Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Abstract

Cerebral cortical encephalitis (CCE) is a recently described myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) phenotype. In this observational retrospective study, we characterized 19 CCE patients (6.7% of our MOGAD cohort). Headache (n=15, 79%), seizures (n=13, 68%), and encephalopathy (n=12, 63%) were frequent. Magnetic resonance imaging revealed unilateral (n=12, 63%) or bilateral (n=7, 37%) cortical T2 hyperintensity and leptomeningeal enhancement (n=17, 89%). N-Methyl-D-aspartate receptor autoantibodies coexisted in 2 of 15 tested (13%). CCE pathology (n=2) showed extensive subpial cortical demyelination (n=2), microglial reactivity (n=2), and inflammatory infiltrates (perivascular, n=1; meningeal, n=1). Most received high-dose steroids (n=17, 89%), and all improved, but 3 had CCE relapses. This study highlights the CCE spectrum and provides insight into its pathogenesis. ANN NEUROL 2023;93:297-302.