Christian Lechner1, Matthias Baumann1, Eva-Maria Hennes2, Kathrin Schanda3, Klaus Marquard2, Michael Karenfort4, Steffen Leiz5, Daniela Pohl6, Sunita Venkateswaran6, Martin Pritsch7, Johannes Koch8, Mareike Schimmel9, Martin Häusler10, Andrea Klein11, Astrid Blaschek12, Charlotte Thiels13, Thomas Lücke13, Ursula Gruber-Sedlmayr14, Barbara Kornek15, Andreas Hahn16, Frank Leypoldt17, Torsten Sandrieser18, Helge Gallwitz19, Johannes Stoffels20, Christoph Korenke21, Markus Reindl3, Kevin Rostásy22. 1. Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Innsbruck, Austria. 2. Department of Pediatric Neurology, Olgahospital Stuttgart, Stuttgart, Germany. 3. Clinical Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria. 4. Department of Pediatric Neurology, University Children's Hospital Dusseldorf, Dusseldorf, Germany. 5. Department of Pediatric Neurology, Children's Hospital Dritter Orden, Munich, Germany. 6. Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, Canada. 7. Department of Pediatric Neurology, DRK Children's Hospital Siegen, Siegen, Germany. 8. Department of Pediatrics, Paracelsus Medical University Salzburg, Salzburg, Austria. 9. Department of Pediatric Neurology, Children's Hospital Augsburg, Augsburg, Germany. 10. Division of Neuropediatrics and Social Pediatrics, University Hospital, RWTH Aachen, Aachen, Germany. 11. Department of Pediatric Neurology, University Children's Hospital Zurich, Zurich, Switzerland. 12. Department of Pediatric Neurology and Developmental Medicine, Dr von Hauner's Children's Hospital, Ludwig Maximilian University of Munich, Munich, Germany. 13. Department of Pediatric Neurology with Social Pediatrics, Children's Hospital, Ruhr University Bochum, Bochum, Germany. 14. Department of Pediatrics, Medical University of Graz, Graz, Austria. 15. Department of Neurology, Medical University of Vienna, Vienna, Austria. 16. Department of Pediatric Neurology, University Children's Hospital Giessen, Giessen, Germany. 17. Department of Neuroimmunology, Institute of Clinical Chemistry, Kiel, Germany Department of Neurology, University Hospital Schleswig-Holstein Campus Kiel, Kiel, Germany. 18. Department of Pediatric Neurology, Children's Hospital Koblenz, Koblenz, Germany. 19. Department of Pediatric Neurology, Children's Hospital Memmingen, Memmingen, Germany. 20. Department of Pediatric Neurology, Children's Hospital Neuburg, Neuburg, Germany. 21. Department of Pediatric Neurology, University Children's Hospital Oldenburg, Oldenburg, Germany. 22. Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Datteln, Germany.
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
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. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/
Authors: Yael Hacohen; Yu Yi Wong; Christian Lechner; Maciej Jurynczyk; Sukhvir Wright; Bahadir Konuskan; Judith Kalser; Anne Lise Poulat; Helene Maurey; Esther Ganelin-Cohen; Evangeline Wassmer; Chery Hemingway; Rob Forsyth; Eva Maria Hennes; M Isabel Leite; Olga Ciccarelli; Banu Anlar; Rogier Hintzen; Romain Marignier; Jacqueline Palace; Matthias Baumann; Kevin Rostásy; Rinze Neuteboom; Kumaran Deiva; Ming Lim Journal: JAMA Neurol Date: 2018-04-01 Impact factor: 18.302
Authors: Matthias Baumann; Astrid Grams; Tanja Djurdjevic; Eva-Maria Wendel; Christian Lechner; Bettina Behring; Astrid Blaschek; Katharina Diepold; Astrid Eisenkölbl; Joel Fluss; Michael Karenfort; Johannes Koch; Bahadir Konuşkan; Steffen Leiz; Andreas Merkenschlager; Daniela Pohl; Mareike Schimmel; Charlotte Thiels; Barbara Kornek; Kathrin Schanda; Markus Reindl; Kevin Rostásy Journal: J Neurol Date: 2018-02-08 Impact factor: 4.849