Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody‐Associated Disease
Cristina Valencia‐Sanchez, Yong Guo, Karl N. Krecke, John J. Chen, Vyanka Redenbaugh, Mayra Montalvo, Paul Elsbernd, Jan‐Mendelt Tillema, A. Sebastian López‐Chiriboga, Adrian Budhram, Elia Sechi, Amy Kunchok, Divyanshu Dubey, Sean J. Pittock, Claudia F. Lucchinetti, Eoin P. Flanagan
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.