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. MRI revealed unilateral (n=12[63%]) or bilateral (n=7[37%]) cortical T2-hyperintensity and leptomeningeal-enhancement (n=17[89%]). NMDA-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, 1; meningeal, 1). Most received high-dose steroids (n=17[90%]) and all improved but 3 had CCE relapses. This study highlights the CCE spectrum and provides insight into its pathogenesis. This article is protected by copyright. All rights reserved.
Browse by Audience
