Neuromyelitis optica spectrum disorder (NMOSD): although in many ways similar, a number of features are helpful in distinguishing individuals with aquaporin-4-IgG-positive NMOSD from those with MOGAD It remains unclear what, if any, long-term medications are required in individuals with a relapsing time course, although it seems that response to immunotherapeutic agents is different to multiple sclerosis and that some agents efficacious in the latter may actually worsen anti-MOG related diseases 2. Treatment and prognosisĪlthough treatment and prognosis remain to be fully understood, it appears that generally, individuals with anti-MOG antibodies have fewer relapses and less severe clinical course than individuals with anti-aquaporin 4 antibodies presenting with NMOSD 2.Īcute management with intravenous methylprednisolone, plasma exchange, intravenous immunoglobulin or cyclophosphamide have been reported and appear to be efficacious 2. A helpful distinguishing feature is the presence of leptomeningeal enhancement, relatively common in MOGAD is rarely seen in multiple sclerosis and NMOSD 6. The lower cord is often preferentially affected, with the conus medullaris being classically affected 6. The involvement of grey matter forms the "H" sign on axial view and thin linear T2 hyperintense signals on sagittal view 8. Spinal involvement usually is central affecting both grey matter and central white matter and can be both lengthy ( longitudinally extensive spinal cord lesions) or short segment, and both patterns may be encountered simultaneously 6. There is periorbital enhancement as well 8. Involvement of the optic chiasm and optic tracts is uncommon 6. The optic nerves are involved in the majority of patients with MOGAD at the time of presentation (>80%) typically bilateral, involving the anterior parts, with prominently swollen edematous nerves resulting in tortuosity and optic disc swelling 6. Leptomeningeal enhancement is uncommon but encountered either in isolation or along with cortical lesions (e.g. Deep grey matter and brainstem involvement is more common in children 6. When present, lesions tend to be few in number but sizable, typically bilateral with ill-defined borders. Cerebral involvementĪcute disseminated encephalomyelitis (ADEM) is a common presenting clinical and imaging phenotype of patients with MOGAD, particularly in childhood 6. In contrast, less than half of adults presenting with MOGAD have brain lesions at the time of diagnosis. Nonetheless, certain imaging features are increasingly recognized as being more typical of MOGAD, allowing the diagnosis to be suspected on the basis of imaging. acute disseminated encephalomyelitis (ADEM), neuromyelitis optica spectrum disorder (NMOSD) and to a lesser degree multiple sclerosis). Imaging presentation of MOGAD is variable and with no pathognomonic imaging features, with patients having an imaging phenotype often indistinguishable from other inflammatory white matter diseases of the central nervous system (e.g. Myelin oligodendrocyte glycoprotein (MOG) is expressed on oligodendrocytes and the outer lamellae of myelin sheaths 6. 2022) no single set of diagnostic criteria are universally accepted 5. No specific presentation distinguishes individuals with anti-MOG antibodies from those presenting with similar clinical manifestation but without the antibodies and at the time of writing (c. Transverse myelitis (30%) including conus medullaris syndromeĪssociated with longitudinally extensive spinal cord lesionsĪssociated with FLAIR-hyperintense lesions in anti-MOG associated encephalitis with seizures (FLAMES) 7 This may encompass cases previously termed chronic relapsing inflammatory optic neuropathy (CRION) Not all presentations are equally prevalent: In approximately half of cases there is viral prodrome 2. Clinical presentationĬlinical presentation is similar to that of other acquired demyelinating conditions and varies from individual to individual. In children with acquired demyelination syndrome, MOG-IgGs are more commonly detected than aquaporin 4 antibodies 5.Īdditionally, children with MOGAD are more likely to present with an ADEM-like clinical picture, whereas adults are more likely to present with an NMO-like syndrome 5. MOGAD is primarily encountered in children and young adults 1. As research was undergoing into MOGAD, many alternate terms were used in the literature, including MOG-IgG-associated optic neuritis, encephalitis, and myelitis (MONEM), anti-MOG associated encephalomyelitis, anti-MOG encephalitis and other variations on this theme 1-4.
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