Neuroimaging spectrum of myelin oligodendrocyte glycoprotein antibody-associated disease with brain involvement: description of various cerebral syndromes.

Abstract

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a notable cause of acquired central nervous system inflammatory disorders in children.

Objective: This study aimed to characterize the neuroimaging spectrum of pediatric MOGAD with brain involvement.

Materials and methods: In this retrospective, single-center study, 55 children diagnosed with MOGAD involving the brain between January 2010 and October 2020 were included. Clinical data and neuroimaging-brain and spinal magnetic resonance imaging (MRI) at presentation-were reviewed. Imaging patterns were categorized into six radiologic phenotypes: acute disseminated encephalomyelitis (ADEM), cerebral cortical encephalitis, aseptic meningitis, tumefactive demyelinating lesion, cerebellitis/brainstem encephalitis, and miscellaneous. Imaging features were further analyzed in the ADEM subgroup.

Results: ADEM was the most common phenotype (39 of 55 patients, 71%), though atypical features were frequent, with 62% showing at least one atypical MRI finding. Unlike classic ADEM with large confluent white matter lesions, MOGAD-associated ADEM often showed small (31%) or subcortical (44%) white matter lesions. Spinal lesions typically appeared as longitudinally extensive myelitis with central gray matter involvement. Other phenotypes included cortical encephalitis (three patients), aseptic meningitis (six), tumefactive demyelinating lesions (three), cerebellitis/brainstem encephalitis (two), and two miscellaneous patterns. Non-ADEM phenotypes presented at an older age than ADEM (11.5 years vs. 5.2 years, P < 0.01), with a threshold of 7.6 years.

Conclusion: Pediatric MOGAD with brain involvement presents a range of imaging patterns. ADEM is most frequent but often displays atypical features. Non-ADEM phenotypes tend to occur in older children.