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This MRI study aimed to characterize lower limb muscle involvement in classic infantile and young late\u2010onset Pompe patients compared to healthy controls, and examine its relationship with motor function. Quantitative MRI (3\u2010point Dixon and multi\u2010echo spin\u2010echo at 3T) was used to assess fat fraction (FF) and water T2 (T2\n                    <jats:sub>water<\/jats:sub>\n                    ) in the leg muscles of eight classic infantile patients (6.6\u201315.0\u2009years), 12 late\u2010onset patients (6.8\u201327.3\u2009years), and 13 healthy controls (6.5\u201326.9\u2009years). Group comparisons were performed (Kruskal\u2013Wallis, post hoc Dunn), and FF and T2\n                    <jats:sub>water<\/jats:sub>\n                    values were combined to identify the most frequently affected muscles. MRI findings were correlated with Quick Motor Function Test (QMFT) scores for thigh involvement and Medical Research Council (MRC) scores for foot dorsiflexion strength. MRI showed fat replacement and T2\n                    <jats:sub>water<\/jats:sub>\n                    abnormalities in numerous muscles in both patient groups. Fat replacement was generally mild (&lt;\u200920%) but reached 69% in some cases. T2\n                    <jats:sub>water<\/jats:sub>\n                    values ranged from 21 to 37\u2009ms. Classic infantile patients showed more frequent lower leg involvement (80% vs. 40%), while late\u2010onset patients had predominant thigh involvement, particularly in the adductors. Extensive thigh involvement correlated with lower QMFT scores. Foot dorsiflexion weakness occurred only in classic infantile patients with tibialis anterior involvement. This data suggests a phenotype\u2010specific pattern of muscle involvement and demonstrates the value of combining FF and T2\n                    <jats:sub>water<\/jats:sub>\n                    via quantitative MRI for detection of muscle involvement in Pompe disease.\n                  <\/jats:p>","DOI":"10.1002\/jimd.70209","type":"journal-article","created":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:55:35Z","timestamp":1780275335000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Quantitative Muscle\n                    <scp>MRI<\/scp>\n                    of the Lower Extremities Reveals Different Patterns of Involvement in Classic Infantile and Young Late\u2010Onset Pompe Patients"],"prefix":"10.1002","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0948-7313","authenticated-orcid":false,"given":"Jan J. 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