Clinical and immunological differences between MOG associated disease and anti AQP4 antibody-positive neuromyelitis optica spectrum disorders: Blood-brain barrier breakdown and peripheral plasmablasts

Mult Scler Relat Disord. 2020 Jun:41:102005. doi: 10.1016/j.msard.2020.102005. Epub 2020 Feb 12.

Abstract

Background: Patients with anti-aquaporin-4 (AQP4) water channel antibody-positive neuromyelitis optica spectrum disorders (AQP4-NMOSD) and myelin oligodendrocyte glycoprotein (MOG) associated disease (MOGAD) often present with similar clinical symptoms, and some cases are hard to differentiate at the time of onset. In this study, we compared the clinical characteristics, cerebrospinal fluid (CSF) analysis parameters, and peripheral T/B lymphocyte subsets during the active and chronic phases in AQP4-NMOSD and MOGAD.

Methods: A total of 17 MOGAD cases and 24 AQP4-NMOSD cases were studied. The clinical characteristics in both groups were summarized, including disease duration, total number of attacks, lesions, prevention of relapse during remission, and CSF analysis results during the active phase. T/B lymphocyte subsets were further investigated in the active and chronic phases.

Results: In the comparative study on clinical symptoms, a large proportion of optic neuritis was unilateral in MOGAD. In the comparative study on CSF analysis, protein level was significantly lower in MOGAD compared with AQP4-NMOSD (p = 0.006); myelin basic protein was significantly lower in MOGAD compared with AQP4-NMOSD (p = 0.04); albumin quotient was significantly lower in MOGAD compared with AQP4-NMOSD (p = 0.02); and IgG Quotient was significantly lower in MOGAD compared with AQP4-NMOSD (p = 0.05). In the analysis of T/B lymphocyte subsets, plasmablasts of the B cell subset in the active phase were significantly lower in MOGAD (2.1 ± 2.4) compared to AQP4-NMOSD (7.8 ± 7.2) (p < 0.05). In the chronic phase, transitional B cells were significantly higher in MOGAD (2.1 ± 1.8) compared to AQP4-NMOSD (0.6 ± 0.4) (p < 0.01).

Conclusion: Clinical characteristics of MOGAD were similar to those of AQP4-NMOSD, but increased blood brain barrier permeability was suggested to be less severe in MOGAD compared to AQP4-NMOSD from CSF analysis. Furthermore, the pathogenesis of the two diseases was clearly distinct as plasmablasts in the active phase were not elevated in MOGAD, but were increased in AQP4-NMOSD.

Keywords: Aquaporin 4; Blood brain barrier; Myelin oligodendrocyte glycoprotein associated disease; Neuromyelitis optica spectrum disorders; Plasmablast.

Publication types

  • Comparative Study

MeSH terms

  • Adult
  • Aquaporin 4 / immunology*
  • B-Lymphocyte Subsets*
  • Blood-Brain Barrier / physiopathology*
  • Demyelinating Autoimmune Diseases, CNS* / blood
  • Demyelinating Autoimmune Diseases, CNS* / cerebrospinal fluid
  • Demyelinating Autoimmune Diseases, CNS* / immunology
  • Demyelinating Autoimmune Diseases, CNS* / physiopathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Neuromyelitis Optica / blood
  • Neuromyelitis Optica / cerebrospinal fluid
  • Neuromyelitis Optica / immunology
  • Neuromyelitis Optica / physiopathology
  • Optic Neuritis* / blood
  • Optic Neuritis* / cerebrospinal fluid
  • Optic Neuritis* / immunology
  • Optic Neuritis* / physiopathology
  • T-Lymphocyte Subsets*

Substances

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