High association of MOG-IgG antibodies in children with bilateral optic neuritis. (July 2020)

• Record Type: Journal Article
• Authors: Wendel, Eva-Maria; Baumann, Matthias; Barisic, Nina; Blaschek, Astrid; Coelho de Oliveira Koch, Eliana; Della Marina, Adela; Diepold, Katharina; Hackenberg, Annette; Hahn, Andreas; von Kalle, Thekla; Karenfort, Michael; Kornek, Barbara; Lechner, Christian; Leiz, Steffen; Merkenschlager, Andreas; Nosadini, Margherita; Sartori, Stefano; Schanda, Kathrin; Schimmel, Mareike; Seemann, Larissa; Tüngler, Victoria; Waltz, Stephan; Wegener-Panzer, Andreas; Wiegand, Gert; Reindl, Markus; Rostásy, Kevin
• Is Part Of: European journal of paediatric neurology. Volume 27(2020)
• Page Start: 86
• Page End: 93
• Publication Date: 2020-07
• Subjects: Antibodies -- Autoimmune -- Encephalitis -- Myelin-oligodendrocyte-glycoprotein; Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2020.04.002
• Languages: English
• ISSNs: 1090-3798

Abstract

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.