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Research paper
Antibodies to MOG and AQP4 in children with neuromyelitis optica and limited forms of the disease
  1. Christian Lechner1,
  2. Matthias Baumann1,
  3. Eva-Maria Hennes2,
  4. Kathrin Schanda3,
  5. Klaus Marquard2,
  6. Michael Karenfort4,
  7. Steffen Leiz5,
  8. Daniela Pohl6,
  9. Sunita Venkateswaran6,
  10. Martin Pritsch7,
  11. Johannes Koch8,
  12. Mareike Schimmel9,
  13. Martin Häusler10,
  14. Andrea Klein11,
  15. Astrid Blaschek12,
  16. Charlotte Thiels13,
  17. Thomas Lücke13,
  18. Ursula Gruber-Sedlmayr14,
  19. Barbara Kornek15,
  20. Andreas Hahn16,
  21. Frank Leypoldt17,18,
  22. Torsten Sandrieser19,
  23. Helge Gallwitz20,
  24. Johannes Stoffels21,
  25. Christoph Korenke22,
  26. Markus Reindl3,
  27. Kevin Rostásy23
  1. 1Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria
  2. 2Department of Pediatric Neurology, Olgahospital Stuttgart, Stuttgart, Germany
  3. 3Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria
  4. 4Department of Pediatric Neurology, University Children's Hospital Dusseldorf, Dusseldorf, Germany
  5. 5Department of Pediatric Neurology, Children's Hospital Dritter Orden, Munich, Germany
  6. 6Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, Canada
  7. 7Department of Pediatric Neurology, DRK Children's Hospital Siegen, Siegen, Germany
  8. 8Department of Pediatrics, Paracelsus Medical University Salzburg, Salzburg, Austria
  9. 9Department of Pediatric Neurology, Children's Hospital Augsburg, Augsburg, Germany
  10. 10Division of Neuropediatrics and Social Pediatrics, University Hospital, RWTH Aachen, Aachen, Germany
  11. 11Department of Pediatric Neurology, University Children's Hospital Zurich, Zurich, Switzerland
  12. 12Department of Pediatric Neurology and Developmental Medicine, Dr von Hauner's Children's Hospital, Ludwig Maximilian University of Munich, Munich, Germany
  13. 13Department of Pediatric Neurology with Social Pediatrics, Children's Hospital, Ruhr University Bochum, Bochum, Germany
  14. 14Department of Pediatrics, Medical University of Graz, Graz, Austria
  15. 15Department of Neurology, Medical University of Vienna, Vienna, Austria
  16. 16Department of Pediatric Neurology, University Children's Hospital Giessen, Giessen, Germany
  17. 17Department of Neuroimmunology, Institute of Clinical Chemistry, Kiel, Germany
  18. 18Department of Neurology, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany
  19. 19Department of Pediatric Neurology, Children's Hospital Koblenz, Koblenz, Germany
  20. 20Department of Pediatric Neurology, Children's Hospital Memmingen, Memmingen, Germany
  21. 21Department of Pediatric Neurology, Children's Hospital Neuburg, Neuburg, Germany
  22. 22Department of Pediatric Neurology, University Children's Hospital Oldenburg, Oldenburg, Germany
  23. 23Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany
  1. Correspondence to Dr Kevin Rostásy, Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Dr Friedrich Steiner Str 5, Datteln 45711, Germany; k.rostasy{at}kinderklinik-datteln.de

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.

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