MOG antibody-related disorders: common features and uncommon presentations

J Neurol. 2017 Sep;264(9):1945-1955. doi: 10.1007/s00415-017-8583-z. Epub 2017 Aug 2.

Abstract

Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) have been reported in acute demyelinating encephalomyelitis (ADEM), optic neuritis (ON), and neuromyelitis optica spectrum disorders (NMOSD) in adults and pediatrics. We aimed to delineate the common features of MOG-Ab-related disorders in children and adults, and report uncommon presentations. Twenty-seven consecutive pediatric and adult patients testing positive for MOG-Ab, with a minimum follow-up of 6 months, were included. Comprehensive epidemiological, clinical, radiological, and laboratory data were retrospectively analyzed. Additionally, we compared radiological features between ADEM MOG-Ab-positive patients, and a group of ADEM MOG-Ab-negative ones, recruited during the same period. Among the whole cohort, 13 (48.1%) were pediatric, and 14 (51.9%) were female. MOG-Ab-related disorders comprised eight ADEM, eight ON, five isolated myelitis, four with NMOSD and two patients with multiple sclerosis, at last follow-up. After a median follow-up of 17.8 months, 11 (40.7%) patients presented a relapse. The most frequent clinical phenotype at onset was encephalopathy in pediatrics (53.9%) and myelitis in adults (50%) (p = 0.013). There were no other differences between both groups. When comparing ADEM MOG-Ab positive and negative patients, bilateral thalamic lesions were more often found in the positive group (p = 0.010). Unusual presentations were identified in three patients: patchy spinal cord gadolinium-enhancing lesions, an associated teratoma, and one presented with status epilepticus. MOG-Ab-related disorders shared common clinical and prognostic features, but encompass a spectrum wider than recently reported.

Keywords: Aquaporin-4 antibodies; MOG antibodies; Multiple sclerosis; Neuromyelitis optica.

MeSH terms

  • Adolescent
  • Adult
  • Age Factors
  • Aged
  • Autoantibodies / blood*
  • Child
  • Child, Preschool
  • Cohort Studies
  • Encephalomyelitis* / blood
  • Encephalomyelitis* / diagnostic imaging
  • Encephalomyelitis* / immunology
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Myelin-Oligodendrocyte Glycoprotein / immunology*
  • Neuromyelitis Optica* / blood
  • Neuromyelitis Optica* / diagnostic imaging
  • Neuromyelitis Optica* / immunology
  • Optic Neuritis* / blood
  • Optic Neuritis* / diagnostic imaging
  • Optic Neuritis* / immunology
  • Statistics, Nonparametric
  • Young Adult

Substances

  • Autoantibodies
  • Myelin-Oligodendrocyte Glycoprotein