{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T11:23:07Z","timestamp":1783596187713,"version":"3.55.0"},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,3,14]],"date-time":"2025-03-14T00:00:00Z","timestamp":1741910400000},"content-version":"vor","delay-in-days":13,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,3]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Most patients with aquaporin\u20104 antibody\u2010positive neuromyelitis optica spectrum disorder (AQP4\u2010IgG NMOSD) require life\u2010long immunosuppression to prevent relapses. Patients who are untreated or undergo de\u2010escalation of therapy typically experience severe disabling relapses. We present a series of patients who, despite not receiving immunosuppression, developed minimal disability.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Case series from a UK national NMOSD referral centre. We defined benign disease as an estimated disability status scale score of \u2264\u20093 after a minimum of 4\u2009years without immunotherapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 153 AQP4\u2010IgG NMOSD patients, 8 (5.2%) had a benign disease course after a median follow\u2010up of 7.5\u2009years (Q1: 5.8, Q3: 13.3) without immunotherapy. All patients were female, and 7\/8 were of White racial background. Clinical attacks included isolated optic neuritis, transverse myelitis, area postrema syndrome or combinations of these syndromes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The presence of benign NMOSD and the potential for safe de\u2010escalation of therapy in NMOSD remains unclear. This study suggests that both may be possible. Further studies of similar cases could provide valuable insights and identify biomarkers for safe treatment discontinuation.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70049","type":"journal-article","created":{"date-parts":[[2025,3,15]],"date-time":"2025-03-15T05:49:11Z","timestamp":1742017751000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Is a Benign Disease Course Possible in Untreated <scp>AQP4<\/scp>\u2010<scp>IgG NMOSD<\/scp>?"],"prefix":"10.1111","volume":"32","author":[{"given":"Pakeeran","family":"Siriratnam","sequence":"first","affiliation":[{"name":"School of Translational Medicine Monash University  Melbourne Australia"},{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"},{"name":"Department of Neurology Alfred Health  Melbourne Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Rocchi","sequence":"additional","affiliation":[{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily","family":"Gibbons","sequence":"additional","affiliation":[{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Kelly","sequence":"additional","affiliation":[{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha","family":"Linaker","sequence":"additional","affiliation":[{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Saif","family":"Huda","sequence":"additional","affiliation":[{"name":"Neurology Department Walton Centre NHS Foundation Trust  Liverpool UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,3,14]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001729"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2023.103465"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000209940"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2011.127"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.msard.2023.104730"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000947"},{"issue":"3","key":"e_1_2_8_8_1","first-page":"38","article-title":"Redefining Acute Relapses in Multiple Sclerosis: Implications for Phase 3 Clinical Trials and Treatment Algorithms","volume":"14","author":"Avasarala J.","year":"2017","journal-title":"Innovations in Clinical Neuroscience"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.53.5.1107"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000351"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.52092"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26623"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000727"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.26722"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.msard.2022.104414"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000572"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.70049","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,12]],"date-time":"2025-05-12T10:48:43Z","timestamp":1747046923000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.70049"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,3]]},"references-count":15,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,3]]}},"alternative-id":["10.1111\/ene.70049"],"URL":"https:\/\/doi.org\/10.1111\/ene.70049","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,3]]},"assertion":[{"value":"2024-10-25","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-01-10","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-03-14","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70049"}}