Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management
Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is the most recently defined inflammatory demyelinating disease of the central nervous system (CNS). Over the last decade, several studies have helped delineate the characteristic clinical-MRI phenotypes of the disease, al...
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| Format: | Article |
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Frontiers Media S.A.
2022-06-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.885218/full |
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| author | Elia Sechi Laura Cacciaguerra Laura Cacciaguerra John J. Chen John J. Chen Sara Mariotto Giulia Fadda Alessandro Dinoto A. Sebastian Lopez-Chiriboga Sean J. Pittock Sean J. Pittock Eoin P. Flanagan Eoin P. Flanagan |
| author_facet | Elia Sechi Laura Cacciaguerra Laura Cacciaguerra John J. Chen John J. Chen Sara Mariotto Giulia Fadda Alessandro Dinoto A. Sebastian Lopez-Chiriboga Sean J. Pittock Sean J. Pittock Eoin P. Flanagan Eoin P. Flanagan |
| author_sort | Elia Sechi |
| collection | DOAJ |
| description | Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is the most recently defined inflammatory demyelinating disease of the central nervous system (CNS). Over the last decade, several studies have helped delineate the characteristic clinical-MRI phenotypes of the disease, allowing distinction from aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD) and multiple sclerosis (MS). The clinical manifestations of MOGAD are heterogeneous, ranging from isolated optic neuritis or myelitis to multifocal CNS demyelination often in the form of acute disseminated encephalomyelitis (ADEM), or cortical encephalitis. A relapsing course is observed in approximately 50% of patients. Characteristic MRI features have been described that increase the diagnostic suspicion (e.g., perineural optic nerve enhancement, spinal cord H-sign, T2-lesion resolution over time) and help discriminate from MS and AQP4+NMOSD, despite some overlap. The detection of MOG-IgG in the serum (and sometimes CSF) confirms the diagnosis in patients with compatible clinical-MRI phenotypes, but false positive results are occasionally encountered, especially with indiscriminate testing of large unselected populations. The type of cell-based assay used to evaluate for MOG-IgG (fixed vs. live) and antibody end-titer (low vs. high) can influence the likelihood of MOGAD diagnosis. International consensus diagnostic criteria for MOGAD are currently being compiled and will assist in clinical diagnosis and be useful for enrolment in clinical trials. Although randomized controlled trials are lacking, MOGAD acute attacks appear to be very responsive to high dose steroids and plasma exchange may be considered in refractory cases. Attack-prevention treatments also lack class-I data and empiric maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the presenting attack. A variety of empiric steroid-sparing immunosuppressants can be considered and may be efficacious based on retrospective or prospective observational studies but prospective randomized placebo-controlled trials are needed to better guide treatment. In summary, this article will review our rapidly evolving understanding of MOGAD diagnosis and management. |
| first_indexed | 2024-04-13T17:55:15Z |
| format | Article |
| id | doaj.art-8ea3145bad9b497e8ca5f0062cdd90ae |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-13T17:55:15Z |
| publishDate | 2022-06-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-8ea3145bad9b497e8ca5f0062cdd90ae2022-12-22T02:36:33ZengFrontiers Media S.A.Frontiers in Neurology1664-22952022-06-011310.3389/fneur.2022.885218885218Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and ManagementElia Sechi0Laura Cacciaguerra1Laura Cacciaguerra2John J. Chen3John J. Chen4Sara Mariotto5Giulia Fadda6Alessandro Dinoto7A. Sebastian Lopez-Chiriboga8Sean J. Pittock9Sean J. Pittock10Eoin P. Flanagan11Eoin P. Flanagan12Neurology Unit, Department of Medical, Surgical and Experimental Sciences, University of Sassari, Sassari, ItalyNeuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute and Vita-Salute San Raffaele University, Milan, ItalyDepartment of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology Mayo Clinic, Rochester, MN, United StatesDepartment of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology Mayo Clinic, Rochester, MN, United StatesDepartment of Ophthalmology, Mayo Clinic, Rochester, MN, United StatesNeurology Unit, Department of Neurosciences, Biomedicine, and Movement Sciences, University of Verona, Verona, ItalyDepartment of Neurology and Neurosurgery, McGill University, Montreal, QC, CanadaNeurology Unit, Department of Neurosciences, Biomedicine, and Movement Sciences, University of Verona, Verona, ItalyDepartment of Neurology, Mayo Clinic, Jacksonville, FL, United StatesDepartment of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology Mayo Clinic, Rochester, MN, United StatesDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United StatesDepartment of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology Mayo Clinic, Rochester, MN, United StatesDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, United StatesMyelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is the most recently defined inflammatory demyelinating disease of the central nervous system (CNS). Over the last decade, several studies have helped delineate the characteristic clinical-MRI phenotypes of the disease, allowing distinction from aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG+NMOSD) and multiple sclerosis (MS). The clinical manifestations of MOGAD are heterogeneous, ranging from isolated optic neuritis or myelitis to multifocal CNS demyelination often in the form of acute disseminated encephalomyelitis (ADEM), or cortical encephalitis. A relapsing course is observed in approximately 50% of patients. Characteristic MRI features have been described that increase the diagnostic suspicion (e.g., perineural optic nerve enhancement, spinal cord H-sign, T2-lesion resolution over time) and help discriminate from MS and AQP4+NMOSD, despite some overlap. The detection of MOG-IgG in the serum (and sometimes CSF) confirms the diagnosis in patients with compatible clinical-MRI phenotypes, but false positive results are occasionally encountered, especially with indiscriminate testing of large unselected populations. The type of cell-based assay used to evaluate for MOG-IgG (fixed vs. live) and antibody end-titer (low vs. high) can influence the likelihood of MOGAD diagnosis. International consensus diagnostic criteria for MOGAD are currently being compiled and will assist in clinical diagnosis and be useful for enrolment in clinical trials. Although randomized controlled trials are lacking, MOGAD acute attacks appear to be very responsive to high dose steroids and plasma exchange may be considered in refractory cases. Attack-prevention treatments also lack class-I data and empiric maintenance treatment is generally reserved for relapsing cases or patients with severe residual disability after the presenting attack. A variety of empiric steroid-sparing immunosuppressants can be considered and may be efficacious based on retrospective or prospective observational studies but prospective randomized placebo-controlled trials are needed to better guide treatment. In summary, this article will review our rapidly evolving understanding of MOGAD diagnosis and management.https://www.frontiersin.org/articles/10.3389/fneur.2022.885218/fullMOGNMOSDneuromyelitis opticamultiple sclerosisdemyelinating diseasesfalse positive |
| spellingShingle | Elia Sechi Laura Cacciaguerra Laura Cacciaguerra John J. Chen John J. Chen Sara Mariotto Giulia Fadda Alessandro Dinoto A. Sebastian Lopez-Chiriboga Sean J. Pittock Sean J. Pittock Eoin P. Flanagan Eoin P. Flanagan Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management Frontiers in Neurology MOG NMOSD neuromyelitis optica multiple sclerosis demyelinating diseases false positive |
| title | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management |
| title_full | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management |
| title_fullStr | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management |
| title_full_unstemmed | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management |
| title_short | Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease (MOGAD): A Review of Clinical and MRI Features, Diagnosis, and Management |
| title_sort | myelin oligodendrocyte glycoprotein antibody associated disease mogad a review of clinical and mri features diagnosis and management |
| topic | MOG NMOSD neuromyelitis optica multiple sclerosis demyelinating diseases false positive |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.885218/full |
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