Dataset Information

Comparison of Slow and Forced Vital Capacity on Ability to Evaluate Respiratory Function in Bulbar-Involved Amyotrophic Lateral Sclerosis

ABSTRACT:

Background and Objective

The percent-predicted forced vital capacity (FVC%) in the pulmonary function test (PFT) is generally used to evaluate the respiratory function in amyotrophic lateral sclerosis (ALS). The slow vital capacity (SVC) is another method to evaluate the respiratory function. Some neurologists found that the FVC% was not reflective of respiratory symptoms and the percent-predicted SVC (SVC%) was found to be higher in some patients with bulbar-onset ALS. We aimed to compare the percent predicted SVC (SVC%) with FVC% in evaluating the respiratory function and investigate the associations between the associations between clinical characteristics and the difference between the SVC% and the FVC% (SVC%-FVC%) in bulbar-involved ALS patients.

Method

This prospective study included patients with bulbar-involved ALS who visited the Peking University Third Hospital between October 2020 and November 2021. They underwent comprehensive clinical assessments, including bulbar symptom assessments, revised ALS functional rating scale (ALSFRS-R), Rasch-Built Overall Amyotrophic Lateral Sclerosis Disability Scale (Roads), and PFTs. The group differences were analyzed using parametric and non-parametric tests.

Results

A total of 59 participants were initially enrolled, and 51 of them were included in the final analysis. In patients with bulbar-involved ALS, the SVC% (73.82 ± 21.95%) was significantly higher (p = 0.013) than the FVC% (71.42 ± 23.15%). After controlling for other relevant variables, a partial correlation analysis showed a significant correlation (r = −0.352, p = 0.041) between ALSFRS-R1 score and SVC%-FVC%.

Conclusion

Our prospective study found that the SVC% was significantly higher and more reflective of actual respiratory function than the FVC% in patients with bulbar-involved ALS. Furthermore, the severity of dysarthria was found to be positively correlated with SVC%-FVC%, providing a clinical marker for predicting SVC%-FVC%.

SUBMITTER: Huang X

PROVIDER: S-EPMC9275792 | biostudies-literature |

REPOSITORIES: biostudies-literature

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Project description:ImportanceThe prognostic value of slow vital capacity (SVC) in relation to respiratory function decline and disease progression in patients with amyotrophic lateral sclerosis (ALS) is not well understood.ObjectiveTo investigate the rate of decline in percentage predicted SVC and its association with respiratory-related clinical events and mortality in patients with ALS.Design, setting, and participantsThis retrospective study included 893 placebo-treated patients from 2 large clinical trials (EMPOWER and BENEFIT-ALS, conducted from March 28, 2011, to November 1, 2012, and from October 23, 2012, to March 21, 2014, respectively) and an ALS trial database (PRO-ACT, containing studies completed between 1990 and 2010) to investigate the rate of decline in SVC. Data from the EMPOWER trial (which enrolled adults with possible, probable, or definite ALS; symptom onset within 24 months before screening; and upright SVC at least 65% of predicted value for age, height, and sex) were used to assess the relationship of SVC to respiratory-related clinical events; 456 patients randomized to placebo were used in this analysis. The 2 clinical trials included patients from North America, Australia, and Europe.Main outcomes and measuresClinical events included the earlier of time to death or time to decline in the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) respiratory subdomain, time to onset of respiratory insufficiency, time to tracheostomy, and all-cause mortality.ResultsAmong 893 placebo-treated patients with ALS, the mean (SD) patient age was 56.7 (11.2) years, and the mean (SD) SVC was 90.5% (17.1%) at baseline; 65.5% (585 of 893) were male, and 20.5% (183 of 893) had bulbar-onset ALS. In EMPOWER, average decline of SVC from baseline through 1.5-year follow-up was -2.7 percentage points per month. Steeper declines were found in patients older than 65 years (-3.6 percentage points per month [P = .005 vs <50 years and P = .007 vs 50-65 years) and in patients with an ALSFRS-R total score of 39 or less at baseline (-3.1 percentage points per month [P < .001 vs >39]). When the rate of decline of SVC was slower by 1.5 percentage points per month in the first 6 months, risk reductions for events after 6 months were 19% for decline in the ALSFRS-R respiratory subdomain or death after 6 months, 22% for first onset of respiratory insufficiency or death after 6 months, 23% for first occurrence of tracheostomy or death after 6 months, and 23% for death at any time after 6 months (P < .001 for all).Conclusions and relevanceThe rate of decline in SVC is associated with meaningful clinical events in ALS, including respiratory failure, tracheostomy, or death, suggesting that it is an important indicator of clinical progression.

Project description:Improved methods for assessing bulbar impairment are necessary for expediting diagnosis of bulbar dysfunction in ALS, for predicting disease progression across speech subsystems, and for addressing the critical need for sensitive outcome measures for ongoing experimental treatment trials. To address this need, we are obtaining longitudinal profiles of bulbar impairment in 100 individuals based on a comprehensive instrumentation-based assessment that yield objective measures. Using instrumental approaches to quantify speech-related behaviors is very important in a field that has primarily relied on subjective, auditory-perceptual forms of speech assessment(1). Our assessment protocol measures performance across all of the speech subsystems, which include respiratory, phonatory (laryngeal), resonatory (velopharyngeal), and articulatory. The articulatory subsystem is divided into the facial components (jaw and lip), and the tongue. Prior research has suggested that each speech subsystem responds differently to neurological diseases such as ALS. The current protocol is designed to test the performance of each speech subsystem as independently from other subsystems as possible. The speech subsystems are evaluated in the context of more global changes to speech performance. These speech system level variables include speaking rate and intelligibility of speech. The protocol requires specialized instrumentation, and commercial and custom software. The respiratory, phonatory, and resonatory subsystems are evaluated using pressure-flow (aerodynamic) and acoustic methods. The articulatory subsystem is assessed using 3D motion tracking techniques. The objective measures that are used to quantify bulbar impairment have been well established in the speech literature and show sensitivity to changes in bulbar function with disease progression. The result of the assessment is a comprehensive, across-subsystem performance profile for each participant. The profile, when compared to the same measures obtained from healthy controls, is used for diagnostic purposes. Currently, we are testing the sensitivity and specificity of these measures for diagnosis of ALS and for predicting the rate of disease progression. In the long term, the more refined endophenotype of bulbar ALS derived from this work is expected to strengthen future efforts to identify the genetic loci of ALS and improve diagnostic and treatment specificity of the disease as a whole. The objective assessment that is demonstrated in this video may be used to assess a broad range of speech motor impairments, including those related to stroke, traumatic brain injury, multiple sclerosis, and Parkinson disease.