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Original Investigation
December 21, 2018

Clinical, Radiologic, and Prognostic Features of Myelitis Associated With Myelin Oligodendrocyte Glycoprotein Autoantibody

Author Affiliations
  • 1Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota
  • 2Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota
  • 3Department of Radiology (Division of Neuroradiology), Mayo Clinic College of Medicine, Rochester, Minnesota
  • 4Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, Minnesota
  • 5Department of Pediatrics, University of Colorado School of Medicine, Aurora
  • 6Department of Neurology, University of Colorado School of Medicine, Aurora
JAMA Neurol. Published online December 21, 2018. doi:10.1001/jamaneurol.2018.4053
Key Points

Question  What are the clinical and radiologic features of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis, and what distinguishes it from other myelitis etiologies?

Findings  In this observational study, MOG-IgG myelitis may present with an acute flaccid myelitis phenotype. Longitudinally extensive T2-hyperintense lesions are typical, but most patients have more than 1 lesion, and an H-shaped axial T2 pattern confined to gray matter and lack of enhancement are discriminating features from aquaporin-4–IgG and MS myelitis.

Meaning  Recognition of the clinical and radiologic characteristics of MOG-IgG myelitis and its discriminators from other etiologies will help clinicians identify patients with myelitis in whom MOG-IgG should be tested.

Abstract

Importance  Recognizing the characteristics of myelin oligodendrocyte glycoprotein autoantibody (MOG-IgG) myelitis is essential for early accurate diagnosis and treatment.

Objective  To evaluate the clinical, radiologic, and prognostic features of MOG-IgG myelitis and compare with myelitis with aquaporin-4–IgG (AQP4-IgG) and multiple sclerosis (MS).

Design, Setting, and Participants  We retrospectively identified 199 MOG-IgG–positive Mayo Clinic patients from January 1, 2000, through December 31, 2017, through our neuroimmunology laboratory. Fifty-four patients met inclusion criteria of (1) clinical myelitis; (2) MOG-IgG positivity; and (3) medical records available. We excluded 145 patients without documented myelitis. Myelitis of AQP4-IgG (n = 46) and MS (n = 26) were used for comparison.

Main Outcomes and Measures  Outcome variables included modified Rankin score and need for gait aid. A neuroradiologist analyzed spine magnetic resonance imaging of patients with MOG-IgG and control patients blinded to diagnosis.

Results  Of 54 included patients with MOG-IgG myelitis, the median age was 25 years (range, 3-73 years) and 24 were women (44%). Isolated transverse myelitis was the initial manifestation in 29 patients (54%), and 10 (19%) were initially diagnosed as having viral/postviral acute flaccid myelitis. Cerebrospinal fluid–elevated oligoclonal bands occurred in 1 of 38 (3%). At final follow-up (median, 24 months; range, 2-120 months), 32 patients (59%) had developed 1 or more relapses of optic neuritis (n = 31); transverse myelitis (n = 7); or acute disseminated encephalomyelitis (n = 1). Clinical features favoring MOG-IgG myelitis vs AQP4-IgG or MS myelitis included prodromal symptoms and concurrent acute disseminated encephalomyelitis. Magnetic resonance imaging features favoring MOG-IgG over AQP4-IgG or MS myelitis were T2-signal abnormality confined to gray matter (sagittal line and axial H sign) and lack of enhancement. Longitudinally extensive T2 lesions were of similar frequency in MOG-IgG and AQP4-IgG myelitis (37 of 47 [79%] vs 28 of 34 [82%]; P = .52) but not found in MS. Multiple spinal cord lesions and conus involvement were more frequent with MOG-IgG than AQP4-IgG but not different from MS. Wheelchair dependence at myelitis nadir occurred in one-third of patients with MOG-IgG and AQP4-IgG but never with MS, although patients with MOG-IgG myelitis recovered better than those with AQP4-IgG.

Conclusions and Relevance  Myelitis is an early manifestation of MOG-IgG–related disease and may have a clinical phenotype of acute flaccid myelitis. We identified a variety of clinical and magnetic resonance imaging features that may help clinicians identify those at risk in whom MOG-IgG should be tested.

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