European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society · 2020
High association of MOG-IgG antibodies in children with bilateral optic neuritis.
СтатьяМногоцентровое исследованиеНаблюдательное исследованиеНазвание и аннотация: Перевод пока не подготовлен
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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
Аннотация
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.
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.