{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T10:23:02Z","timestamp":1790677382942,"version":"4.1.0"},"reference-count":22,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2010,9,16]],"date-time":"2010-09-16T00:00:00Z","timestamp":1284595200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2010,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Muscle quality is defined as muscle strength generated per unit muscle mass. If enzyme replacement therapy (ERT) has some effects on type II glycogenosis (GSDII) skeletal muscle pathology, we should be able to measure a change in strength and mass. We conducted a prospective study including 11 patients aged 54.2\u2009\u00b1\u200911.2 years, referring to a single institution and receiving ERT for \u22652 years. Median Walton score was 3 (2.5\u20136). Lower limb skeletal muscles were assessed by dynamometry and quantitative muscle MRI. Three segments (anterior thigh, posterior thigh, leg) were analysed separately. Clinical\u2010MRI correlations were searched for at T0, T6\/T8, and T18\/24. Changes in lean and fat body composition were assessed by bioelectrical impedance analysis. We found that the anterior thigh showed the best therapeutic response, with an improvement in muscle quality (muscle mass: +7.5%, <jats:italic>p<\/jats:italic>\u2009=\u20090.035; strength: +45%, <jats:italic>p<\/jats:italic>\u2009=\u20090.002). BMI and lean body mass increased (<jats:italic>p<\/jats:italic>\u2009=\u20090.007). Patients with low BMI showed a better outcome. Intramuscular fat accumulation significantly progressed in spite of ERT (+3.7%, <jats:italic>p<\/jats:italic>\u2009=\u20090.001), especially in the poorly responsive posterior thigh muscles. Both clinical assessment and MRI revealed a definite improvement in the anterior thigh muscles. However, progression of intramuscular fat accumulation during ERT, as well as the limited responsiveness of posterior thigh muscles, suggests the necessity for early treatment intervention. The better outcome of patients with low BMI, if confirmed, may indicate that dietary protocols could be adopted as adjuvant measures to ERT in adult GSDII.<\/jats:p>","DOI":"10.1007\/s10545-010-9204-5","type":"journal-article","created":{"date-parts":[[2010,9,15]],"date-time":"2010-09-15T07:52:34Z","timestamp":1284537154000},"page":"737-745","source":"Crossref","is-referenced-by-count":35,"title":["Changes in skeletal muscle qualities during enzyme replacement therapy in late\u2010onset type II glycogenosis: temporal and spatial pattern of mass vs. strength response"],"prefix":"10.1002","volume":"33","author":[{"given":"Sabrina","family":"Ravaglia","sequence":"first","affiliation":[{"name":"Department of Neurological Sciences University of Pavia Pavia Italy"},{"name":"Institute of Neurology \u201cC. Mondino\u201d via Mondino 2 Pavia 27100 Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Pichiecchio","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit Institute of Neurology IRCCS \u201cC. Mondino\u201d Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michela","family":"Ponzio","sequence":"additional","affiliation":[{"name":"Department of Health Sciences, Section of Medical Statistics and Epidemiology University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cesare","family":"Danesino","sequence":"additional","affiliation":[{"name":"Medical Genetics and IRCCS Policlinico San Matteo University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kolsoum","family":"Saeidi Garaghani","sequence":"additional","affiliation":[{"name":"Medical Genetics and IRCCS Policlinico San Matteo University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guy Umberto","family":"Poloni","sequence":"additional","affiliation":[{"name":"Neuroradiology Unit Institute of Neurology IRCCS \u201cC. Mondino\u201d Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Toscano","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Messina Messina Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arrigo","family":"Moglia","sequence":"additional","affiliation":[{"name":"Department of Neurological Sciences University of Pavia Pavia Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annalisa","family":"Carlucci","sequence":"additional","affiliation":[{"name":"Respiratory Intensive Care Unit Fondazione S. 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