Respiratory function tests in amyotrophic lateral sclerosis: The role of maximal voluntary ventilation

Carvalho, Mamede de; Fernandes, Sofia R.; Pereira, Mariana; Gromicho, Marta; Santos, Miguel Oliveira; Alves, Inês; Pinto, Susana; Swash, Michael

doi:10.1016/j.jns.2022.120143

Cited by 4 publications

(6 citation statements)

References 32 publications

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“…Additionally, the values of PEF were moderately correlated with disease severity (assessed by ALSFRS-R score at baseline) and disease progression rate (expressed as ΔALSFRS-R), which was consistent with the results from Pirola A and colleagues [ 17 ]. However, Carvalho et al recently observed that PEF did not significantly reflect the progression rate of ALS in a small ( n = 51) but longitudinal ALS cohort [ 23 ], and further large and prospective study is necessary to confirm the correlation between PEF and disease progression. Yamada et al also reported that PEF could be a specific respiratory marker for the severity and progression of patients with spinal and bulbar muscular atrophy, a rare neuromuscular disease, also known as Kennedy’s disease [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

Peak expiratory flow is a reliably household pulmonary function parameter correlates with disease severity and survival of patients with amyotrophic lateral sclerosis

et al. 2022

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Background Amyotrophic lateral sclerosis (ALS) is an incurable and fatal neurodegenerative disease; most ALS patients die within 3 to 5 years after symptom onset, usually as a consequence of respiratory failure. In the present study, we aim to screen the survival-related pulmonary function parameters, and to explore the predictive value of peak expiratory flow (PEF) in disease severity and prognosis in patients with ALS. Methods The discovery cohort included 202 ALS patients, and the demographic and clinical characteristics of eligible patients were collected and pulmonary function tests were performed using MS-PFT spirometer. In the validation cohort, 62 newly diagnosed ALS patients performed the pulmonary function test by MS-PFT spirometer and household peak flow meter (KOKA) simultaneously. Results Among 12 pulmonary function parameters, FVC, FEV1, PEF, MEF75%, and MVV were identified to be independent predictive factors for survival. PEF was highly correlated with FVC (r = 0.797), MVV (r = 0.877), FEV1 (r = 0.847), and MEF75% (r = 0.963). Besides, the values of PEF were positively associated with disease severity (ALSFRS-R score, rs = 0.539, P < 0.0001), and negatively associated with progression rate (ΔALSFRS-R, rs = -0.316, P < 0.0001). Finally, we also confirmed that the values of KOKA-measured PEF were highly correlated with the ones measured using MS-PFT spirometer (r = 0.9644, p < 0.0001). Conclusions Our work emphasizes the critical role of PFTs in predicting prognosis of ALS patients. PEF is an easily available pulmonary function index, which is also a promising indicator in predicting disease severity and survival for ALS patients.

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Section: Discussionmentioning

confidence: 99%

Peak expiratory flow is a reliably household pulmonary function parameter correlates with disease severity and survival of patients with amyotrophic lateral sclerosis

et al. 2022

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“…In other ALS cohorts, MVV has already been shown to have good correlations with other PFTs, such as FVC, slow vital capacity (SVC), FEV1, SNIP, phrenic nerve amplitude, peak expiratory flow (PEF), and total ALSFRS-R [20]. Recent studies examining independent cohorts [12,13,31] have agreed upon the role of blood carbonate (HCO 3 − ) and base excess (BE) in evaluating early respiratory failure phenomena such as nocturnal hypoventilation, and we also observed a moderate correlation of cMVV with these two ABGs measures.…”

Section: Discussionmentioning

confidence: 99%

“…Concerning ALS, a recent paper suggested MVV as a potentially useful diagnostic and monitoring marker, as it is able to capture subtle early respiratory modifications and is correlated with both PFTs and neurophysiological studies of respiratory muscles [20]. No study of cMVV in ALS has been performed yet, necessitating exploration of the correlation of cMVV with other respiratory measurements, as well as its prognostic role.…”

Section: Introductionmentioning

confidence: 99%

Calculated Maximal Volume Ventilation (cMVV) as a Marker of Early Respiratory Failure in Amyotrophic Lateral Sclerosis (ALS)

Manera,

Torrieri,

Moglia

et al. 2024

Brain Sciences

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Respiratory failure assessment is among the most debatable research topics in amyotrophic lateral sclerosis (ALS) clinical research due to the wide heterogeneity of its presentation. Among the different pulmonary function tests (PFTs), maximal voluntary ventilation (MVV) has shown potential utility as a diagnostic and monitoring marker, able to capture early respiratory modification in neuromuscular disorders. In the present study, we explored calculated MVV (cMVV) as a prognostic biomarker in a center-based, retrospective ALS population belonging to the Piemonte and Valle d’Aosta registry for ALS (PARALS). A Spearman’s correlation analysis with clinical data and PFTs showed a good correlation of cMVV with forced vital capacity (FVC) and a moderate correlation with some other features such as bulbar involvement, ALSFRS-R total score, blood oxygen (pO2), carbonate (HCO3−), and base excess (BE), measured with arterial blood gas analysis. Both the Cox proportional hazard models for survival and the time to non-invasive ventilation (NIV) measurement highlighted that cMVV at diagnosis (considering cMVV(40) ≥ 80) is able to stratify patients across different risk levels for death/tracheostomy and NIV indication, especially considering patients with FVC% ≥ 80. In conclusion, cMVV is a useful marker of early respiratory failure in ALS, and is easily derivable from standard PFTs, especially in asymptomatic ALS patients with normal FVC measures.

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“…In a recent paper, de Carvalho et al . aimed to assess the timechange of various respiratory tests in a group of 51 ALS patients to assess the disease progression [ 18 ]. They found a strong correlation between some spirometric tests, in particular MVV (maximal voluntary ventilation) and phrenic nerve amplitude/area while respiratory muscle strength (MIP and MEP) did not change significantly at any time evaluation.…”

Section: Literature Analysismentioning

confidence: 99%

Diagnosis of amyotrophic lateral sclerosis by respiratory function test

Polverino,

Sampaolo,

Capuozzo

et al. 2023

Multidis Res Med

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The diagnostic criterion for amyotrophic lateral sclerosis (ALS) based on the findings of concomitant clinical and electrophysiological evidence of upper and lower motor neuron involvement may remain unsatisfied for months and in some patients, even for years in the early stage of the disease. Since respiratory involvement is an onset symptom of ALS in only 1-3% of patients, pulmonary assessment has never been considered useful in the early diagnosis of ALS. However, studies on pulmonary function are lacking, especially in those early stages where neurologic tests are also inconclusive. In contrast to the scarcity of data in the early stages, as the disease progresses, it is increasingly enriched by a rich set of symptoms and positive respiratory tests until respiratory failure occurs, which represents the main cause of death in ALS. Hereby we analyze the main pulmonary function tests (PFT) in the various stages of the disease, up to the recent evidence for the possibility of an early diagnosis.

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Respiratory function tests in amyotrophic lateral sclerosis: The role of maximal voluntary ventilation

Cited by 4 publications

References 32 publications

Peak expiratory flow is a reliably household pulmonary function parameter correlates with disease severity and survival of patients with amyotrophic lateral sclerosis

Peak expiratory flow is a reliably household pulmonary function parameter correlates with disease severity and survival of patients with amyotrophic lateral sclerosis

Calculated Maximal Volume Ventilation (cMVV) as a Marker of Early Respiratory Failure in Amyotrophic Lateral Sclerosis (ALS)

Diagnosis of amyotrophic lateral sclerosis by respiratory function test

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