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Updated: May 9, 2025
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Repeated Measurement of Respiratory Muscle Activity and Ventilation in Mouse Models of Neuromuscular Disease
Published on: April 17, 2017
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Respiratory function, survival, and NIV prevalence over time in ALS - a PRECISION ALS study.
Stefan Sennfält1,2, Ammar Al-Chalabi3,4, Alejandro Caravaca Puchades5
1Department of Neurology, Karolinska University Hospital, Stockholm, Sweden.
Summary
Respiratory function declines as Amyotrophic Lateral Sclerosis (ALS) progresses, impacting survival. Noninvasive ventilation (NIV) use increases with worsening respiratory function, but its introduction varies among patients.
Area of Science:
- Neurology
- Pulmonology
- Epidemiology
Background:
- Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disease.
- Respiratory function decline is a major determinant of survival in ALS patients.
Purpose of the Study:
- To describe respiratory function trajectory in ALS.
- To determine the prevalence of noninvasive ventilation (NIV) use in ALS patients.
- To analyze the relationship between respiratory function, NIV, and survival.
Main Methods:
- Prospective data from 3449 ALS patients across six European cohorts.
- Analysis of vital capacity percent predicted (VC%) and ALS Functional Rating Scale-Revised (ALSFRS-R).
- Investigation of genetic status (C9orf72, SOD1, FUS, TARDBP) and NIV use in relation to survival.
Main Results:
- Median VC% declined significantly over time, averaging a 24 percentage point decrease per year.
- Respiratory symptoms increased relative to general functional impairment (ALSFRS-R).
- NIV prevalence correlated with VC%: >80% (3%), 50-80% (15%), <50% (25%).
Conclusions:
- Worsening respiratory function is a key feature of ALS progression.
- Respiratory function is strongly associated with patient survival.
- Significant variability exists in NIV implementation for patients with impaired respiratory function.
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