Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

Ann Neurol. 2023 Feb;93(2):297-302. doi: 10.1002/ana.26549. Epub 2022 Dec 2.

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.

Publication types

  • Observational Study
  • Research Support, N.I.H., Extramural

MeSH terms

  • Autoantibodies
  • Encephalitis* / diagnostic imaging
  • Humans
  • Magnetic Resonance Imaging
  • Myelin-Oligodendrocyte Glycoprotein
  • Retrospective Studies

Substances

  • Myelin-Oligodendrocyte Glycoprotein
  • Autoantibodies