Clinical and MRI phenotype of children with MOG antibodies

Mult Scler. 2016 Feb;22(2):174-84. doi: 10.1177/1352458515587751. Epub 2015 Jun 3.

Abstract

Objective: To investigate the clinical and magnetic resonance imaging (MRI) features of anti-myelin oligodendrocyte glycoprotein (MOG) antibody-seropositive pediatric demyelinating syndromes.

Methods: Serum samples collected from 74 children with suspected demyelinating disorders whom were being followed at Massachusetts General Hospital were incubated with control green fluorescent protein (GFP)- and MOG-GFP-transfected Jurkat cell clones. The binding ratios were calculated using flow cytometry. Using statistical analyses, we compared the demographic, clinical and radiological features in our seropositive and seronegative patients.

Results: We found that 13 out of 74 (17.5%) patients were seropositive for MOG. The MOG-seropositive patients were younger than the seronegative patients (p = 0.049). No single disease category predominated among the seropositive patients, nor was one group more likely to have a polyphasic course. There were two out of four neuromyelitis optica (NMO) patients who had MOG antibodies; both were seronegative for aquaporin -4 (AQP4) antibodies. One had monophasic disease and the other had frequent relapses. There was a bimodal distribution of the MOG-seropositive patients by age at onset, with a distinct younger group (4-8 years) having a high prevalence of encephalopathy and an older group (13-18 years), whom presented almost exclusively with optic neuritis. MRI analysis demonstrated the absence of corpus callosum lesions in the seropositive patients (p = 0.012). The annualized relapse rate (ARR) and the Expanded Disability Status Scale (EDSS) results at 2 years did not differ between the seropositive and seronegative patients.

Conclusion: MOG antibodies are found across a variety of pediatric demyelinating syndromes having some distinct clinical and MRI features.

Keywords: Acute disseminated encephalomyelitis; demyelinating syndromes; encephalopathy; glycoprotein; magnetic resonance imaging; multiple sclerosis; myelin; myelin oligodendrocyte glycoprotein; neuromyelitis optica; optic neuritis; pediatric multiple sclerosis; serology.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Aquaporin 4 / immunology
  • Autoantibodies / immunology*
  • Brain / pathology*
  • Child
  • Demyelinating Autoimmune Diseases, CNS / immunology
  • Demyelinating Autoimmune Diseases, CNS / pathology
  • Demyelinating Autoimmune Diseases, CNS / physiopathology
  • Demyelinating Diseases / immunology
  • Demyelinating Diseases / pathology
  • Demyelinating Diseases / physiopathology
  • Encephalomyelitis, Acute Disseminated / immunology*
  • Female
  • Flow Cytometry
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Multiple Sclerosis / immunology*
  • Multiple Sclerosis / pathology
  • Multiple Sclerosis / physiopathology
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Neuromyelitis Optica / immunology*
  • Neuromyelitis Optica / pathology
  • Neuromyelitis Optica / physiopathology
  • Optic Neuritis / immunology*
  • Optic Neuritis / pathology
  • Optic Neuritis / physiopathology
  • Phenotype
  • Spinal Cord / pathology*

Substances

  • AQP4 protein, human
  • Aquaporin 4
  • Autoantibodies
  • MOG protein, human
  • Myelin-Oligodendrocyte Glycoprotein