CTICasesAmyotrophic Lateral Sclerosis (ALS) with Chronic Respiratory Failure
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Imaging findings
Coronal and axial CT images from 2010 and 2016 demonstrate progressive, very significant atrophy of chest muscles, including severe thinning of the diaphragmatic crura. Lung volumes are very low with no evidence of fibrosis, honeycombing, or architectural distortion. PFTs show a typical restrictive pattern with decreased FVC.
Key takeaways
ALS can lead to chronic respiratory failure due to neuromuscular weakness, resulting in profound muscle atrophy, particularly of the diaphragm, which is the main muscle of breathing. This manifests as significantly reduced lung volumes and a restrictive pattern on PFTs.
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