Assessment and monitoring of ventilatory function and cough efficacy in patients with amyotrophic lateral sclerosis

Chetta, Alfredo; Aiello, Marina; Tzani, Panagiota; Olivieri, Dario

doi:10.4081/monaldi.2007.509

Cited by 10 publications

(11 citation statements)

References 66 publications

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“…Clinical Use of PFTs.-Monitoring of PFT results can be particularly important in patients with progressive neuromuscular diseases such as ALS, in which impaired respiratory muscle function can lead to respiratory failure or recurrent pulmonary infections. In these patients, standard PFTs as well as measurement of cough efficacy can guide the aggressiveness of supportive therapy (57). In connective tissue diseases such as lupus erythematosus, function testing may help suggest the contribution of diaphragmatic dysfunction to pulmonary dysfunction.…”

Section: Neuromuscular Causes Of a Restrictive Patternmentioning

confidence: 99%

Pulmonary Function Tests for the Radiologist

et al. 2017

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Pulmonary function tests (PFTs) provide important quantitative information about lung function and can be used to elucidate pathologic conditions responsible for respiratory symptoms, assess the severity and course of disease, and evaluate the patient for suitability and timing for lung transplantation. They are typically used in tandem with chest imaging, along with other ancillary data, to arrive at a specific diagnosis. PFTs may provide the radiologist with clues to the diagnosis and grading of a wide variety of pulmonary diseases. In this review, the authors discuss the clinical use of PFTs, their major components, and important measurements and graphical representations that are essential for understanding and interpreting the results. The key components of PFT panels-static lung volumes, dynamic lung function (spirometry), and diffusion capacity-are explained. The authors present a general algorithmic approach for problem solving, with recognition of common patterns of results (obstructive, restrictive, mixed, nonspecific, and normal). Pulmonary diseases from each of the major patterns and chest imaging are illustrated, and correlations between particular PFT results and disease severity and morphology at imaging are examined. Common pitfalls encountered during interpretation are also highlighted. A basic understanding of the mechanics of PFTs, characteristic patterns in important diseases, and correlation between lung function and imaging findings may assist the radiologist in diagnosis and follow-up of key pulmonary diseases and strengthen the radiologist's role as part of a multidisciplinary diagnostic team. Online supplemental material is available for this article. RSNA, 2017.

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Section: Neuromuscular Causes Of a Restrictive Patternmentioning

confidence: 99%

Pulmonary Function Tests for the Radiologist

et al. 2017

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“…It examines the gas transfer from the alveoli into the interior of red blood cells. DLco is reduced but normal or rose when adjusted for volume, and this distinguishes respiratory muscle weakness in ALS patients from alveolar disorders such as pulmonary fibrosis 18 .…”

Section: Gas Exchangementioning

confidence: 99%

Current issues in the respiratory care of patients with amyotrophic lateral sclerosis

Orsini

Lopes

Menezes

et al. 2015

Arq. Neuro-Psiquiatr.

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SHORT OVERVIEW OF ALSAmyotrophic lateral sclerosis (ALS) is characterized by progressive degeneration of both cortical and alpha motor neurons of the final common pathway. Despite being a progressive degenerative process which primarily affects motor systems, ALS is now recognized to involve several non-motor systems and in long survivors affects many subcortical structures. Many hypotheses have been formulated about what causes ALS, including chemical exposures, occupational exposure, military service, infectious agents, nutritional intake, physical activity, and trauma. Worldwide, ALS affects white males aged > 60 years more often than any other group 1 . In the past three decades new practice parameters to the clinical care in ALS were developed and several clinical trials were performed². As ALS remains as an incurable, progressive and fatal condition, the treatment strategies are focused in improving the quality of life 3 . The only effective pharmacological treatment is Riluzole, a putative glutamate release blocker linked to modestly prolonged survival 2 . Other therapeutic measures concerning respiratory support are outlined in this paper. PULMONARY FUNCTION IN ALS Respiratory muscle strengthProgressive weakness remains the foremost characteristic of ALS. However, while some ALS patients progress rapidly, others have slow progression of muscle weakness; ABSTRACTAmyotrophic lateral sclerosis is a progressive neuromuscular disease, resulting in respiratory muscle weakness, reduced pulmonary volumes, ineffective cough, secretion retention, and respiratory failure. Measures as vital capacity, maximal inspiratory and expiratory pressures, sniff nasal inspiratory pressure, cough peak flow and pulse oximetry are recommended to monitor the respiratory function. The patients should be followed up by a multidisciplinary team, focused in improving the quality of life and deal with the respiratory symptoms. The respiratory care approach includes airway clearance techniques, mechanically assisted cough and noninvasive mechanical ventilation. Vaccination and respiratory pharmacological support are also recommended. To date, there is no enough evidence supporting the inspiratory muscle training and diaphragmatic pacing.Keywords: amyotrophic lateral sclerosis, respiratory function, rehabilitation. RESUMOEsclerose lateral amiotrófica é uma doença neuromuscular progressiva que resulta em fraqueza muscular, redução dos volumes pulmonares, tosse ineficaz, retenção de secreção e insuficiência respiratória. Medidas como a capacidade vital, pressão inspiratória e pressão expiratória máximas, pressão inspiratória máxima nasal, pico de fluxo de tosse e oximetria de pulso são recomendados para monitorar a função respiratória. Os pacientes devem ser acompanhados por uma equipe multidisciplinar, buscando melhorias na qualidade de vida e melhores estratégias para lidar com os sintomas respiratórios. A abordagem de cuidados respiratórios inclui técnicas de desobstrução das vias respiratórias, tosse assistida mecanicamente e ventilaç...

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“…Eventually, hypoventilation is observed especially during sleep (7)(8)(9)(10)(11)(12). The ability to generate respiratory-related behaviors such as coughing and swallowing is also impaired in ALS, leading to impaired airway defense and risk of respiratory infections (8,13,14).…”

Section: Alsmentioning

confidence: 99%

Mechanisms of compensatory plasticity for respiratory motor neuron death

Seven

Mitchell

2019

Respiratory Physiology & Neurobiology

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Respiratory motor neuron death arises from multiple neurodegenerative and traumatic neuromuscular disorders. Despite motor neuron death, compensatory mechanisms minimize its functional impact by harnessing intrinsic mechanisms of compensatory respiratory plasticity. However, the capacity for compensation eventually reaches limits and pathology ensues. Initially, challenges to the system such as increased metabolic demand reveal sub-clinical pathology. With greater motor neuron loss, the eventual result is de-compensation, ventilatory failure, ventilator dependence and then death. In this brief review, we discuss recent advances in our understanding of mechanisms giving rise to compensatory respiratory plasticity in response to respiratory motor neuron death including: 1) increased central respiratory drive, 2) plasticity in synapses on spared phrenic motor neurons, 3) enhanced neuromuscular transmission and 4) shifts in respiratory muscle utilization from more affected to less affected motor pools. Some of these compensatory mechanisms may prolong breathing function, but hasten the demise of surviving motor neurons. Improved understanding of these mechanisms and their impact on survival of spared motor neurons will guide future efforts to develop therapeutic interventions that preserve respiratory function with neuromuscular injury/disease.

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Assessment and monitoring of ventilatory function and cough efficacy in patients with amyotrophic lateral sclerosis

Cited by 10 publications

References 66 publications

Pulmonary Function Tests for the Radiologist

Pulmonary Function Tests for the Radiologist

Current issues in the respiratory care of patients with amyotrophic lateral sclerosis

Mechanisms of compensatory plasticity for respiratory motor neuron death

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