Respiratory measures in amyotrophic lateral sclerosis

Lechtzin, Noah; Cudkowicz, Merit; Carvalho, Mamede de; Genge, Angela; Hardiman, Orla; Mitsumoto, Hiroshi; Mora, Jesús; Shefner, Jeremy M.; Berg, Leonard H. van den; Andrews, Jinsy

doi:10.1080/21678421.2018.1452945

Cited by 55 publications

(46 citation statements)

References 83 publications

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“…ALS affects about 0.03% of the world’s population, and death usually occurs 3–4 years after the initial diagnosis ( Longinetti and Fang, 2019 ). ALS causes muscle weakness and atrophy, which affects all of the muscles, ultimately resulting in death due to respiratory failure ( Lechtzin et al, 2018 ). ALS pathogenesis is characterized by pronounced vascular changes and blood flow disturbances, accompanied by decreased expressions of EPO and VEGF ( Miyazaki et al, 2011 ; Pronto-Laborinho et al, 2014 ), which result in tissue hypoxia.…”

Section: Role Of Hif-1α In Neurodegenerative Diseasesmentioning

confidence: 99%

Hypoxia-Inducible Factor (HIF) in Ischemic Stroke and Neurodegenerative Disease

Митрошина

Savyuk

Ponimaskin

et al. 2021

Front. Cell Dev. Biol.

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Hypoxia is one of the most common pathological conditions, which can be induced by multiple events, including ischemic injury, trauma, inflammation, tumors, etc. The body’s adaptation to hypoxia is a highly important phenomenon in both health and disease. Most cellular responses to hypoxia are associated with a family of transcription factors called hypoxia-inducible factors (HIFs), which induce the expression of a wide range of genes that help cells adapt to a hypoxic environment. Basic mechanisms of adaptation to hypoxia, and particularly HIF functions, have being extensively studied over recent decades, leading to the 2019 Nobel Prize in Physiology or Medicine. Based on their pivotal physiological importance, HIFs are attracting increasing attention as a new potential target for treating a large number of hypoxia-associated diseases. Most of the experimental work related to HIFs has focused on roles in the liver and kidney. However, increasing evidence clearly demonstrates that HIF-based responses represent an universal adaptation mechanism in all tissue types, including the central nervous system (CNS). In the CNS, HIFs are critically involved in the regulation of neurogenesis, nerve cell differentiation, and neuronal apoptosis. In this mini-review, we provide an overview of the complex role of HIF-1 in the adaptation of neurons and glia cells to hypoxia, with a focus on its potential involvement into various neuronal pathologies and on its possible role as a novel therapeutic target.

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Section: Role Of Hif-1α In Neurodegenerative Diseasesmentioning

confidence: 99%

Hypoxia-Inducible Factor (HIF) in Ischemic Stroke and Neurodegenerative Disease

Митрошина

Savyuk

Ponimaskin

et al. 2021

Front. Cell Dev. Biol.

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“…DD was diagnosed if at least one of the following criteria was present: PAM, which is an objective finding of DD 5,11,12 sitting‐supine FVC difference ≥ 20%, 14 which is an objective finding of DD. Supine FVC was measured when the patient was lying relaxed. sitting‐supine SpO 2 difference ≥ 4%.…”

Section: Methodsmentioning

confidence: 99%

“…PAM, which is an objective finding of DD. 5,11,12 2. sitting-supine FVC difference ≥ 20%, 14 which is an objective finding of DD. Supine FVC was measured when the patient was lying relaxed.…”

Section: Methodsmentioning

confidence: 99%

Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis

et al. 2021

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Introduction In this study, we aimed to evaluate diaphragmatic dysfunction (DD) by using a practical approach in patients with amyotrophic lateral sclerosis (ALS) at the first visit to a chest diseases outpatient clinic. Methods Patients with ALS seen in our outpatient clinic for the past 5 y and followed up for at least 1 y, were retrospectively evaluated. Having at least one of the following three criteria was accepted as DD: (a) paradoxical abdominal movement (PAM), (b) sitting‐supine forced vital capacity (FVC) difference ≥ 20%, (c) sitting‐supine arterial oxygen saturation measured by pulse oximetry (SpO2) difference ≥ 4%. Respiratory symptoms, arterial blood gas analysis, sleep studies, noninvasive mechanical ventilation use, and mortality were recorded. Results Five‐hundred patients with ALS were included (female/male: 220/280, age: 58.9 ± 11.3 y). Of the patients, 22.8% had daytime hypercapnia. DD was observed in 55% of the patients (PAM in 112, sitting‐supine FVC difference ≥ 20% in 50, and sitting‐supine SpO2 difference ≥ 4% in 113 patients). Of the patients with DD, 31.6% (n = 87) had no respiratory symptoms, 46.4% had FVC > 70% and 33.5% had FVC <50%. Nocturnal hypoxemia (sleep time spent with SpO2 < 90% ≥30%) was present in 59.7%, and all patients with nocturnal hypoxemia had DD. Obstructive sleep apnea (8 severe, 14 moderate, 39 mild) was detected in 55% of the patients with polysomnography (n = 61) or polygraphy (n = 50). During follow‐up, 52.2% of the patients died. Mean survival time was shorter in patients with DD (P < .001). Conclusion Paradoxical abdomimal movement (PAM), sitting‐supine SpO2 difference ≥ 4% and sitting‐supine FVC difference ≥ 20% are indicators of DD, which should be routinely evaluated at every outpatient visit.

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“…18 However, when FVC or SVC are used as the main indication criterion for NIV, one must keep in mind that results of bulbar patients who lack facial strength can be inaccurate when a mouthpiece is used. 19 Also, FVC and SVC are late markers of respiratory decline and may be normal, although nocturnal hypoventilation is already present. 20 Recently, the measurement of diaphragm compound muscle action potential (CMAP) to phrenic nerve stimulation has been reported as a reliable method that is easy to apply.…”

Section: Indication Criteria and Timing Of Niv Initiationmentioning

confidence: 99%

Non-invasive ventilation in amyotrophic lateral sclerosis

Dorst

Ludolph

2019

Ther Adv Neurol Disord

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Non-invasive ventilation (NIV) has become an important cornerstone of symptomatic treatment in amyotrophic lateral sclerosis (ALS), improving survival and quality of life. In this review, we summarize the most important recent developments and insights, including evidence of efficacy, indication criteria and time of initiation, ventilation parameters and adaptation strategies, treatment of complicating factors, transition from NIV to invasive ventilation, termination of NIV and end-of-life management. Recent publications have questioned former conventions and guideline recommendations, especially with regard to timing and prognostic factors; therefore, a fresh look and re-evaluation of current evidence is needed.

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Respiratory measures in amyotrophic lateral sclerosis

Abstract: 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.

Cited by 55 publications

References 83 publications

Hypoxia-Inducible Factor (HIF) in Ischemic Stroke and Neurodegenerative Disease

Hypoxia-Inducible Factor (HIF) in Ischemic Stroke and Neurodegenerative Disease

Diaphragmatic dysfunction at the first visit to a chest diseases outpatient clinic in 500 patients with amyotrophic lateral sclerosis

Non-invasive ventilation in amyotrophic lateral sclerosis

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