CTICasesRespiratory Onset Amyotrophic Lateral Sclerosis (ALS)
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Imaging findings
CT initially showed reduced lung volumes with crowding, atelectasis, and elevated hemidiaphragms. The diaphragms and crura appeared very thin, suggesting diaphragmatic dysfunction. Furthermore, localized atrophy of the paraspinal muscles, including the erector spinae and deep paraspinals, was noted.
Key takeaways
Respiratory onset amyotrophic lateral sclerosis (ALS) is a rare but recognized presentation of ALS, where initial symptoms relate to respiratory muscle weakness. Imaging findings suggestive of this include reduced lung volumes, elevated diaphragms (thin), and bilateral reduced diaphragmatic excursion on sniff tests, along with atrophy of paraspinal muscles. Mild elevations of muscle enzymes (CPK) and CRP can support the diagnosis.
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