{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T22:15:04Z","timestamp":1787004904127,"version":"build-2736575974"},"reference-count":7,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neuropediatrics"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Acquired demyelinating syndromes (ADS) are frequently associated with myelin oligodendrocytes glycoprotein (MOG) antibodies in children. Clinical phenotypes are heterogeneous and may delay the diagnosis, especially when they relapse and are atypical, mimicking diseases such as multiple sclerosis or neuromyelitis optica spectrum disorders . Here, we describe two children: one with a progressive cognitive and behavioral deterioration with seizures after only one relapse and the other with similar clinical impairments associated with multiple relapses. Brain magnetic resonance imaging revealed a subsequent progressive leukodystrophy-like lesion with diffuse bilateral white matter injuries in both patients. Cerebrospinal fluid analysis showed pleiocytosis, increased level of proteins with no oligoclonal bands. Metabolic and inflammatory blood markers were all negative. Brain biopsy was performed in the second child and nonspecific inflammatory lesions with no argument for histiocytosis or tumor were observed. Clinical and radiological stabilization were obtained after active immunotherapy. Retrospective analysis of anti-MOG antibodies in these two children was positive at the earlier stage of the disease and turned negative after treatment and during follow-up. Leukodystrophy-like ADS with anti-MOG-antibodies may display distinct progressive phenotype and have a severe neurological prognosis. Early diagnosis and appropriate treatment may improve outcome in these children.<\/jats:p>","DOI":"10.1055\/s-0041-1726289","type":"journal-article","created":{"date-parts":[[2021,4,1]],"date-time":"2021-04-01T02:01:46Z","timestamp":1617242506000},"page":"337-340","source":"Crossref","is-referenced-by-count":13,"title":["Progressive Leukodystrophy-Like Demyelinating Syndromes with MOG-Antibodies in Children: A Rare Under-Recognized Phenotype"],"prefix":"10.1055","volume":"52","author":[{"given":"Elise","family":"Yazbeck","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department, Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pitaux Universitaires Paris Saclay, Bic\u00eatre Hospital, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e8ne","family":"Maurey","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department, Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pitaux Universitaires Paris Saclay, Bic\u00eatre Hospital, Le Kremlin Bic\u00eatre, France"},{"name":"National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carole","family":"Leroy","sequence":"additional","affiliation":[{"name":"National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bic\u00eatre, France"},{"name":"Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe","family":"Horellou","sequence":"additional","affiliation":[{"name":"Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvia","family":"Napuri","sequence":"additional","affiliation":[{"name":"Pediatric Department, Centre Hospitalo-Universitaire de Rennes - H\u00f4pital Sud, Rennes, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohammed","family":"Lali","sequence":"additional","affiliation":[{"name":"Pediatric Department, Centre hospitalier Bretagne Atlantique, Vannes, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clovis","family":"Adam","sequence":"additional","affiliation":[{"name":"Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pitaux Universitaires Paris-Saclay, Neuropathology Laboratory, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beatrice","family":"Husson","sequence":"additional","affiliation":[{"name":"Pediatric Radiology Department, Assistance Publique-H\u00f4pitaux de Paris, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Caroline","family":"Sevin","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department, Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pitaux Universitaires Paris Saclay, Bic\u00eatre Hospital, Le Kremlin Bic\u00eatre, France"},{"name":"National Referral Center for Leucodystrophy, Le Kremlin-Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kumaran","family":"Deiva","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department, Assistance Publique-H\u00f4pitaux de Paris, H\u00f4pitaux Universitaires Paris Saclay, Bic\u00eatre Hospital, Le Kremlin Bic\u00eatre, France"},{"name":"National Referral Center for Rare Inflammatory and Auto-Immune Brain and Spinal Diseases, Le Kremlin-Bic\u00eatre, France"},{"name":"Inserm UMR 1184, Immunology of Viral Infections and Autoimmune Diseases, CEA, IDMIT, Le Kremlin Bic\u00eatre, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2021,3,31]]},"reference":[{"issue":"09","key":"ref1","doi-asserted-by":"crossref","first-page":"1075","DOI":"10.1111\/dmcn.14594","article-title":"Risk factors for academic difficulties in children with myelin oligodendrocyte glycoprotein antibody-associated acute demyelinating syndromes","volume":"62","author":"K Deiva","year":"2020","journal-title":"Dev Med Child Neurol"},{"issue":"02","key":"ref2","doi-asserted-by":"crossref","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":"Y Hacohen","year":"2015","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"issue":"09","key":"ref3","doi-asserted-by":"crossref","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":"E M Hennes","year":"2017","journal-title":"Neurology"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jneuroim.2015.10.002","article-title":"Increased interleukin-6 correlates with myelin oligodendrocyte glycoprotein antibodies in pediatric monophasic demyelinating diseases and multiple sclerosis","volume":"289","author":"P Horellou","year":"2015","journal-title":"J Neuroimmunol"},{"issue":"04","key":"ref5","doi-asserted-by":"crossref","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":"Y Hacohen","year":"2018","journal-title":"JAMA Neurol"},{"issue":"04","key":"ref6","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1111\/dmcn.13649","article-title":"\u2018Leukodystrophy-like\u2019 phenotype in children with myelin oligodendrocyte glycoprotein antibody-associated disease","volume":"60","author":"Y Hacohen","year":"2018","journal-title":"Dev Med Child Neurol"},{"issue":"10","key":"ref7","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1016\/S1474-4422(19)30143-7","article-title":"Diagnosis, prognosis, and treatment of leukodystrophies","volume":"18","author":"M S van der Knaap","year":"2019","journal-title":"Lancet Neurol"}],"container-title":["Neuropediatrics"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/s-0041-1726289.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,7,14]],"date-time":"2021-07-14T18:58:40Z","timestamp":1626289120000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/s-0041-1726289"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,3,31]]},"references-count":7,"journal-issue":{"issue":"04","published-online":{"date-parts":[[2021,7,14]]},"published-print":{"date-parts":[[2021,8]]}},"URL":"https:\/\/doi.org\/10.1055\/s-0041-1726289","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0174-304X","1439-1899"],"issn-type":[{"value":"0174-304X","type":"print"},{"value":"1439-1899","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,3,31]]}}}