Clinical spectrum and prognostic value of CNS MOG autoimmunity in adults: The MOGADOR study

Neurology. 2018 May 22;90(21):e1858-e1869. doi: 10.1212/WNL.0000000000005560. Epub 2018 Apr 25.

Abstract

Objective: To describe clinical and radiologic features associated with myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) in a large French nationwide adult cohort, to assess baseline prognostic features of MOG-Ab-associated diseases after a first acute demyelinating syndrome, and to evaluate the clinical value of MOG-Ab longitudinal analysis.

Methods: Clinical data were obtained from 197 MOG-Ab-positive patients ≥18 years of age. Complete imaging data were available in 108, and 54 serum samples were eligible for longitudinal evaluation. For survival analysis comparison, 169 aquaporin-4 antibody (AQP4-Ab)-positive patients from the NOMADMUS database were included.

Results: Median age at onset was 36.46 (range 18.0-76.8) years, and patients were predominantly white (92.9%) with male:female ratio, 1.1. Clinical phenotype at onset included optic neuritis or myelitis in 90.86%, isolated brainstem or encephalopathy syndromes in 6.6%, and a combination of syndromes in 2.5%. Distinctive brain MRI findings in MOG-Ab-positive patients were thalamic and pontine lesions. Cortical and leptomeningeal lesions were found in 16.3% and 6.1%, respectively. The probability of reaching a first relapse after 2 and 5 years was 44.8% and 61.8%, respectively. MOG-Ab-positive patients were at lower risk at presentation of further clinical relapse (hazard ratio [HR] 0.45, 95% confidence interval [CI] 0.26-0.79) compared to AQP4-Ab-positive individuals. MOG-Ab-positive individuals had a lower risk of reaching Disability Status Scale score of 3.0 (HR 0.46, 95% CI 0.22-0.94) and visual acuity of 20/100 (HR 0.23, 95% CI 0.07-0.72). Finally, MOG-Ab titers were higher at relapse than in remission (p = 0.009).

Conclusion: In adults, MOG-Ab-associated disease extends beyond clinical and radiologic abnormalities in the optic nerve and spinal cord. Despite the relapsing course, the overall visual and motor outcome is better compared with AQP4-Ab-positive patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aquaporin 4 / blood
  • Autoantibodies
  • Brain Diseases / blood
  • Brain Diseases / diagnosis*
  • Brain Diseases / immunology
  • Brain Diseases / pathology
  • Demyelinating Autoimmune Diseases, CNS / blood
  • Demyelinating Autoimmune Diseases, CNS / diagnosis*
  • Demyelinating Autoimmune Diseases, CNS / pathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein / blood
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Prognosis
  • Retrospective Studies
  • Spinal Cord Diseases / blood
  • Spinal Cord Diseases / diagnosis*
  • Spinal Cord Diseases / immunology
  • Spinal Cord Diseases / pathology
  • Young Adult

Substances

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