Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis

Vitacca, Michele; Clini, Enrico; Facchetti, D.; Pagani, Marco; Poloni, M.; Porta, Roberto; Ambrosino, Nicolino

doi:10.1183/09031936.97.10071614

Cited by 80 publications

(54 citation statements)

References 35 publications

(42 reference statements)

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“…A truncated VT has been suggested as an alteration in ventilatory control that ultimately leads to chronic CO 2 retention (23). In another study (36), longitudinal data on breathing pattern and respiratory mechanics in ALS patients showed evidence consistent with a restrictive ventilatory pattern derived from progressive weakness of respiratory muscle function. Patients compensated for a decline in respiratory muscle function by demonstrating a rapid shallow breathing pattern (i.e., increasing f and decreasing VT).…”

Section: Discussionmentioning

confidence: 81%

Respiratory impairment in a mouse model of amyotrophic lateral sclerosis

Tankersley¹,

Haenggeli²,

Rothstein³

2007

Journal of Applied Physiology

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Tankersley CG, Haenggeli C, Rothstein JD. Respiratory impairment in a mouse model of amyotrophic lateral sclerosis. J Appl Physiol 102: 926 -932, 2007. First published November 16, 2006; doi:10.1152/japplphysiol.00193.2006.-Amyothrophic lateral sclerosis (ALS) is a progressive, lethal neuromuscular disease that is associated with the degeneration of cortical and spinal motoneurons, leading to atrophy of limb, axial, and respiratory muscles. Patients with ALS invariably develop respiratory muscle weakness and most die from pulmonary complications. Overexpression of superoxide dismutase 1 (SOD1) gene mutations in mice recapitulates several of the clinical and pathological characteristics of ALS and is therefore a valuable tool to study this disease. The present study is intended to evaluate an age-dependent progression of respiratory complications in SOD1 G93A mutant mice. In each animal, baseline measurements of breathing pattern [i.e., breathing frequency and tidal volume (VT)], minute ventilation (VE), and metabolism (i.e., oxygen consumption and carbon dioxide production) were repeatedly sampled at variable time points between 10 and 20 wk of age with the use of whole-body plethysmographic chambers. To further characterize the neurodegeneration of breathing, VE was also measured during 5-min challenges of hypercapnia (5% CO 2) and hypoxia (10% O2). At baseline, breathing characteristics and metabolism remained relatively unchanged from 10 to 14 wk of age. From 14 to 18 wk of age, there were significant (P Ͻ 0.05) increases in baseline VT, VE, and the ventilatory equivalent (VE/oxygen consumption). After 18 wk of age, there was a rapid decline in VE due to significant (P Ͻ 0.05) reductions in both breathing frequency and VT. Whereas little change in hypoxic VE responses occurred between 10 and 18 wk, hypercapnic VE responses were significantly (P Ͻ 0.05) elevated at 18 wk due to an augmented VT response. Like baseline breathing characteristics, hypercapnic VE responses also declined rapidly after 18 wk of age. The phenotypic profile of SOD1 G93A mutant mice was apparently unique because similar changes in respiration and metabolism were not observed in SOD1 controls. The present results outline the magnitude and time course of respiratory complications in SOD1 G93A mutant mice as the progression of disease occurs in this mouse model of ALS.

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Section: Discussionmentioning

confidence: 81%

Respiratory impairment in a mouse model of amyotrophic lateral sclerosis

Tankersley¹,

Haenggeli²,

Rothstein³

2007

Journal of Applied Physiology

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show abstract

“…In addition to weakness of peripheral muscles, respiratory muscle weakness develops during the course of the disease, leading to reduced alveolar ventilation and respiratory failure, which is the main cause of death in ALS. 1,2 A randomized controlled trial showed improvement in survival and quality of life (QoL) in ALS patients treated with noninvasive ventilation (NIV). 3 Although survival in the subgroup of patients with severe bulbar impairment did not improve, small improvements in QoL were observed.…”

Section: Discussionmentioning

confidence: 99%

Noninvasive Ventilation Improves Sleep in Amyotrophic Lateral Sclerosis: A Prospective Polysomnographic Study

Vrijsen

Buyse

Belge

et al. 2015

Journal of Clinical Sleep Medicine

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“…ure, loss of independence, and mortality (10,16,27,28,32). In patients with diabetic neuropathy, localized denervation of small foot muscles leads to foot deformities, a major risk factor for ulcers and amputations (1,2,13,19,30).…”

mentioning

confidence: 99%

Skeletal muscle denervation causes skeletal muscle atrophy through a pathway that involves both Gadd45a and HDAC4

Bongers

Fox

Ebert

et al. 2013

American Journal of Physiology-Endocrinology and Metabolism

125

111

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Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis

Abstract: These results suggest a progressive deterioration in breathing pattern and in respiratory muscle strength with progression of disease.

Cited by 80 publications

References 35 publications

Respiratory impairment in a mouse model of amyotrophic lateral sclerosis

Respiratory impairment in a mouse model of amyotrophic lateral sclerosis

Noninvasive Ventilation Improves Sleep in Amyotrophic Lateral Sclerosis: A Prospective Polysomnographic Study

Skeletal muscle denervation causes skeletal muscle atrophy through a pathway that involves both Gadd45a and HDAC4

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