Relapsing meningitis and limbic encephalitis in anti-AQP4-Ab-associated neuromyelitis optica spectrum disorder

Mult Scler. 2024 Nov;30(13):1692-1696. doi: 10.1177/13524585241261549. Epub 2024 Jul 30.

Abstract

Objectives: neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease mainly affecting optic nerves and the spinal cord. Due to the potentially irreversible tissue damage, prevention of relapses is of utmost importance.

Methods: We describe the atypical clinical course and pathology results of a patient with anti-aquaporin-4 antibody (anti-AQP4-Ab)-associated NMOSD who developed aseptic meningitis followed by limbic-encephalitis-like presentation with extensive brain lesions upon treatment with rituximab and tocilizumab.

Results: The patient developed subacute cognitive decline with magnetic resonance imaging (MRI) evidence of extensive brain white matter lesions. In the hypothesis of an opportunistic brain infection, she underwent brain biopsy of the temporal pole. Pathology results revealed typical NMOSD findings with complement activation, supporting the hypothesis of an atypical presentation of anti-AQP-Ab-associated NMOSD. Accordingly, treatment with the complement-targeting drug eculizumab was started, leading to a dramatic clinical and MRI improvement.

Discussion: aseptic meningitis and limbic encephalitis could represent a rare phenotype of anti-AQP4-Ab-associated NMOSD.

Keywords: NMOSD; eculizumab; limbic encephalitis; meningitis.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Aquaporin 4* / immunology
  • Autoantibodies*
  • Female
  • Humans
  • Limbic Encephalitis* / drug therapy
  • Limbic Encephalitis* / immunology
  • Magnetic Resonance Imaging
  • Meningitis, Aseptic / drug therapy
  • Meningitis, Aseptic / etiology
  • Meningitis, Aseptic / immunology
  • Middle Aged
  • Neuromyelitis Optica* / diagnostic imaging
  • Neuromyelitis Optica* / drug therapy
  • Neuromyelitis Optica* / immunology
  • Neuromyelitis Optica* / pathology
  • Recurrence

Substances

  • Aquaporin 4
  • AQP4 protein, human
  • Autoantibodies
  • eculizumab
  • Antibodies, Monoclonal, Humanized