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

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

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...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) Vol. 15; no. 2; p. e34994
Main Authors: Maturu, Mohan V Sumedha, Datla, Aravind Varma, Maturu, Prajwala, Talla, Vinay B, Dalai, Sibasankar
Format: Journal Article
Language:English
Published: United States Cureus Inc 14-02-2023
Subjects:
Antibodies
Case reports
Children & youth
Convulsions & seizures
Edema
Encephalitis
Glycoproteins
Magnetic resonance imaging
Nervous system
Pathology
Patients
Pediatrics
epilepsia partialis continua (epc)
pediatric brain mri
intravenous immunoglobulins (ivig)
mog antibody-associated disease
inflammatory demyelination
intravenous methylprednisolone pulse
mogad
cortical encephalitis
flames
myelin oligodendrocyte glycoprotein (mog) antibodies
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Summary: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.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.34994