Abstract

Objective: To clarify the clinical and radiological features of adult onset anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated bilateral medial frontal cerebral cortical encephalitis (BFCCE).Methods: We systematically reviewed the literature for patients with anti-MOG antibody-associated BFCCE. Patients who were also positive for other encephalitis-related autoantibodies were excluded from the study. The frequency of several characteristic neurological symptoms and lesion distributions were analyzed.Results: We identified six patients with anti-MOG antibody-associated BFCCE. Among them, 6/6 had headache, 4/6 had fever, 3/6 had seizure, 2/6 had paraparesis, 2/6 had lethargy, and 2/6 had memory disturbance. CSF pleocytosis was observed in 5/6 patients, while CSF myelin basic protein was not elevated in any of the six patients. On brain MRI, 6/6 had bilateral medial frontal cortical lesions, 3/6 had corpus callosum lesions, and 3/6 had leptomeningeal enhancements. Most of the lesions were distributed in the territory of the anterior cerebral artery (ACA).Conclusion: Our results indicate that anti-MOG antibody-associated BFCCE presents with characteristic clinical symptoms and MRI findings, which might reflect lesion formation in the ACA territory.

Highlights

  • Anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a recently established spectrum of diseases

  • Conformation-sensitive antibodies against MOG are detectable in patients with optic neuritis, myelitis, opticomyelitis, acute or multiphasic disseminated encephalomyelitis (ADEM/MDEM) and brainstem/cerebral cortical encephalitis, they are rarely detected in patients with prototypic multiple sclerosis or anti-aquaporin 4 (AQP4) antibody-positive neuromyelitis optica spectrum disorders (NMOSDs) [1,2,3,4,5,6,7,8,9,10,11,12,13]

  • Cerebrospinal fluid (CSF) pleocytosis was observed in these patients, and brain MRI demonstrated unilateral cerebral cortical hyperintensities best seen on fluid-attenuated inversion recovery (FLAIR) sequence, which were swollen and corresponded to hyperperfusion on single photon emission computed tomography (SPECT)

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Summary

Introduction

Anti-myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a recently established spectrum of diseases. Cerebrospinal fluid (CSF) pleocytosis was observed in these patients, and brain MRI demonstrated unilateral cerebral cortical hyperintensities best seen on fluid-attenuated inversion recovery (FLAIR) sequence, which were swollen and corresponded to hyperperfusion on single photon emission computed tomography (SPECT). Subsequent to these reports, similar cases have been reported that have broadened the clinical spectrum of this disorder [11, 14,15,16,17]. The bilateral medial frontal type was reported in a case report in which an adult patient presented with secondary generalized seizure, CSF pleocytosis, and bilateral cerebral cortical hyperintensities best seen on FLAIR sequence [10]

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