Persisting myelin oligodendrocyte glycoprotein antibodies in aquaporin-4 antibody negative pediatric neuromyelitis optica

Mult Scler. 2013 Jul;19(8):1052-9. doi: 10.1177/1352458512470310. Epub 2012 Dec 20.


Background: Recently we showed that antibodies to myelin oligodendrocyte glycoprotein (MOG) can be found in aquaporin-4 (AQP4)-immunoglobulin (IgG) seronegative pediatric and adult patients with definite and high-risk neuromyelitis optica (NMO).

Objective: The purpose of this study was to describe the clinical characteristics and temporal dynamics of MOG-IgG in AQP4-IgG seronegative pediatric patients presenting with definite NMO.

Methods: Children with definite NMO who were referred for further testing of serum antibodies for AQP4 and MOG with a cell-based assay were included in this study. Clinical disease course, cerebrospinal fluid and magnetic resonance imaging (MRI) studies of these patients were reviewed.

Results: Between 2008 and 2012 eight children who fulfilled the diagnostic criteria of definite NMO were recruited. Two children with definite NMO tested positive for AQP4-IgG but were negative for MOG-IgG antibodies. Three children had an absence of AQP4-IgG and MOG-IgG antibodies. Three children with definite NMO had high titers of serum MOG-IgG antibodies (≥1: 160), but no AQP4-directed humoral immune response. Longitudinal analysis of serum samples of the latter three children showed persisting high MOG-IgG titers over time.

Conclusion: Pediatric patients presenting with clinical symptoms and MRI findings highly suggestive of NMO but with high and persisting MOG-IgG antibody titers are most likely to represent a distinct subgroup of acute demyelinating diseases with important clinical and therapeutic implications.

Keywords: AQP-4 antibodies; MOG antibodies; Neuromyelitis optica; children; optic neuritis.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aquaporin 4 / immunology
  • Autoantibodies / blood*
  • Autoantibodies / immunology
  • Autoantigens / immunology
  • Child
  • Female
  • Humans
  • Immunoglobulin G / immunology
  • Magnetic Resonance Imaging
  • Male
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Neuromyelitis Optica / blood*
  • Neuromyelitis Optica / immunology*


  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
  • Autoantigens
  • Immunoglobulin G
  • Myelin-Oligodendrocyte Glycoprotein