{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T01:10:45Z","timestamp":1785546645611,"version":"3.56.0"},"reference-count":15,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T00:00:00Z","timestamp":1718323200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2024,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Two novel enzyme replacement therapies (ERTs), studied in phase 3 trials in late\u2010onset Pompe patients, reached marketing authorization by the European Medicines Agency in 2022 and 2023. The European Pompe Consortium (EPOC) updates and extends the scope of the 2017 recommendations for starting, switching and stopping ERT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The European Pompe Consortium consists of 25 neuromuscular and metabolic experts from eight European countries. This update was performed after an in\u2010person meeting, three rounds of discussion and voting to provide a consensus recommendation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The patient should be symptomatic, that is, should have skeletal muscle weakness or respiratory muscle involvement. Muscle magnetic resonance imaging findings showing substantial fat replacement can support the decision to start in a patient\u2010by\u2010patient scenario. Limited evidence supports switching ERT if there is no indication that skeletal muscle and\/or respiratory function have stabilized or improved during standard ERT of 12\u2009months or after severe infusion\u2010associated reactions. Switching of ERT should be discussed on a patient\u2010by\u2010patient shared\u2010decision basis. If there are severe, unmanageable infusion\u2010associated reactions and no stabilization in skeletal muscle function during the first 2\u2009years after starting or switching treatment, stopping ERT should be considered. After stopping ERT for inefficacy, restarting ERT can be considered. Six\u2010monthly European Pompe Consortium muscle function assessments are recommended.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The triple\u2010S criteria on ERT start, switch and stop include muscle magnetic resonance imaging as a supportive finding and the potential option of home infusion therapy. Six\u2010monthly long\u2010term monitoring of muscle function is highly recommended to cover insights into the patient's trajectory under ERT.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.16383","type":"journal-article","created":{"date-parts":[[2024,6,14]],"date-time":"2024-06-14T10:35:58Z","timestamp":1718361358000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":36,"title":["Start, switch and stop (triple\u2010S) criteria for enzyme replacement therapy of late\u2010onset Pompe disease: European Pompe Consortium recommendation update 2024"],"prefix":"10.1111","volume":"31","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2757-8131","authenticated-orcid":false,"given":"Benedikt","family":"Schoser","sequence":"first","affiliation":[{"name":"Department of Neurology, Friedrich\u2010Baur\u2010Institute LMU Clinics Munich  Munich Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9161-3301","authenticated-orcid":false,"given":"Nadine A. 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