{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,9]],"date-time":"2026-03-09T22:12:32Z","timestamp":1773094352084,"version":"3.50.1"},"reference-count":38,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,3,26]],"date-time":"2021-03-26T00:00:00Z","timestamp":1616716800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:p><jats:bold>Objective:<\/jats:bold> To analyze the positive and recurrence rates of different autoantibody-associated demyelination disorders in children in Southwest China, and describe the clinical, radiological, and prognostic features of the myelin oligodendrocyte glycoprotein antibody (MOG-ab) and aquaporin-4 antibody (AQP4-ab) associated disease. This study also summarizes steroid maintenance therapy approaches for MOG-ab-positive children.<\/jats:p><jats:p><jats:bold>Methods:<\/jats:bold> A total of 160 children presenting with acquired demyelinating syndromes (ADS) between January 2016 and December 2019 were tested for MOG-ab and AQP4-ab. Clinical data, MRI scans, and survival analyses were compared between MOG-ab-positive and AQP4-ab-positive children. Evolution of serologic status and treatment response to immunosuppressants were collected in MOG-ab-positive children.<\/jats:p><jats:p><jats:bold>Results:<\/jats:bold> Of the 160 included children, the MOG-ab positivity rate (47.4%) was significantly higher than the AQP4-ab (5%) positivity rate. The recurrence rate for AQP4-ab disease (71.4%) was higher than that of MOG-ab disease (30.1%). For 135 children with both MOG-ab and AQP4-ab tested, the median age at onset was 7 (interquartile range [IQR] 5\u201310) years, and the median follow-up period was 19 (IQR 13\u201327.5) months. MOG-ab-positive children more frequently presented with acute disseminated encephalomyelitis, had deep gray matter lesions on MRI, had a better clinical and radiological recovery, and were less likely to have sustained disability than AQP4-ab-positive children. In MOG-ab-positive and AQP4-ab-positive children, maintenance therapy was a protective factor for recurrence, but presenting optic neuritis was a predictor of earlier relapse. A high Expanded Disability Status Scale score at onset was associated with sustained disability. Steroid maintenance therapy longer than 6 months after the initial attack was associated with a lower risk of a second relapse in MOG-ab-positive children. On serial serum MOG antibody analysis, clinical relapse occurred in 34.6% of children with persistent seropositivity, but none of the children who converted to seronegative status experienced relapse.<\/jats:p><jats:p><jats:bold>Conclusion:<\/jats:bold> The MOG antibody is more common in children with ADS than the AQP4 antibody. MOG-ab-positive children are characterized by distinct clinical and radiological features. Although some MOG-ab-positive children experience relapsing courses or have persistently seropositive status, they still predict a better outcome than AQP4-ab-positive children.<\/jats:p>","DOI":"10.3389\/fneur.2021.642664","type":"journal-article","created":{"date-parts":[[2021,3,26]],"date-time":"2021-03-26T06:00:43Z","timestamp":1616738443000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":10,"title":["Clinical and Prognostic Analysis of Autoantibody-Associated CNS Demyelinating Disorders in Children in Southwest China"],"prefix":"10.3389","volume":"12","author":[{"given":"Ziyan","family":"Li","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong","family":"Sun","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiao","family":"Fan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ping","family":"Yuan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yan","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peng","family":"Wu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Min","family":"Zhong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiannan","family":"Ma","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li","family":"Jiang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiujuan","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2021,3,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"S67","DOI":"10.1212\/wnl.0000000000002881","article-title":"Pediatric acquired CNS demyelinating syndromes: Features associated with multiple sclerosis","volume":"87","author":"Hintzen","year":"2016","journal-title":"Neurology."},{"key":"B2","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1016\/s1474-4422(17)30470-2","article-title":"Diagnosis of multiple sclerosis: 2017 revisions of the McDonald criteria","volume":"17","author":"Thompson","year":"2018","journal-title":"Lancet Neurol."},{"key":"B3","doi-asserted-by":"publisher","first-page":"2106","DOI":"10.1016\/s0140-6736(04)17551-x","article-title":"A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis","volume":"364","author":"Lennon","year":"2004","journal-title":"Lancet."},{"key":"B4","doi-asserted-by":"publisher","first-page":"8985","DOI":"10.1074\/jbc.M413174200","article-title":"Signaling cascades activated upon antibody cross-linking of myelin oligodendrocyte glycoprotein: potential implications for multiple sclerosis","volume":"280","author":"Marta","year":"2005","journal-title":"J Biol Chem."},{"key":"B5","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1046\/j.1471-4159.1999.0720001.x","article-title":"The structure and function of myelin oligodendrocyte glycoprotein","volume":"72","author":"Johns","year":"1999","journal-title":"J Neurochem."},{"key":"B6","doi-asserted-by":"publisher","first-page":"269","DOI":"10.1212\/wnl.0000000000004117","article-title":"Diagnostic algorithm for relapsing acquired demyelinating syndromes in children","volume":"89","author":"Hacohen","year":"2017","journal-title":"Neurology."},{"key":"B7","doi-asserted-by":"publisher","first-page":"845","DOI":"10.1007\/s00415-018-8781-3","article-title":"MRI of the first event in pediatric acquired demyelinating syndromes with antibodies to myelin oligodendrocyte glycoprotein","volume":"265","author":"Baumann","year":"2018","journal-title":"J Neurol."},{"key":"B8","doi-asserted-by":"publisher","first-page":"1355","DOI":"10.1001\/jamaneurol.2018.1814","article-title":"Association of MOG-IgG serostatus with relapse after acute disseminated encephalomyelitis and proposed diagnostic criteria for MOG-IgG-associated disorders","volume":"75","author":"L\u00f3pez-Chiriboga","year":"2018","journal-title":"JAMA Neurol."},{"key":"B9","doi-asserted-by":"publisher","first-page":"e322","DOI":"10.1212\/nxi.0000000000000322","article-title":"MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy","volume":"4","author":"Ogawa","year":"2017","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B10","doi-asserted-by":"publisher","first-page":"476960","DOI":"10.1016\/j.jneuroim.2019.05.001","article-title":"MOG antibody seropositive aseptic meningitis: A new clinical phenotype","volume":"333","author":"Nagabushana","year":"2019","journal-title":"J Neuroimmunol."},{"key":"B11","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1177\/1352458513484547","article-title":"International Pediatric Multiple Sclerosis Study Group criteria for pediatric multiple sclerosis and immune-mediated central nervous system demyelinating disorders: revisions to the 2007 definitions","volume":"19","author":"Krupp","year":"2013","journal-title":"Mult Scler."},{"key":"B12","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1093\/cid\/cit458","article-title":"Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium","volume":"57","author":"Venkatesan","year":"2013","journal-title":"Clin Infect Dis."},{"key":"B13","doi-asserted-by":"publisher","first-page":"177","DOI":"10.1212\/wnl.0000000000001729","article-title":"International consensus diagnostic criteria for neuromyelitis optica spectrum disorders","volume":"85","author":"Wingerchuk","year":"2015","journal-title":"Neurology."},{"key":"B14","doi-asserted-by":"publisher","first-page":"265","DOI":"10.1136\/jnnp-2014-308346","article-title":"Clinical and neuroradiological differences of paediatric acute disseminating encephalomyelitis with and without antibodies to the myelin oligodendrocyte glycoprotein","volume":"86","author":"Baumann","year":"2015","journal-title":"J Neurol Neurosurg Psychiatry."},{"key":"B15","doi-asserted-by":"publisher","first-page":"191","DOI":"10.1016\/s2352-4642(18)30026-9","article-title":"MRI and laboratory features and the performance of international criteria in the diagnosis of multiple sclerosis in children and adolescents: a prospective cohort study","volume":"2","author":"Fadda","year":"2018","journal-title":"Lancet Child Adolesc Health."},{"key":"B16","doi-asserted-by":"publisher","first-page":"234","DOI":"10.1016\/s1474-4422(19)30488-0","article-title":"Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study","volume":"19","author":"Armangue","year":"2020","journal-title":"Lancet Neurol."},{"key":"B17","doi-asserted-by":"publisher","first-page":"e81","DOI":"10.1212\/nxi.0000000000000081","article-title":"Myelin oligodendrocyte glycoprotein antibodies are associated with a non-MS course in children","volume":"2","author":"Hacohen","year":"2015","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B18","doi-asserted-by":"publisher","first-page":"900","DOI":"10.1212\/wnl.0000000000004312","article-title":"Prognostic relevance of MOG antibodies in children with an acquired demyelinating syndrome","volume":"89","author":"Hennes","year":"2017","journal-title":"Neurology."},{"key":"B19","doi-asserted-by":"publisher","first-page":"279","DOI":"10.1186\/s12974-016-0717-1","article-title":"MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 1: Frequency, syndrome specificity, influence of disease activity, long-term course, association with AQP4-IgG, and origin","volume":"13","author":"Jarius","year":"2016","journal-title":"J Neuroinflammation."},{"key":"B20","doi-asserted-by":"publisher","first-page":"3128","DOI":"10.1093\/brain\/awx276","article-title":"Clinical presentation and prognosis in MOG-antibody disease: a UK study","volume":"140","author":"Jurynczyk","year":"2017","journal-title":"Brain."},{"key":"B21","doi-asserted-by":"publisher","first-page":"e1858","DOI":"10.1212\/wnl.0000000000005560","article-title":"Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study","volume":"90","author":"Cobo-Calvo","year":"2018","journal-title":"Neurology."},{"key":"B22","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1111\/ene.13791","article-title":"Myelin oligodendrocyte glycoprotein antibody-associated demyelination: comparison between onset phenotypes","volume":"26","author":"Zhou","year":"2019","journal-title":"Eur J Neurol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1136\/jnnp-2017-316880","article-title":"Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination","volume":"89","author":"Ramanathan","year":"2018","journal-title":"J Neurol Neurosurg Psychiatry."},{"key":"B24","doi-asserted-by":"publisher","first-page":"e731","DOI":"10.1212\/nxi.0000000000000731","article-title":"Clinical and imaging features of children with autoimmune encephalitis and MOG antibodies","volume":"7","author":"Wegener-Panzer","year":"2020","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B25","doi-asserted-by":"publisher","first-page":"e1867","DOI":"10.1212\/wnl.0000000000008479","article-title":"Relevance of antibodies to myelin oligodendrocyte glycoprotein in CSF of seronegative cases","volume":"93","author":"Mariotto","year":"2019","journal-title":"Neurology."},{"key":"B26","doi-asserted-by":"publisher","first-page":"1854","DOI":"10.1177\/1352458519893093","article-title":"MRI differences between MOG antibody disease and AQP4 NMOSD","volume":"26","author":"Salama","year":"2020","journal-title":"Mult Scler."},{"key":"B27","doi-asserted-by":"publisher","first-page":"301","DOI":"10.1001\/jamaneurol.2018.4053","article-title":"Clinical, radiologic, and prognostic features of myelitis associated with myelin oligodendrocyte glycoprotein autoantibody","volume":"76","author":"Dubey","year":"2019","journal-title":"JAMA Neurol."},{"key":"B28","doi-asserted-by":"publisher","first-page":"2048","DOI":"10.1002\/acn3.50913","article-title":"Brain lesion distribution criteria distinguish demyelinating diseases in China","volume":"6","author":"Cai","year":"2019","journal-title":"Ann Clin Transl Neurol."},{"key":"B29","doi-asserted-by":"publisher","first-page":"617","DOI":"10.1093\/brain\/aww350","article-title":"Distinct brain imaging characteristics of autoantibody-mediated CNS conditions and multiple sclerosis","volume":"140","author":"Jurynczyk","year":"2017","journal-title":"Brain."},{"key":"B30","doi-asserted-by":"publisher","first-page":"390","DOI":"10.1111\/dmcn.14336","article-title":"Rituximab in children with myelin oligodendrocyte glycoprotein antibody and relapsing neuroinflammatory disease","volume":"62","author":"Albassam","year":"2020","journal-title":"Dev Med Child Neurol."},{"key":"B31","doi-asserted-by":"publisher","first-page":"478","DOI":"10.1001\/jamaneurol.2017.4601","article-title":"Disease course and treatment responses in children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease","volume":"75","author":"Hacohen","year":"2018","journal-title":"JAMA Neurol."},{"key":"B32","doi-asserted-by":"publisher","first-page":"e705","DOI":"10.1212\/nxi.0000000000000705","article-title":"Long-term efficacy of mycophenolate mofetil in myelin oligodendrocyte glycoprotein antibody-associated disorders: A prospective study","volume":"7","author":"Li","year":"2020","journal-title":"Neurol Neuroimmunol Neuroinflamm."},{"key":"B33","doi-asserted-by":"publisher","first-page":"1310","DOI":"10.1093\/brain\/awz054","article-title":"Outcome prediction models in AQP4-IgG positive neuromyelitis optica spectrum disorders","volume":"142","author":"Palace","year":"2019","journal-title":"Brain."},{"key":"B34","doi-asserted-by":"publisher","first-page":"1628","DOI":"10.1016\/j.ophtha.2018.03.041","article-title":"Aquaporin-4 and myelin oligodendrocyte glycoprotein autoantibody status predict outcome of recurrent optic neuritis","volume":"125","author":"Jitprapaikulsan","year":"2018","journal-title":"Ophthalmology."},{"key":"B35","doi-asserted-by":"publisher","first-page":"811","DOI":"10.1136\/jnnp-2017-315998","article-title":"Longitudinal analysis of myelin oligodendrocyte glycoprotein antibodies in CNS inflammatory diseases","volume":"88","author":"Hyun","year":"2017","journal-title":"J Neurol Neurosurg Psychiatry."},{"key":"B36","doi-asserted-by":"publisher","first-page":"958","DOI":"10.1111\/dmcn.13703","article-title":"Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes","volume":"60","author":"Duignan","year":"2018","journal-title":"Dev Med Child Neurol."},{"key":"B37","doi-asserted-by":"publisher","first-page":"82","DOI":"10.1001\/jamaneurol.2019.2940","article-title":"Serial anti-myelin oligodendrocyte glycoprotein antibody analyses and outcomes in children with demyelinating syndromes","volume":"77","author":"Waters","year":"2020","journal-title":"JAMA Neurol."},{"key":"B38","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/j.ejpn.2020.10.005","article-title":"EU paediatric MOG consortium consensus: Part 5 - Treatment of paediatric myelin oligodendrocyte glycoprotein antibody-associated disorders","volume":"29","author":"Bruijstens","year":"2020","journal-title":"Eur J Paediatr Neurol."}],"container-title":["Frontiers in Neurology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2021.642664\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,3,26]],"date-time":"2021-03-26T06:00:45Z","timestamp":1616738445000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fneur.2021.642664\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,26]]},"references-count":38,"alternative-id":["10.3389\/fneur.2021.642664"],"URL":"https:\/\/doi.org\/10.3389\/fneur.2021.642664","relation":{},"ISSN":["1664-2295"],"issn-type":[{"value":"1664-2295","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,26]]},"article-number":"642664"}}