Cerebral Cortical Encephalitis and Other Meningocortical Manifestations of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children: Case Series and Review of the Literature

J Child Neurol. 2024 Nov;39(13-14):487-493. doi: 10.1177/08830738241282354. Epub 2024 Sep 23.

Abstract

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody-associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody-associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG-positive meningocortical syndromes.

Keywords: cortical cerebral encephalitis (CCE); demyelinating; myelin oligodendrocyte glycoprotein (MOG); myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD); seizure.

Publication types

  • Review
  • Case Reports

MeSH terms

  • Adolescent
  • Autoantibodies* / blood
  • Cerebral Cortex / diagnostic imaging
  • Cerebral Cortex / pathology
  • Child
  • Child, Preschool
  • Electroencephalography
  • Encephalitis* / diagnosis
  • Encephalitis* / immunology
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Myelin-Oligodendrocyte Glycoprotein* / immunology
  • Retrospective Studies

Substances

  • Myelin-Oligodendrocyte Glycoprotein
  • Autoantibodies
  • MOG protein, human