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

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          Abstract

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

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          MOG encephalomyelitis: international recommendations on diagnosis and antibody testing

          S. Jarius, F Paul, O Aktas … (2018)
          Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD). Owing to a substantial overlap in clinicoradiological presentation, MOG-EM was often unwittingly misdiagnosed as MS in the past. Accordingly, increasing numbers of patients with suspected or established MS are currently being tested for MOG-IgG. However, screening of large unselected cohorts for rare biomarkers can significantly reduce the positive predictive value of a test. To lessen the hazard of overdiagnosing MOG-EM, which may lead to inappropriate treatment, more selective criteria for MOG-IgG testing are urgently needed. In this paper, we propose indications for MOG-IgG testing based on expert consensus. In addition, we give a list of conditions atypical for MOG-EM (“red flags”) that should prompt physicians to challenge a positive MOG-IgG test result. Finally, we provide recommendations regarding assay methodology, specimen sampling and data interpretation.
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            Clinical presentation and prognosis in MOG-antibody disease: a UK study

            Maciej Juryńczyk, Silvia Messina, Mark R. Woodhall … (2017)
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              Clinical course, therapeutic responses and outcomes in relapsing MOG antibody-associated demyelination

              Sudarshini Ramanathan, Shekeeb S. Mohammad, Esther Tantsis … (2017)
              Objective We characterised the clinical course, treatment and outcomes in 59 patients with relapsing myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination. Methods We evaluated clinical phenotypes, annualised relapse rates (ARR) prior and on immunotherapy and Expanded Disability Status Scale (EDSS), in 218 demyelinating episodes from 33 paediatric and 26 adult patients. Results The most common initial presentation in the cohort was optic neuritis (ON) in 54% (bilateral (BON) 32%, unilateral (UON) 22%), followed by acute disseminated encephalomyelitis (ADEM) (20%), which occurred exclusively in children. ON was the dominant phenotype (UON 35%, BON 19%) of all clinical episodes. 109/226 (48%) MRIs had no brain lesions. Patients were steroid responsive, but 70% of episodes treated with oral prednisone relapsed, particularly at doses <10 mg daily or within 2 months of cessation. Immunotherapy, including maintenance prednisone (P=0.0004), intravenous immunoglobulin, rituximab and mycophenolate, all reduced median ARRs on-treatment. Treatment failure rates were lower in patients on maintenance steroids (5%) compared with non-steroidal maintenance immunotherapy (38%) (P=0.016). 58% of patients experienced residual disability (average follow-up 61 months, visual loss in 24%). Patients with ON were less likely to have sustained disability defined by a final EDSS of ≥2 (OR 0.15, P=0.032), while those who had any myelitis were more likely to have sustained residual deficits (OR 3.56, P=0.077). Conclusion Relapsing MOG antibody-associated demyelination is strongly associated with ON across all age groups and ADEM in children. Patients are highly responsive to steroids, but vulnerable to relapse on steroid reduction and cessation.
              Article 0 comments Cited 207 times – based on 0 reviews     Review now
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                Author and article information

                Journal
                Title: JAMA Neurology
                Abbreviated Title: JAMA Neurol
                Publisher: American Medical Association (AMA)
                ISSN (Print): 2168-6149
                Publication date (Electronic): December 21 2018
                Affiliations
                [1 ]Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minnesota
                [2 ]Department of Laboratory Medicine and Pathology, Mayo Clinic College of Medicine, Rochester, Minnesota
                [3 ]Department of Radiology (Division of Neuroradiology), Mayo Clinic College of Medicine, Rochester, Minnesota
                [4 ]Department of Ophthalmology, Mayo Clinic College of Medicine, Rochester, Minnesota
                [5 ]Department of Pediatrics, University of Colorado School of Medicine, Aurora
                [6 ]Department of Neurology, University of Colorado School of Medicine, Aurora
                Article
                DOI: 10.1001/jamaneurol.2018.4053
                PMC ID: 6440233
                PubMed ID: 30575890
                SO-VID: 3189b64e-9d73-41c3-b15e-f1d341e6436f
                Copyright © © 2018
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