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Proposed Criteria for the Diagnosis of MOGAD
TAKE-HOME MESSAGE
- The spectrum of myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD) is ever-expanding. There is an utmost need for diagnostic criteria to help clinicians accurately diagnose and counsel patients and their families, and to reduce the chances of under- or overdiagnosis. A distinct international panel of experts reviewed the literature and proposed diagnostic criteria for MOGAD. The criteria combine clinical, laboratory, and imaging findings to support the diagnosis of MOGAD.
- The detailed and easy-to-follow proposed guidelines will help clinicians in diagnosing MOGAD and in counselling patients and their families on this relatively new and not uncommon disease entity.
abstract
This abstract is available on the publisher's site.
Access this abstract now Full Text Available for ClinicalKey SubscribersSerum antibodies directed against myelin oligodendrocyte glycoprotein (MOG) are found in patients with acquired CNS demyelinating syndromes that are distinct from multiple sclerosis and aquaporin-4-seropositive neuromyelitis optica spectrum disorder. Based on an extensive literature review and a structured consensus process, we propose diagnostic criteria for MOG antibody-associated disease (MOGAD) in which the presence of MOG-IgG is a core criterion. According to our proposed criteria, MOGAD is typically associated with acute disseminated encephalomyelitis, optic neuritis, or transverse myelitis, and is less commonly associated with cerebral cortical encephalitis, brainstem presentations, or cerebellar presentations. MOGAD can present as either a monophasic or relapsing disease course, and MOG-IgG cell-based assays are important for diagnostic accuracy. Diagnoses such as multiple sclerosis need to be excluded, but not all patients with multiple sclerosis should undergo screening for MOG-IgG. These proposed diagnostic criteria require validation but have the potential to improve identification of individuals with MOGAD, which is essential to define long-term clinical outcomes, refine inclusion criteria for clinical trials, and identify predictors of a relapsing versus a monophasic disease course.
Additional Info
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Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria
Lancet Neurol 2023 Mar 01;22(3)268-282, B Banwell, JL Bennett, R Marignier, HJ Kim, F Brilot, EP Flanagan, S Ramanathan, P Waters, S Tenembaum, JS Graves, T Chitnis, AU Brandt, C Hemingway, R Neuteboom, L Pandit, M Reindl, A Saiz, DK Sato, K Rostasy, F Paul, SJ Pittock, K Fujihara, J PalaceFrom MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
Myelin oligodendrocyte glycoprotein (MOG) antibody–associated disease (MOGAD) is a recently recognized inflammatory demyelinating disease that is distinct from MS and aquaporin-4-IgG–positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD). There are existing international consensus diagnostic criteria for MS and AQP4+ NMODSD; but, until this publication, no such international panel consensus diagnostic criteria existed for MOGAD.
This study brought together a diverse international group of experts that proposed diagnostic criteria for MOGAD.1 The first section of the manuscript focused on selecting the correct patients for MOG antibody testing and ensuring that the correct assay is utilized (cell-based assay). Testing for serum MOG-IgG via a cell-based assay is highly specific (>98%) but low positive MOG-IgG has been recognized in 1% to 2% of patients with other diseases.2 For this reason, the panel does not recommend universal screening for MOG-IgG in all patients with MS, as it could lead to a high frequency of false-positive results. Moreover, the criteria required additional supportive features for those with low positive MOG-IgG.
The criteria are divided into three parts (A, B, and C), and fulfillment of all three are required for MOGAD diagnosis. Part A requires at least one core clinical demyelinating event, which includes optic neuritis, myelitis, acute disseminated encephalomyelitis, cerebral monofocal or polyfocal deficits, brainstem or cerebellar deficits, or cerebral cortical encephalitis. Cerebral cortical encephalitis syndrome is a recently recognized MOGAD phenotype that often manifests with headache, seizures, encephalopathy, and fever and shows extensive cortical T2 hyperintensity with or without leptomeningeal enhancement.3,4 Part B requires positivity for MOG-IgG using a cell-based assay. A clear positive serum MOG-IgG result fulfills part B. However, a low positive serum MOG-IgG result, serum positive MOG-IgG without titer results available, and CSF MOG-IgG positivity of any titer all need additional supportive features to fulfill part B. These requirements include a negative aquaporin-4-IgG test result and at least one additional supporting clinical or MRI feature. Finally, for part C, there should be exclusion of a better explanation, including MS. The manuscript includes tables comparing MOGAD with MS and AQP4+ NMOSD. It also highlights red flags that might suggest an alternative diagnosis, including a hyperacute onset, a progressive course, MRI features favoring MS more, and positive CSF oligoclonal bands.
This publication represents a major step forward for patients with MOGAD and ensures that this disease has its own identity separate from MS and NMOSD.5 The criteria are likely to be used for inclusion in upcoming clinical trials, which are so crucial for this disease, which lacks any proven treatment. Future studies are needed to validate these MOGAD criteria in patient cohorts around the world. In summary, these proposed MOGAD diagnostic criteria require all three of the following: A) a core clinical demyelinating event, B) positivity for MOG antibody, and C) exclusion of a better explanation.
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