Explore the topics of neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein-associated disease (MOGAD). Learn about the historical background and classification of neuromyelitis optica, the clinical features and diagnostic criteria of Devic syndrome, treatment options for NMOSD and MOGAD, an overview of NMOSD and MOG-associated disorders, disease-modifying therapies for myelin-oligodendrocyte glycoprotein-associated disease, and a comparison of MOGAD, NMOSD, and MS.
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Quick takeaways
NMO is characterized by severe optic neuritis, poor visual recovery, and longitudinally extensive transverse myelitis, while MOGAD presents with bilateral optic neuritis, more severe myelitis, and potential leukoencephalopathy involvement.
Treatment options for NMO include high-dose IV glucocorticoids, plasma exchange, and disease-modifying therapies, while treatment options for MOGAD are less well-established but may include glucocorticoids, plasma exchange, and various immunosuppressive agents.
Deep dives
Overview of Neuromyelitis Optic Spectrum Disorders (NMO)
Neuromyelitis optic spectrum disorders (NMO) is a subset of inflammatory optic neuropathies characterized by optic neuritis and myelitis syndrome. The Aquaporin-4 (AQP4) protein plays a key role in the pathophysiology of NMO. The diagnosis is based on clinical, radiographic, and serologic findings, with a positive serum test for AQP4-IgG antibodies being crucial. NMO is more common in individuals of African and Afro-Caribbean ancestry, and the age of onset is typically around 40 years. Optic neuritis in NMO is usually severe and bilateral, with poor visual recovery. Longitudinally extensive transverse myelitis is another common feature seen in NMO. Treatment involves high-dose IV glucocorticoids, plasma exchange, and disease-modifying therapies like Eculizumab, Inebelizumab, and Satralizumab.
Overview of Myelin Oligodendrocyte Glycoprotein-Associated Disease (MOGAD)
Myelin oligodendrocyte glycoprotein-associated disease (MOGAD) is a newer condition characterized by antibodies targeting MOG proteins found on myelin sheaths and oligodendrocytes. MOGAD typically presents with optic neuritis, myelitis, and brain involvement, often resembling acute disseminated encephalomyelitis (ADEM). The disease course of MOGAD can be either monophasic or relapsing. Diagnostic criteria include clinical features and positive serum MOG antibody titers determined via cell-based assays. Treatment options for MOGAD are not well-established, but options can include glucocorticoids, plasma exchange, and immunosuppressive agents like azathioprine, mycophenolate, or rituximab.
Comparing and Contrasting NMO and MOGAD
Distinguishing features of Neuromyelitis optica spectrum disorders (NMO) include severe optic neuritis with poor visual recovery, longitudinally extensive transverse myelitis, and absence of significant cortical or white matter findings. Myelin oligodendrocyte glycoprotein-associated disease (MOGAD) is characterized by bilateral, longitudinally extensive optic neuritis with better visual recovery, more severe myelitis, presence of acute disseminated encephalomyelitis (ADEM) in children, and potential leukoencephalopathy involvement. NMO is more closely associated with AQP4-IgG antibodies, while MOGAD is associated with MOG antibodies. Treatment options for NMO include high-dose IV glucocorticoids, plasma exchange, and disease-modifying therapies such as Eculizumab, Inebelizumab, or Satralizumab. MOGAD treatment options are less well-established but may include glucocorticoids, plasma exchange, and various immunosuppressive agents.
A review of neuromyelitis optic spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein associated disease (MOGAD) with Drs. Kevin Yan and Aaron Bower.
Note: This podcast is intended solely as an educational tool for learners, especially neurology residents. The contents should not be interpreted as medical advice.
Further Reading:
Costello F. Neuromyelitis Optica Spectrum Disorders. Continuum (Minneap Minn). 2022 Aug 1;28(4):1131-1170. doi: 10.1212/CON.0000000000001168. Erratum in: Continuum (Minneap Minn). 2022 Dec 1;28(6):1859. PMID: 35938660.
Longbrake E. Myelin Oligodendrocyte Glycoprotein-Associated Disorders. Continuum (Minneap Minn). 2022 Aug 1;28(4):1171-1193. doi: 10.1212/CON.0000000000001127. PMID: 35938661; PMCID: PMC9523511.
Banwell B, Bennett JL, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. Lancet Neurol. 2023 Mar;22(3):268-282. doi: 10.1016/S1474-4422(22)00431-8. Epub 2023 Jan 24. PMID: 36706773.
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