title: Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica.
abstract: BACKGROUND
  Recently we showed that antibodies to myelin oligodendrocyte glycoprotein (MOG) can be found in aquaporin-4 (AQP4)-immunoglobulin (IgG) seronegative pediatric and adult patients with definite and high-risk neuromyelitis optica (NMO).
  
  
  OBJECTIVE
  The purpose of this study was to describe the clinical characteristics and temporal dynamics of MOG-IgG in AQP4-IgG seronegative pediatric patients presenting with definite NMO.
  
  
  METHODS
  Children with definite NMO who were referred for further testing of serum antibodies for AQP4 and MOG with a cell-based assay were included in this study. Clinical disease course, cerebrospinal fluid and magnetic resonance imaging (MRI) studies of these patients were reviewed.
  
  
  RESULTS
  Between 2008 and 2012 eight children who fulfilled the diagnostic criteria of definite NMO were recruited. Two children with definite NMO tested positive for AQP4-IgG but were negative for MOG-IgG antibodies. Three children had an absence of AQP4-IgG and MOG-IgG antibodies. Three children with definite NMO had high titers of serum MOG-IgG antibodies (≥1: 160), but no AQP4-directed humoral immune response. Longitudinal analysis of serum samples of the latter three children showed persisting high MOG-IgG titers over time.
  
  
  CONCLUSIONS
  Pediatric patients presenting with clinical symptoms and MRI findings highly suggestive of NMO but with high and persisting MOG-IgG antibody titers are most likely to represent a distinct subgroup of acute demyelinating diseases with important clinical and therapeutic implications.
authors.affiliation: Department of Pediatrics I, Innsbruck Medical University, Austria. Kevin.Rostasy@uki.at
authors.forename: K
authors.initials: K
authors.lastname: Rostásy
authors.forename: S
authors.initials: S
authors.lastname: Mader
authors.forename: E M
authors.initials: EM
authors.lastname: Hennes
authors.forename: K
authors.initials: K
authors.lastname: Schanda
authors.forename: V
authors.initials: V
authors.lastname: Gredler
authors.forename: A
authors.initials: A
authors.lastname: Guenther
authors.forename: A
authors.initials: A
authors.lastname: Blaschek
authors.forename: C
authors.initials: C
authors.lastname: Korenke
authors.forename: M
authors.initials: M
authors.lastname: Pritsch
authors.forename: D
authors.initials: D
authors.lastname: Pohl
authors.forename: O
authors.initials: O
authors.lastname: Maier
authors.forename: G
authors.initials: G
authors.lastname: Kuchukhidze
authors.forename: M
authors.initials: M
authors.lastname: Brunner-Krainz
authors.forename: T
authors.initials: T
authors.lastname: Berger
authors.forename: M
authors.initials: M
authors.lastname: Reindl
mesh_terms: D000293:Adolescent; D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D002648:Child; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000276:immunology*; D009471:Neuromyelitis Optica / Q000097:blood* / Q000276:immunology*
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D001323:Autoantibodies; D001324:Autoantigens; D007074:Immunoglobulin G; D063308:Myelin-Oligodendrocyte Glycoprotein