Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature.

Jia Song,Pei Shang,Manqiu Ding,Yue Lang,Hang Shu,Li Cui

doi:10.3389/fnhum.2021.782490

Abstract

Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

Highlights

Myelin oligodendrocyte glycoprotein is a membrane protein uniquely expressed on the surface of oligodendrocytes and myelin in the central nervous system of humans and other mammals (Ramanathan et al, 2016; Jain et al, 2021)
We report two cases of cortical encephalitis (CCE) with positive myelin oligodendrocyte glycoprotein (MOG)-Ab and perform a systematic analysis of previously reported cases
To obtain a more thorough understanding of MOG-Abassociated CCE, we searched and screened studies in PubMed and Web of Science until August 2021 to identify patients according to the following process (Figure 2, review flowchart)

Summary

Introduction

Myelin oligodendrocyte glycoprotein is a membrane protein uniquely expressed on the surface of oligodendrocytes and myelin in the central nervous system of humans and other mammals (Ramanathan et al, 2016; Jain et al, 2021). MOG Antibody Associated Cortical Encephalitis acute disseminated encephalomyelitis, and aquaporin 4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorders (NMOSD) in terms of clinical phenotype, and is considered to be a new and independent entity in inflammatory demyelinating diseases of the central nervous system (Ramanathan et al, 2016; Cobo-Calvo et al, 2019). The female-to-male ratio is similar (Jurynczyk et al, 2017; Cobo-Calvo et al, 2021), and presentation at onset is usually acute disseminated encephalomyelitis, especially under 10 years of age, followed by optic neuritis (ON), transverse myelitis (TM), and brainstem demyelination (Fernandez-Carbonell et al, 2016; Duignan et al, 2018). Some patients relapsed during or after the withdrawal of steroids, most of whom showed ON (Reindl and Waters, 2019); a previous study suggested that a prolonged steroid taper can reduce early relapse of MOGAD (Narayan et al, 2018)

Objectives

Methods

Results

Conclusion