Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody‐Associated Disease. Issue 2 (2nd December 2022)

• Record Type: Journal Article
• Authors: Valencia‐Sanchez, Cristina; Guo, Yong; Krecke, Karl N.; Chen, John J.; Redenbaugh, Vyanka; Montalvo, Mayra; Elsbernd, Paul M.; Tillema, Jan‐Mendelt; Lopez‐Chiriboga, Sebastian; Budhram, Adrian; Sechi, Elia; Kunchok, Amy; Dubey, Divyanshu; Pittock, Sean J.; Lucchinetti, Claudia F.; Flanagan, Eoin P.
• Is Part Of: Annals of neurology. Volume 93:Issue 2(2023)
• Page Start: 297
• Page End: 302
• Publication Date: 2022-12-02
• Subjects: Neurology -- Periodicals; Pediatric neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; 616.8
• DOI: 10.1002/ana.26549
• Languages: English
• ISSNs: 0364-5134

Abstract

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