Eva-Maria Wendel1, Matthias Baumann2, Nina Barisic3, Astrid Blaschek4, Eliana Coelho de Oliveira Koch5, Adela Della Marina6, Katharina Diepold7, Annette Hackenberg8, Andreas Hahn9, Thekla von Kalle10, Michael Karenfort11, Barbara Kornek12, Christian Lechner2, Steffen Leiz13, Andreas Merkenschlager14, Margherita Nosadini15, Stefano Sartori15, Kathrin Schanda16, Mareike Schimmel17, Larissa Seemann18, Victoria Tüngler19, Stephan Waltz20, Andreas Wegener-Panzer21, Gert Wiegand22, Markus Reindl16, Kevin Rostásy23. 1. Department of Pediatrics, Olgahospital, Klinikum, Stuttgart, Germany. 2. Division of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Austria. 3. Department of Pediatrics, Clinical Medical Center Zagreb, University of Zagreb Medical School, Croatia. 4. Department of Pediatric Neurology, Dr. von Hauner Children's Hospital, Ludwig-Maximillian-Universität Munich, Germany. 5. Division of Pediatric Neurology, Department of Pediatrics, Medical University of Geneva, Switzerland. 6. Department of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital, University of Duisburg-Essen, Germany. 7. Division of Pediatric Neurology, Department of Pediatrics, Hospital Kassel, Germany. 8. Department of Pediatric Neurology, University Children's Hospital, Zürich, Switzerland. 9. Division of Pediatric Neurology, Department of Pediatrics, Medical University Giessen, Germany. 10. Department of Pediatric Radiology, Olgahospital, Klinikum Stuttgart, Germany. 11. Department of Pediatrics, Neonatology and Pediatric Cardiology, Children's Hospital, Heinrich-Heine-University, Düsseldorf, Germany. 12. Department of Neurology, Medical University Vienna, Austria. 13. Division of Pediatric Neurology, Department of Pediatrics, Klinikum Dritter Orden, Munich, Germany. 14. Division of Pediatric Neurology, Department of Pediatrics, Medical University of Leipzig, Germany. 15. Paediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Italy. 16. Clinical Department of Neurology, Medical University of Innsbruck, Austria. 17. Division of Pediatric Neurology, Children's Hospital, Medical University of Augsburg, Germany. 18. Department of Pediatric Neurology, Children's Hospital DRK Siegen, Germany. 19. Division of Pediatric Neurology, Department of Pediatrics, Medical University Carl Gustav Carus, Dresden, Germany. 20. Department of Pediatric Neurology, Children's Hospital Amsterdamer Straße, Cologne, Germany. 21. Department of Pediatric Radiology, Children's Hospital Datteln, University Witten/Herdecke, Germany. 22. Division of Pediatric Neurology, Department of Pediatrics, Asklepios Klinik Nord, Heidberg/Hamburg, Germany. 23. Department of Pediatric Neurology, Children's Hospital Datteln, University Witten/Herdecke, Germany. Electronic address: k.rostasy@kinderklinik-datteln.de.
Abstract
BACKGROUND: Bilateral optic neuritis (bilON) is a rare clinical presentation often thought to be associated with relapsing disorders such as neuromyelitis optica spectrum disorders (NMOSD) or multiple sclerosis (MS). OBJECTIVE: To characterize the clinical, radiological phenotype and antibody status of children presenting with bilON. MATERIAL AND METHODS: Retrospective multicenter study on children with bilON age <18 years with a first episode aquired demyelinating syndrome (ADS), cMRI, AQP4- and serum MOG-antibody status and follow-up data were collected. RESULTS: 30 patients (f:m = 15:15, median age 8.0y) with bilON met the inclusion criteria. 22/30 (73%) were MOG-positive (median: 1:1280, range: 1:160-1:1520). No patient showed AQP4-abs. 4/30 patients (13%), all with high MOG-abs titers, had recurrent episodes. No patient developed MS. Improvement after IVMP was observed in most patients (26/30; 87%). Outcome was favorable with no sequelae in 22/30 patients. Serial MOG-abs titers tested in 15/22 patients decreased to a median of 1:160 (range: 0-1:640) over a period of 31 months (range: 2-141 months) in 14/15 (93%) patients. MR imaging showed a predominantly anterior affection of the visual system in seropositive patients with bilateral intraorbital lesions in 68% (15/22), compared to 25% in MOG-negative patients (2/8). CONCLUSION: Pediatric bilON is associated with high MOG-abs titers in combination with anterior involvement of the visual system. Despite severe loss of vision, the majority of patients shows distinct recovery after IVMP.
BACKGROUND:Bilateral optic neuritis (bilON) is a rare clinical presentation often thought to be associated with relapsing disorders such as neuromyelitis optica spectrum disorders (NMOSD) or multiple sclerosis (MS). OBJECTIVE: To characterize the clinical, radiological phenotype and antibody status of children presenting with bilON. MATERIAL AND METHODS: Retrospective multicenter study on children with bilON age <18 years with a first episode aquired demyelinating syndrome (ADS), cMRI, AQP4- and serum MOG-antibody status and follow-up data were collected. RESULTS: 30 patients (f:m = 15:15, median age 8.0y) with bilON met the inclusion criteria. 22/30 (73%) were MOG-positive (median: 1:1280, range: 1:160-1:1520). No patient showed AQP4-abs. 4/30 patients (13%), all with high MOG-abs titers, had recurrent episodes. No patient developed MS. Improvement after IVMP was observed in most patients (26/30; 87%). Outcome was favorable with no sequelae in 22/30 patients. Serial MOG-abs titers tested in 15/22 patients decreased to a median of 1:160 (range: 0-1:640) over a period of 31 months (range: 2-141 months) in 14/15 (93%) patients. MR imaging showed a predominantly anterior affection of the visual system in seropositive patients with bilateral intraorbital lesions in 68% (15/22), compared to 25% in MOG-negative patients (2/8). CONCLUSION: Pediatric bilON is associated with high MOG-abs titers in combination with anterior involvement of the visual system. Despite severe loss of vision, the majority of patients shows distinct recovery after IVMP.
Authors: Eva Maria Wendel; Helen Sophie Thonke; Annikki Bertolini; Matthias Baumann; Astrid Blaschek; Andreas Merkenschlager; Michael Karenfort; Barbara Kornek; Christian Lechner; Daniela Pohl; Martin Pritsch; Kathrin Schanda; Mareike Schimmel; Charlotte Thiels; Stephan Waltz; Gert Wiegand; Banu Anlar; Nina Barisic; Christian Blank; Markus Breu; Philip Broser; Adela Della Marina; Katharina Diepold; Matthias Eckenweiler; Astrid Eisenkölbl; Michael Freilinger; Ursula Gruber-Sedlmayr; Annette Hackenberg; Tobias Iff; Ellen Knierim; Johannes Koch; Georg Kutschke; Steffen Leiz; Grischa Lischetzki; Margherita Nosadini; Alexander Pschibul; Edith Reiter-Fink; Doris Rohrbach; Michela Salandin; Stefano Sartori; Jan-Ulrich Schlump; Johannes Stoffels; Jurgis Strautmanis; Daniel Tibussek; Victoria Tüngler; Norbert Utzig; Markus Reindl; Kevin Rostásy Journal: Neurol Neuroimmunol Neuroinflamm Date: 2022-10-13
Authors: Joachim Havla; Thivya Pakeerathan; Kevin Rostasy; Ilya Ayzenberg; Carolin Schwake; Jeffrey L Bennett; Ingo Kleiter; Ana Felipe-Rucián; Stephanie C Joachim; Amelie S Lotz-Havla; Tania Kümpfel; Markus Krumbholz; Eva M Wendel; Markus Reindl; Charlotte Thiels; Thomas Lücke; Kerstin Hellwig; Ralf Gold Journal: J Neuroinflammation Date: 2021-05-29 Impact factor: 8.322