title: Antibodies to MOG and AQP4 in children with neuromyelitis optica and limited forms of the disease.
abstract: OBJECTIVE
  To determine the frequency and clinical-radiological associations of antibodies to myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) in children presenting with neuromyelitis optica (NMO) and limited forms.
  
  
  METHODS
  Children with a first event of NMO, recurrent (RON), bilateral ON (BON), longitudinally extensive transverse myelitis (LETM) or brainstem syndrome (BS) with a clinical follow-up of more than 12 months were enrolled. Serum samples were tested for MOG- and AQP4-antibodies using live cell-based assays.
  
  
  RESULTS
  45 children with NMO (n=12), LETM (n=14), BON (n=6), RON (n=12) and BS (n=1) were included. 25/45 (56%) children had MOG-antibodies at initial presentation (7 NMO, 4 BON, 8 ON, 6 LETM). 5/45 (11%) children showed AQP4-antibodies (3 NMO, 1 LETM, 1 BS) and 15/45 (33%) were seronegative for both antibodies (2 NMO, 2 BON, 4 RON, 7 LETM). No differences were found in the age at presentation, sex ratio, frequency of oligoclonal bands or median EDSS at last follow-up between the three groups. Children with MOG-antibodies more frequently (1) had a monophasic course (p=0.018) after one year, (2) presented with simultaneous ON and LETM (p=0.004) and (3) were less likely to receive immunosuppressive therapies (p=0.0002). MRI in MOG-antibody positive patients (4) less frequently demonstrated periependymal lesions (p=0.001), (5) more often were unspecific (p=0.004) and (6) resolved more frequently (p=0.016).
  
  
  CONCLUSIONS
  67% of all children presenting with NMO or limited forms tested positive for MOG- or AQP4-antibodies. MOG-antibody positivity was associated with distinct features. We therefore recommend to measure both antibodies in children with demyelinating syndromes.
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria.
authors.forename: Christian
authors.initials: C
authors.lastname: Lechner
authors.affiliation: Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria.
authors.forename: Matthias
authors.initials: M
authors.lastname: Baumann
authors.affiliation: Department of Pediatric Neurology, Olgahospital Stuttgart, Stuttgart, Germany.
authors.forename: Eva-Maria
authors.initials: EM
authors.lastname: Hennes
authors.affiliation: Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors.forename: Kathrin
authors.initials: K
authors.lastname: Schanda
authors.affiliation: Department of Pediatric Neurology, Olgahospital Stuttgart, Stuttgart, Germany.
authors.forename: Klaus
authors.initials: K
authors.lastname: Marquard
authors.affiliation: Department of Pediatric Neurology, University Children's Hospital Dusseldorf, Dusseldorf, Germany.
authors.forename: Michael
authors.initials: M
authors.lastname: Karenfort
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Dritter Orden, Munich, Germany.
authors.forename: Steffen
authors.initials: S
authors.lastname: Leiz
authors.affiliation: Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, Canada.
authors.forename: Daniela
authors.initials: D
authors.lastname: Pohl
authors.affiliation: Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, Canada.
authors.forename: Sunita
authors.initials: S
authors.lastname: Venkateswaran
authors.affiliation: Department of Pediatric Neurology, DRK Children's Hospital Siegen, Siegen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Pritsch
authors.affiliation: Department of Pediatrics, Paracelsus Medical University Salzburg, Salzburg, Austria.
authors.forename: Johannes
authors.initials: J
authors.lastname: Koch
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Augsburg, Augsburg, Germany.
authors.forename: Mareike
authors.initials: M
authors.lastname: Schimmel
authors.affiliation: Division of Neuropediatrics and Social Pediatrics, University Hospital, RWTH Aachen, Aachen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Häusler
authors.affiliation: Department of Pediatric Neurology, University Children's Hospital Zurich, Zurich, Switzerland.
authors.forename: Andrea
authors.initials: A
authors.lastname: Klein
authors.affiliation: Department of Pediatric Neurology and Developmental Medicine, Dr von Hauner's Children's Hospital, Ludwig Maximilian University of Munich, Munich, Germany.
authors.forename: Astrid
authors.initials: A
authors.lastname: Blaschek
authors.affiliation: Department of Pediatric Neurology with Social Pediatrics, Children's Hospital, Ruhr University Bochum, Bochum, Germany.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Thiels
authors.affiliation: Department of Pediatric Neurology with Social Pediatrics, Children's Hospital, Ruhr University Bochum, Bochum, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Lücke
authors.affiliation: Department of Pediatrics, Medical University of Graz, Graz, Austria.
authors.forename: Ursula
authors.initials: U
authors.lastname: Gruber-Sedlmayr
authors.affiliation: Department of Neurology, Medical University of Vienna, Vienna, Austria.
authors.forename: Barbara
authors.initials: B
authors.lastname: Kornek
authors.affiliation: Department of Pediatric Neurology, University Children's Hospital Giessen, Giessen, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Hahn
authors.affiliation: Department of Neuroimmunology, Institute of Clinical Chemistry, Kiel, Germany Department of Neurology, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany.
authors.forename: Frank
authors.initials: F
authors.lastname: Leypoldt
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Koblenz, Koblenz, Germany.
authors.forename: Torsten
authors.initials: T
authors.lastname: Sandrieser
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Memmingen, Memmingen, Germany.
authors.forename: Helge
authors.initials: H
authors.lastname: Gallwitz
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Neuburg, Neuburg, Germany.
authors.forename: Johannes
authors.initials: J
authors.lastname: Stoffels
authors.affiliation: Department of Pediatric Neurology, University Children's Hospital Oldenburg, Oldenburg, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Korenke
authors.affiliation: Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.
authors.forename: Markus
authors.initials: M
authors.lastname: Reindl
authors.affiliation: Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany.
authors.forename: Kevin
authors.initials: K
authors.lastname: Rostásy
mesh_terms: D000293:Adolescent; D051401:Aquaporin 4 / Q000097:blood / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007964:Leukocytosis / Q000134:cerebrospinal fluid; D008279:Magnetic Resonance Imaging; D008297:Male; D063308:Myelin-Oligodendrocyte Glycoprotein / Q000097:blood / Q000276:immunology*; D009188:Myelitis, Transverse / Q000097:blood / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000276:immunology*; D059906:Neuroimaging; D009471:Neuromyelitis Optica / Q000097:blood / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000276:immunology*; D043025:Oligoclonal Bands / Q000134:cerebrospinal fluid; D012307:Risk Factors; D013577:Syndrome
chemical_list: D051401:Aquaporin 4; D001323:Autoantibodies; D063308:Myelin-Oligodendrocyte Glycoprotein; D043025:Oligoclonal Bands