Acute Disseminated Encephalomyelitis in Children and Adolescents: A Multicenter Retrospective Study of Relapse and Outcome

Abstract

ObjectivesTo evaluate the demographic, clinical, laboratory, and prognostic data of children with acute disseminated encephalomyelitis with respect to anti-myelin oligodendrocyte glycoprotein (MOG) antibody status.MethodsAcute disseminated encephalomyelitis patients (n = 245) from 24 centers followed up between 2010 and 2022 were evaluated retrospectively. The short- and long-term outcome characteristics (disease severity and course, clinical relapse, and recovery rates) were assessed. Incomplete clinical recovery was defined as modified Rankin Score ≥1 or the presence of epilepsy. Univariant and multivariant analysis were performed for outcome characteristics.ResultsThe mean age at diagnosis was 6.3 ± 3.8 (0.5-17.7) years and the median follow-up was 22 (3-132) months. The outcome characteristics were evaluated in 180 of 245 patients (73.4%) with at least 12 months' follow-up. Twenty-three patients (12.6%) relapsed. The multivariable logistic regression analysis revealed the following clinical parameters as predictors of relapse: sex, visual impairment, and ataxia at initial presentation. Incomplete clinical recovery (n = 42/180, 23.3%) was associated with the presence of seizures on admission and the need for an intensive care unit. Anti-MOG antibody positivity was not associated with an increased risk of relapse (25% vs 13.1%, P = .164) or incomplete clinical recovery (P = .511).ConclusionThe nationwide cohort presented further supports the typically monophasic nature of acute disseminated encephalomyelitis, and a high rate of complete recovery. The presence of certain symptoms in the acute period may assist the clinician in estimating the outcome.

Keywords: autoimmune; encephalitis; neuroimmunology; outcome.