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Area of Science:

  • Neuroimmunology
  • Neuropathology

Background:

  • Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease targeting the central nervous system.
  • Aquaporin 4 antibodies are a key biomarker for NMOSD, particularly in relapsing myelitis.

Observation:

  • A 51-year-old woman with aquaporin 4-positive NMOSD presented with extensive brain lesions and fatal outcome.
  • Neuropathological examination revealed lesions in radiologically and grossly normal areas.
  • Immunostaining demonstrated massive astrocyte disintegration in both acute and chronic lesions.

Findings:

  • Astrocytes are confirmed as an early and primary target in the disease process.
  • Reverse-transcriptase polymerase chain reaction analysis indicated an induced immune response.
  • The findings support the role of astrocyte damage in the pathogenesis of NMOSD.

Implications:

  • This case contributes to the understanding of astrocyte pathology in neuromyelitis optica.
  • It highlights the critical role of immune mechanisms in the development of NMOSD.
  • Further research into astrocyte-targeted therapies for NMOSD is warranted.