Autoantikūnų prieš mielino oligodendrocitų glikoproteiną sukeltų ligų ypatumai vaikams: ryšys su optiniu neuritu ir lyčių skirtumai

Abstract

Summary. Background and aim. Despite the increasing clinical data on anti-myelin oligodendrocyte glycoprotein (anti-MOG)-associated inflammatory demyelinating dis- eases, there are many un answered questions. This article aims to elu cidate the peculiarities of clinical presentation, association with optic neuritis (ON), relapsing course, investigations, treatment, and gender differences in pediatric anti-MOG-related disorders. Methods. The study involved 51 patients: 46 patients described in publications and 5 pa- tients treated in Vilnius University Hospital Santaros klinikos Pediatrics Centre between 2017 and 2020. Result. Acute disseminated encephalomyelitis (ADEM) was the most common (61%) clinical syndrome as the first presentation in anti-MOG-associated inflammatory demyelinating diseases in children. ON was present in 23.5% of patients at the beginning of the disease. 49% of chil dren had relapses. ON was present in 49% of patients during the course of the relapsing anti-MOG spectrum disease. There was a statistically significant dif- ference in relapse rate between the patients with and without ON during the course of illness – relapses occurred in 72% of patients with ON and 27% of patients without ON. We found a statistically significant gender difference – girls were more likely to relapse than boys, regard- less of the presence of ON. 76% of patients reached full recovery during the follow-up period. Conclusions. For the first time, the disease usually presents as acute disseminating encephalomyelitis. About half of children with myelin-oligodendrocyte glycoprotein spec- trum disease experience recurrence of the disease and ON. Girls are significantly more likely to relapse than boys. Children with ON have a higher risk of the disease recurrence