Mechanisms of disease: Aquaporin-4 antibodies in neuromyelitis optica

Sven Jarius, Friedemann Paul, Diego Franciotta, Patrick Waters, Frauke Zipp, Reinhard Hohlfeld, Angela Vincent, Brigitte Wildemann

Research output: Contribution to journalArticlepeer-review

Abstract

Neuromyelitis optica (NMO) is a rare CNS inflammatory disorder that predominantly affects the optic nerves and spinal cord. Recent serological findings strongly suggest that NMO is a distinct disease rather than a subtype of multiple sclerosis. In NMO, serum antibodies, collectively known as NMO-IgG, characteristically bind to cerebral microvessels, pia mater and Virchow-Robin spaces. The main target antigen for this immunoreactivity has been identified as aquaporin-4 (AQP4). The antibodies are highly specific for NMO, and they are also found in patients with longitudinally extensive transverse myelitis without optic neuritis, which is thought to be a precursor to NMO in some cases. An antibody-mediated pathogenesis for NMO is supported by several observations, including the characteristics of the AQP4 antibodies, the distinct NMO pathology - which includes IgG and complement deposition and loss of AQP4 from spinal cord lesions - and emerging evidence of the beneficial effects of B-cell depletion and plasma exchange. Many aspects of the pathogenesis, however, remain unclear.

Original languageEnglish
Pages (from-to)202-214
Number of pages13
JournalNature clinical practice. Neurology
Volume4
Issue number4
DOIs
Publication statusPublished - Apr 2008

ASJC Scopus subject areas

  • Neuroscience(all)
  • Medicine(all)

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