Late onset Pompe disease (LOPD) is a genetic disorder characterized by progressive skeletal and respiratory muscle weakness. Enzyme replacement therapy (ERT) is established as the main treatment of these patients. However, most of them develop antibodies against the recombinant enzyme administered and at present it is unknown the possible effect of antibodies in the effectiveness of the drug. We followed up a cohort of 22 LOPD patients treated with ERT during one year. We performed a group of muscle function tests, a blood analysis and a muscle MRI using quantitative 3 point Dixon of the thigh muscles at baseline and one year visit.

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