Progressive myelin oligodendrocyte glycoprotein-associated demyelination mimicking leukodystrophy

Mult Scler. 2022 Aug;28(9):1481-1484. doi: 10.1177/13524585221090737. Epub 2022 Jun 23.

Abstract

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may be associated with relapsing disease, but clinical progression independent of relapse activity is rare.

Objectives: To report progressive disease in a patient with MOGAD.

Methods: A single retrospective case report.

Results: At 4 years of age, the patient had a single episode of acute disseminated encephalomyelitis. She remained well until age 17 years but over the next 9 years developed progressive spastic quadriparesis, cognitive and bulbar dysfunction. Brain imaging showed a leukodystrophy-like pattern of white matter abnormality with contrast enhancement at different time points. Myelin oligodendrocyte glycoprotein (MOG)-IgG was repeatedly positive by live cell-based assay.

Conclusion: Secondary progression may be a rare presentation of MOG-IgG-associated disease.

Keywords: MOGAD; myelin oligodendrocyte glycoprotein antibody-associated disease; neuroinflammation; neuromyelitis optica; progressive; progressive MOGAD.

Publication types

  • Case Reports

MeSH terms

  • Aquaporin 4
  • Autoantibodies
  • Encephalomyelitis, Acute Disseminated*
  • Female
  • Humans
  • Immunoglobulin G
  • Myelin-Oligodendrocyte Glycoprotein
  • Neoplasm Recurrence, Local
  • Neuromyelitis Optica*
  • Retrospective Studies

Substances

  • Aquaporin 4
  • Autoantibodies
  • Immunoglobulin G
  • Myelin-Oligodendrocyte Glycoprotein