{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T03:33:11Z","timestamp":1793503991191,"version":"4.5.0"},"reference-count":31,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,10,14]],"date-time":"2021-10-14T00:00:00Z","timestamp":1634169600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p><jats:bold>Introduction:<\/jats:bold> In slowly progressive myopathies, diaphragm weakness early manifests through sleep-related hypoventilation as reflected by nocturnal hypercapnia. This study investigated whether daytime tests of respiratory muscle function and diaphragm ultrasound predict hypercapnia during sleep.<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> Twenty-seven patients with genetic myopathies (myotonic dystrophy type 1 and 2, late-onset Pompe disease, facioscapulohumeral dystrophy; 48 \u00b1 11 years) underwent overnight transcutaneous capnometry, spirometry, measurement of mouth occlusion pressures, and diaphragm ultrasound.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> Sixteen out of 27 patients showed nocturnal hypercapnia (peak p<jats:sub>tc<\/jats:sub>CO<jats:sub>2<\/jats:sub> \u2265 50 mmHg for \u2265 30 min or increase in p<jats:sub>tc<\/jats:sub>CO<jats:sub>2<\/jats:sub> by 10 mmHg or more from the baseline value). In these patients, forced vital capacity (FVC; % predicted) and maximum inspiratory pressure (MIP; % of lower limit or normal or LLN) were significantly reduced compared to normocapnic individuals. Nocturnal hypercapnia was predicted by reduction in FVC of &amp;lt;60% [sensitivity, 1.0; area under the curve (AUC), 0.82] and MIP (%LLN) &amp;lt;120% (sensitivity, 0.83; AUC, 0.84), the latter reflecting that in patients with neuromuscular disease, pretest likelihood of abnormality is <jats:italic>per se<\/jats:italic> higher than in healthy subjects. Diaphragm excursion velocity during a sniff maneuver excluded nocturnal hypercapnia with high sensitivity (0.90) using a cutoff of 8.0 cm\/s.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> In slowly progressive myopathies, nocturnal hypercapnia is predicted by FVC &amp;lt;60% or MIP &amp;lt;120% (LLN). As a novelty, nocturnal hypercapnia can be excluded with acceptable sensitivity by diaphragm excursion velocity &amp;gt;8.0 cm\/s on diaphragm ultrasound.<\/jats:p>","DOI":"10.3389\/fneur.2021.731865","type":"journal-article","created":{"date-parts":[[2021,10,14]],"date-time":"2021-10-14T05:33:16Z","timestamp":1634189596000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":14,"title":["Respiratory Muscle Function Tests and Diaphragm Ultrasound Predict Nocturnal Hypoventilation in Slowly Progressive 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