Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease Presenting as Intracranial Hypertension: A Case Report

Neurol Neuroimmunol Neuroinflamm. 2022 Oct 19;9(6):e200020. doi: 10.1212/NXI.0000000000200020. Print 2022 Nov.

Abstract

The production of autoantibodies against myelin oligodendrocyte glycoprotein (MOG) can cause a spectrum of autoimmune disorders, including optic neuritis, transverse myelitis, brainstem encephalitis, and acute disseminated encephalomyelitis. In this study, we present the case of a 19-year-old woman with an unusual clinical presentation of intracranial hypertension (IH) and bilateral papilledema. The patient presented with symptoms of increased intracranial pressure, which followed a relapsing, remitting course over several months. Serial CSF studies showed an increased opening pressure during clinical relapses. The CSF and serum tested positive for MOG immunoglobulin G antibodies. Contrast-enhanced MRI of the brain showed mild meningeal enhancement in the left parietal region with subtle underlying cortical hyperintensities, indicating possible fluid-attenuated inversion recovery variable unilateral enhancement of the leptomeninges. The patient responded well to immunosuppressive therapy using rituximab. The presentation of MOG antibody-associated disease (MOGAD) as IH without optic neuritis is rare. This report presents the first description of a relapsing remitting course presenting each time with only symptoms of raised intracranial pressure, without developing any typical clinical manifestations of MOGAD.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies
  • Humans
  • Immunoglobulin G
  • Intracranial Hypertension* / diagnosis
  • Intracranial Hypertension* / etiology
  • Myelin-Oligodendrocyte Glycoprotein
  • Neoplasm Recurrence, Local
  • Optic Neuritis* / diagnosis
  • Optic Neuritis* / drug therapy
  • Rituximab

Substances

  • Myelin-Oligodendrocyte Glycoprotein
  • Rituximab
  • Autoantibodies
  • Immunoglobulin G