Related Experiment Video
Updated: Feb 14, 2026
12:43
A Protocol for Comprehensive Assessment of Bulbar Dysfunction in Amyotrophic Lateral Sclerosis ALS
Published on: February 21, 2011
36.0K
Cognitive Dysfunction Is Associated With an Underestimation of Respiratory Function in ALS.
Horácio de Jesus1, Inês Alves1, Diana Lopes1
1Institute of Physiology, MUHCRI and Centro de Estudos Egas Moniz. Faculty of Medicine, University of Lisbon, Lisbon, Portugal.
Muscle & Nerve
|February 13, 2026
Summary
Forced vital capacity (FVC) may inaccurately reflect respiratory function in amyotrophic lateral sclerosis (ALS) patients with cognitive impairment. Non-volitional measures are recommended for these individuals to better assess diaphragmatic strength.
Area of Science:
- Neurology
- Pulmonology
- Cognitive Science
Background:
- The relationship between reduced forced vital capacity (FVC) and cognitive impairment in amyotrophic lateral sclerosis (ALS) is unclear.
- Cognitive deficits may influence patient effort, complicating the assessment of respiratory function via FVC.
Purpose of the Study:
- To investigate how cognitive status affects the association between diaphragmatic strength and FVC in ALS patients.
- To utilize objective, non-volitional phrenic nerve motor response amplitude (PAmp) to differentiate respiratory dysfunction from cognitive influences on FVC.
Main Methods:
- Retrospective analysis of 73 ALS patients.
- Measurement of FVC and PAmp.
- Cognitive assessment using the Edinburgh Cognitive and Behavioral ALS Screen (ECAS).
- Regression models to analyze FVC, ECAS domains, and PAmp interactions.
Main Results:
- PAmp and its interaction with abnormal ECAS Executive scores significantly predicted FVC.
- Patients with impaired executive function showed lower FVC at comparable PAmp levels.
- Reduced PAmp correlated with a less pronounced decline in FVC among those with executive dysfunction.
- Severe bulbar dysfunction was negatively associated with FVC.
Conclusions:
- FVC may underestimate respiratory capacity in ALS patients with cognitive impairment, particularly those with executive dysfunction.
- Non-volitional measures like PAmp are recommended for a more accurate respiratory assessment in ALS patients with executive dysfunction.
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