High association of MOG-IgG antibodies in children with bilateral optic neuritis.
Wendel EMDepartment of Pediatrics, Olgahospital, Klinikum, Stuttgart, Germany.
Baumann MDivision of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Austria.
Barisic NDepartment of Pediatrics, Clinical Medical Center Zagreb, University of Zagreb Medical School, Croatia.
Blaschek ADepartment of Pediatric Neurology, Dr. von Hauner Children's Hospital, Ludwig-Maximillian-Universität Munich, Germany.
Coelho de Oliveira Koch EDivision of Pediatric Neurology, Department of Pediatrics, Medical University of Geneva, Switzerland.
Della Marina ADepartment of Neuropediatrics, Developmental Neurology and Social Pediatrics, Children's Hospital, University of Duisburg-Essen, Germany.
Diepold KDivision of Pediatric Neurology, Department of Pediatrics, Hospital Kassel, Germany.
Hackenberg ADepartment of Pediatric Neurology, University Children's Hospital, Zürich, Switzerland.
Hahn ADivision of Pediatric Neurology, Department of Pediatrics, Medical University Giessen, Germany.
von Kalle TDepartment of Pediatric Radiology, Olgahospital, Klinikum Stuttgart, Germany.
Karenfort MDepartment of Pediatrics, Neonatology and Pediatric Cardiology, Children's Hospital, Heinrich-Heine-University, Düsseldorf, Germany.
Kornek BDepartment of Neurology, Medical University Vienna, Austria.
Lechner CDivision of Pediatric Neurology, Department of Pediatrics I, Medical University of Innsbruck, Austria.
Leiz SDivision of Pediatric Neurology, Department of Pediatrics, Klinikum Dritter Orden, Munich, Germany.
Merkenschlager ADivision of Pediatric Neurology, Department of Pediatrics, Medical University of Leipzig, Germany.
Nosadini MPaediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Italy.
Sartori SPaediatric Neurology and Neurophysiology Unit, Department of Women's and Children's Health, University Hospital of Padua, Italy.
Schanda KClinical Department of Neurology, Medical University of Innsbruck, Austria.
Schimmel MDivision of Pediatric Neurology, Children's Hospital, Medical University of Augsburg, Germany.
Seemann LDepartment of Pediatric Neurology, Children's Hospital DRK Siegen, Germany.
Tüngler VDivision of Pediatric Neurology, Department of Pediatrics, Medical University Carl Gustav Carus, Dresden, Germany.
English
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.