{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T00:24:51Z","timestamp":1778631891426,"version":"3.51.4"},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2011,7,13]],"date-time":"2011-07-13T00:00:00Z","timestamp":1310515200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Washington University Neuromuscular Research"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["K12 NS00169009"],"award-info":[{"award-number":["K12 NS00169009"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2011,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:italic>Introduction<\/jats:italic>: Acid maltase deficiency (AMD, or Pompe disease) is an inherited myopathic disorder of glycogen degradation. Diagnosis is often delayed. Muscle ultrasound could improve diagnosis. <jats:italic>Methods<\/jats:italic>: We compared skeletal muscle ultrasound images from adults with AMD (<jats:italic>n<\/jats:italic> = 10) to other myopathies (<jats:italic>n<\/jats:italic> = 81) and, in AMD, compared qualitative (Heckmatt) and quantitative (backscatter) ultrasound measurements with strength and function. <jats:italic>Results<\/jats:italic>: Qualitative ultrasound was abnormal in at least one muscle in all AMD subjects. Ultrasound patterns specific for AMD were: normal triceps brachii despite abnormalities in elbow flexors (89% vs. 17%, <jats:italic>P<\/jats:italic> &lt; 0.0001); focal abnormalities affecting deep more than superficial biceps brachii (40% vs. 4%, <jats:italic>P<\/jats:italic> = 0.002); and more severe involvement of vastus intermedius than rectus femoris (40 vs. 11%, <jats:italic>P<\/jats:italic> = 0.03). In AMD, both qualitative (Heckmatt) and quantitative (backscatter) ultrasound measures increased with decreasing strength and function. <jats:italic>Conclusions<\/jats:italic>: Muscle ultrasound identifies the presence and specific patterns of AMD pathology, measures disease severity, and can help in the diagnosis of AMD. 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