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

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.

Figure 1.

Brain magnetic resonance imaging (MRI) of patients with MOG-IgG–positive meningocortical syndromes. Patient 1: Axial T2-weighted fluid-attenuated inversion recovery (T2-FLAIR) image post-gadolinium (A) during initial presentation demonstrates cortical hyperintensity of the left temporal, parietal, and occipital lobes with associated leptomeningeal enhancement as well as juxtacortical hypointensity. Axial T2-weighted fluid-attenuated inversion recovery image post-gadolinium (B) during relapse demonstrates right frontoparietal leptomeningeal enhancement. Axial arterial spin labeling (ASL) image (C) demonstrates corresponding area of localized hyperperfusion. Patient 2: Axial T2-weighted fluid-attenuated inversion recovery image pre-gadolinium demonstrates bilateral frontal lobe hyperintensity and juxtacortical hypointensity (D), and axial T1 image post-gadolinium demonstrates right frontal leptomeningeal enhancement (E). Patient 3: Axial T2-weighted fluid-attenuated inversion recovery image post-gadolinium (F) demonstrates right superior frontal leptomeningeal enhancement. Patient 4: Axial diffusion-weighted imaging (DWI) image (G) demonstrates right frontoparietal restricted diffusion with corresponding apparent diffusion coefficient (ADC) image (H). Patient 5: Axial T2 pre-gadolinium images (I, J) demonstrate left frontal hyperintensity of the cortex with juxtacortical hyperintensity. Axial T1 post-gadolinium images (K, L) demonstrate left frontal leptomeningeal and subcortical enhancement. Axial ASL image (M) demonstrates possible area of corresponding localized hyperperfusion.