Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Cristina Valencia-Sanchez, Yong Guo, Karl N. Krecke, John Chen, Vyanka Redenbaugh, Mayra Montalvo, Paul M. Elsbernd, Jan Mendelt Tillema, Sebastian Lopez-Chiriboga, Adrian Budhram, Elia Sechi, Amy Kunchok, Divyanshu Dubey, Sean J. Pittock, Claudia F. Lucchinetti, Eoin P. Flanagan

Research output: Contribution to journalArticlepeer-review

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 2022.

Original languageEnglish (US)
JournalAnnals of neurology
DOIs
StateAccepted/In press - 2022

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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