The prognostic value of spirometric tests in Amyotrophic Lateral Sclerosis patients

Pirola, Alice; Mattia, Elisa De; Sannicolò, Giulia; Carraro, Elena; Rao, Fabrizio; Sansone, Valeria; Lunetta, Christian

doi:10.1016/j.clineuro.2019.105456

Cited by 26 publications

(29 citation statements)

References 38 publications

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“…Another data constraint was that lack of availability of established prognostic indicators in at least one of the four datasets, such as ALSFRS sub scores, cognitive profile, Riluzole intake, vital capacity 30 , time to generalisation 31 or weight loss, which is considered more relevant than absolute weight at baseline 32 . This limited the model’s ability to discriminate patients within the projection space.…”

Section: Discussionmentioning

confidence: 99%

Development and validation of a 1-year survival prognosis estimation model for Amyotrophic Lateral Sclerosis using manifold learning algorithm UMAP

Grollemund

Chat

Secchi-Buhour

et al. 2020

Sci Rep

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Amyotrophic Lateral Sclerosis (ALS) is an inexorably progressive neurodegenerative condition with no effective disease modifying therapies. The development and validation of reliable prognostic models is a recognised research priority. We present a prognostic model for survival in ALS where result uncertainty is taken into account. Patient data were reduced and projected onto a 2D space using Uniform Manifold Approximation and Projection (UMAP), a novel non-linear dimension reduction technique. Information from 5,220 patients was included as development data originating from past clinical trials, and real-world population data as validation data. Predictors included age, gender, region of onset, symptom duration, weight at baseline, functional impairment, and estimated rate of functional loss. UMAP projection of patients shows an informative 2D data distribution. As limited data availability precluded complex model designs, the projection was divided into three zones with relevant survival rates. These rates were defined using confidence bounds: high, intermediate, and low 1-year survival rates at respectively ( ), ( ) and ( ). Predicted 1-year survival was estimated using zone membership. This approach requires a limited set of features, is easily updated, improves with additional patient data, and accounts for results uncertainty.

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Section: Discussionmentioning

confidence: 99%

Development and validation of a 1-year survival prognosis estimation model for Amyotrophic Lateral Sclerosis using manifold learning algorithm UMAP

Grollemund

Chat

Secchi-Buhour

et al. 2020

Sci Rep

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“…The present article is a cross-sectional study approved by the HC/UFG Ethics Committee (Approval number 3981050-19/08/2018). Participants diagnosed with probable-definite ALS (revised El Escorial criteria) [2,[4][5][6][7][8][9] who were treated at the Tertiary Referral Center for Neuromuscular Diseases of Hospital de Apoio de Brasılia, Brazil [7] were enrolled in this study. Informed consent was obtained from the subjects.…”

Section: Study Design and Participantsmentioning

confidence: 99%

“…Epidemiological data were collected as follows: age, sex, Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) score [1,2,[4][5][6]9], and duration of adherence to previous TR3 treatment (in weeks). Additionally, pulmonary function tests were performed prior to the intervention.…”

Section: Epidemiological Data and Baseline Assessmentmentioning

confidence: 99%

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A Pilot Study of the Breath Stacking Technique Associated with Respiratory Muscle Endurance Training in Patients with Amyotrophic Lateral Sclerosis: Videofluoroscopic Findings in the Upper Airway

Dorça

Sarmet

Rocha

et al. 2021

Advances in Respiratory Medicine

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Introduction: Bulbar impairment represents a hallmark feature of amyotrophic lateral sclerosis (ALS) that significantly impacts survival and quality of life. Respiratory complications arise because of the weakness of the upper airway and respiratory muscles leading to respiratory failure, impaired swallowing, and reduced airway safety. Breath stacking and respiratory muscle endurance training are techniques that have been described to improve respiratory and bulbar function in patients with ALS. Considering the above, a respiratory technique named TR3 was developed. This study aimed to measure the acute effects of this technique on the upper airway through videofluoroscopy and to assess its clinical trial feasibility in patients with ALS. Material and methods: In this cross-sectional study, we enrolled participants diagnosed with ALS to perform a single session of TR3. Epidemiological data and baseline assessments were collected. The assessments included kinematics from videofluoroscopy measuring the retropalatal airspace size, the size of the narrowest airway, and the pharyngeal area during rest and TR3. Results: Eight participants were included. During TR3, an acute increase of 15% was observed in the retropalatal airspace size (t = 5.14, p < 0.01), a 123% increase was observed in the size of the narrowest airway (t = -4.18, p < 0.001), and a 277% increase was observed in the pharyngeal area (t = -5.34, p < 0.001). Conclusions: During the intervention, TR3 showed acute effects in increasing pharyngeal constriction, pharyngeal expansion, retropalatal airspace size, and post-lingual narrowest airway size and is feasible for a larger research program. A clinical trial (NCT04226144) is already being conducted to assess the chronic therapeutic effects of this technique and its impact on the clinical evolution of ALS.

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“…Respiratory function is monitored using forced vital capacity (FVC) every 3 months [9]. It is more accurately done lying down [9].…”

Section: Current Treatmentsmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis

Warner¹

2021

Neurodegenerative Diseases - Molecular Mechanisms and Current Therapeutic Approaches

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The term motor neuron disease refers to several diseases affecting the motor neurons and is sometimes used interchangeably to refer to amyotrophic lateral sclerosis (ALS), which is the most common motor neuron disease. This chapter will focus on ALS. A complex combination of molecular pathways and cell interactions cause ALS. About 10% of ALS cases are genetic, although it has been hypothesized that as more genes are discovered to contribute to the disease, a larger percentage of cases will be quoted. This chapter discusses in detail the most common genetic forms of ALS and current research on targeted treatments.

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The prognostic value of spirometric tests in Amyotrophic Lateral Sclerosis patients

Cited by 26 publications

References 38 publications

Development and validation of a 1-year survival prognosis estimation model for Amyotrophic Lateral Sclerosis using manifold learning algorithm UMAP

Development and validation of a 1-year survival prognosis estimation model for Amyotrophic Lateral Sclerosis using manifold learning algorithm UMAP

A Pilot Study of the Breath Stacking Technique Associated with Respiratory Muscle Endurance Training in Patients with Amyotrophic Lateral Sclerosis: Videofluoroscopic Findings in the Upper Airway

Amyotrophic Lateral Sclerosis

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