Unilateral Autoimmune Encephalitis: A Case Report on a Rare Manifestation of Myelin Oligodendrocyte Glycoprotein Antibody Disease

Cureus. 2023 Feb 14;15(2):e34994. doi: 10.7759/cureus.34994. eCollection 2023 Feb.

Abstract

Myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD) is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system (CNS) that has varying phenotypes. FLAIR (fluid-attenuated inversion recovery)-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES) is a much rarer manifestation of cortical encephalitis encountered in MOGAD. We report a rare case of a nine-year-old girl who presented with a drop in her academic performance and right-sided Epilepsia partialis continua. Magnetic resonance imaging (MRI) of the brain detected evidence for unilateral (left) cortical encephalitis with peri-ictal juxtacortical edema. An electroencephalogram revealed a hemi-generalized poly spike and wave discharges in the left hemisphere, several of which correlated with myoclonic jerks. The cerebrospinal fluid (CSF) analysis was normal. Autoimmune workup resulted in a positive serum MOG-immunoglobulin G (IgG), which confirmed the diagnosis of FLAMES. The child showed an excellent clinical response to intravenous methylprednisolone and intravenous immunoglobulins therapy.

Keywords: cortical encephalitis; epilepsia partialis continua (epc); flames; inflammatory demyelination; intravenous immunoglobulins (ivig); intravenous methylprednisolone pulse; mog antibody-associated disease; mogad; myelin oligodendrocyte glycoprotein (mog) antibodies; pediatric brain mri.

Publication types

  • Case Reports