Work

Phenotype of Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children

Year: 2024

Type: article

Abstract: Background and Purpose To determine the clinical phenotypes, relapse timing, treatment responses, and outcomes of children with relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). MethodsWe collected the demographic, clinical, laboratory, and radiological data of patients aged <18 years who had been diagnosed with MOGAD at Seoul National University Children's Hospital between January 2010 and January 2022; 100 were identified as positive for MOG antibodies, 43 of whom experienced relapse. ResultsThe median age at onset was 7 years (range 2-16 years).The median number of relapses was 2 (range 1-8), and patients were followed up for a median of 65 months (range 5-214 months).The first relapse was experienced before 3 months from onset by 15 patients (34.9%).The most-common initial phenotypes were acute disseminated encephalomyelitis (n=17, 39.5%) and optic neuritis (ON; n=11, 25.6%).The most-common relapse phenotypes were neuromyelitis optica spectrum disorder (n=9, 20.9%), relapsing ON (n=6, 14.0%), and multiphasic disseminated encephalomyelitis (n=6, 14.0%).Many of the patients (n=18, 41.9%) were not specifically categorized.A high proportion of these patients had non-acute disseminated encephalomyelitis encephalitis.Atypical phenotypes such as prolonged fever or hemiplegic migraine-like episodes were also noted.Mycophenolate mofetil and cyclic immunoglobulin treatment significantly reduced the annual relapse rates. ConclusionsOur 43 pediatric patients with relapsing MOGAD showed a tendency toward early relapse and various relapse phenotypes.The overall prognoses of these patients were good regardless of phenotype or response to second-line immunosuppressant treatment.

Source: Journal of Clinical Neurology

DOI: 10.3988/jcn.2024.0276