximum expiratory pressure, cough spirometry, and disease progression in a 71‐year‐old male with amyotrophic lateral sclerosis. Maximum expiratory pressure declined 9% over an 8‐week sham training period, but subsequently improved by 102% following 8 weeks of expiratory muscle strength training. Improvements in cough spirometry and mitigated disease progression were also observed post expiratory muscle strength training. Improvements in maximum expiratory pressures were maintained 6 months following expiratory muscle strength training and were 79% higher than baseline data obtained 301 days prior. In this spinal‐onset amyotrophic lateral sclerosis patient, respiratory training improved subglottic air pressure generation and sequential cough generation.

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“…Inspiratory and expiratory muscle weakness can be assessed by routine respiratory function measurements. 6,7 Patients with ALS usually die from hypoventilation, 8 with hypoxemia and hypercapnia often precipitated by respiratory infections, aspiration pneumonia, or bronchial impaction. 9 The first study of NIV in ALS reported that continuous daily use delayed or even eliminated the need for a tracheostomy.…”

Section: When To Start Niv?mentioning

confidence: 99%

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium

et al. 2015

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During the last few decades, attention has increasingly focused on noninvasive ventilation (NIV) in the treatment of chronic respiratory failure. The University of Leuven and the University Hospitals Leuven therefore chose this topic for a 2-day working group session during their International Symposium on Sleep-Disordered Breathing. Numerous European experts took part in this session and discussed (1) NIV in amyotrophic lateral sclerosis (when to start NIV, NIV and sleep, secretion management, and what to do when NIV fails), (2) recent insights in NIV and COPD (high-intensity NIV, NIV in addition to exercise training, and NIV during exercise training), (3) monitoring of NIV (monitoring devices, built-in ventilator software, leaks, and asynchronies) and identifying events during NIV; and (4) recent and future developments in NIV (target-volume NIV, electromyography-triggered NIV, and autoregulating algorithms)

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Pulmonary Function at Diagnosis of Amyotrophic Lateral Sclerosis

Cited by 163 publications

References 8 publications

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Clinical Measures of Disease Progression in Amyotrophic Lateral Sclerosis

Respiratory training in an individual with amyotrophic lateral sclerosis

Hot Topics in Noninvasive Ventilation: Report of a Working Group at the International Symposium on Sleep-Disordered Breathing in Leuven, Belgium

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