{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,22]],"date-time":"2025-10-22T03:08:11Z","timestamp":1761102491905,"version":"3.40.5"},"reference-count":11,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,1,8]],"date-time":"2022-01-08T00:00:00Z","timestamp":1641600000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Data on interruption of enzyme replacement therapy (ERT) are scarce in late onset Pompe disease. Due to the COVID\u201019 crisis, eight neuromuscular reference centers in France were obligated to stop the treatment for 31 patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We collected the motor and respiratory data from our French registry, before COVID\u201019 and at treatment restart.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In 2.2\u00a0months (mean), patients showed a significant deterioration of 37\u00a0m (mean) in the 6\u2010min walk test and a loss of 210\u00a0ml (mean) of forced vital capacity, without ad integrum restoration after 3\u00a0months of ERT restart.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This national study based on data from the French Pompe Registry shows that the interruption of ERT, even as short as a few months, worsens Pompe patients' motor and respiratory function.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.15222","type":"journal-article","created":{"date-parts":[[2021,12,20]],"date-time":"2021-12-20T08:10:29Z","timestamp":1639987829000},"page":"1181-1186","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["Motor and respiratory decline in patients with late onset Pompe disease after cessation of enzyme replacement therapy during COVID\u201019 pandemic"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6654-8090","authenticated-orcid":false,"given":"C\u00e9line","family":"Tard","sequence":"first","affiliation":[{"name":"Inserm Lille University Hospital Center U1172 Lille Neuroscience &amp; Cognition University of Lille  Lille France"},{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emmanuelle","family":"Salort\u2010Campana","sequence":"additional","affiliation":[{"name":"PACA R\u00e9union Rh\u00f4ne Alpes Reference Center for Neuromuscular Diseases FILNEMUS  France"},{"name":"Service of Pr Attarian AP\u2010HM  Marseille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maud","family":"Michaud","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Department of Neurology Nancy Regional University Hospital Center  Nancy France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marco","family":"Spinazzi","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Department of Neurology Angers University Hospital Center  Angers France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aleksandra","family":"Nadaj Pakleza","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Department of Neurology Strasbourg University Hospitals  Strasbourg France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"H\u00e9l\u00e8ne","family":"Durr","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Department of Neurology Strasbourg University Hospitals  Strasbourg France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fran\u00e7oise","family":"Bouhour","sequence":"additional","affiliation":[{"name":"PACA R\u00e9union Rh\u00f4ne Alpes Reference Center for Neuromuscular Diseases FILNEMUS  France"},{"name":"ENMG Service\/Neuromuscular Pathologies Lyon Civil Hospices  Lyon France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claire","family":"Lefeuvre","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Neurology Department Raymond Poincar\u00e9 University Hospital, Garches Public Hospital Network of Paris  Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Romain","family":"Thomas","sequence":"additional","affiliation":[{"name":"Inserm Lille University Hospital Center U1172 Lille Neuroscience &amp; Cognition University of Lille  Lille France"},{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Azzeddine","family":"Arrassi","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Neurology Department Raymond Poincar\u00e9 University Hospital, Garches Public Hospital Network of Paris  Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nadjib","family":"Taouagh","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Neurology Department Raymond Poincar\u00e9 University Hospital, Garches Public Hospital Network of Paris  Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"Pompe Study Group","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guilhem","family":"Sol\u00e9","sequence":"additional","affiliation":[{"name":"AOC Referral Center for Neuromuscular Diseases, Neurology and Neuromuscular Diseases Department FILNEMUS University Hospitals of Bordeaux  Bordeaux France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pascal","family":"Lafor\u00eat","sequence":"additional","affiliation":[{"name":"North East \u00cele\u2010de\u2010France Reference Center for Neuromuscular Diseases FHU PHENIX FILNEMUS  France"},{"name":"Neurology Department Raymond Poincar\u00e9 University Hospital, Garches Public Hospital Network of Paris  Garches France"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,1,8]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurol.2020.04.004"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-018-0243-7"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.21037\/jtd-cus-2020-007"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa0909859"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.3390\/life10090194"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13285"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-014-7402-z"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004711"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-016-8219-8"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/jimd.12272"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-021-10475-z"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.15222","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.15222","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.15222","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,25]],"date-time":"2023-08-25T22:55:43Z","timestamp":1693004143000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.15222"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,1,8]]},"references-count":11,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2022,4]]}},"alternative-id":["10.1111\/ene.15222"],"URL":"https:\/\/doi.org\/10.1111\/ene.15222","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"type":"print","value":"1351-5101"},{"type":"electronic","value":"1468-1331"}],"subject":[],"published":{"date-parts":[[2022,1,8]]},"assertion":[{"value":"2021-10-09","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-12-08","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-01-08","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}