Bilateral frontal cortex encephalitis and paraparesis in a patient with anti-MOG antibodies

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Myelin oligodendrocyte glycoprotein (MOG) is localized at the outermost layer of the myelin sheath and is accessible for autoantibodies. Although experimental autoimmune encephalitis induced with MOG immunization has been studied for 30 years, the results of previous reports on MOG immunoglobulin G (IgG) detection with enzyme-linked immunosorbent assay and Western blot are confusing. However, after the development of human MOG-transfected cell-based assay to detect conformational-sensitive MOG-IgG, unique groups of patients have been found seropositive, and MOG-IgG-associated disease has become a hot topic in clinical neuroimmunology. Currently, the clinical spectrum of MOG-IgG-associated disease includes idiopathic optic neuritis, acute disseminated encephalomyelitis, encephalitides (brainstem and cerebral cortical), idiopathic myelitis, atypical multiple sclerosis, aquaporin-4 IgG-negative neuromyelitis optica spectrum disorders and others. MOG-IgG-associated disease occurs in both children and adults, and the female:male ratio is almost 1:1. Pleocytosis in the cerebrospinal fluids during acute exacerbation is often seen, but oligoclonal IgG bands are usually negative. T helper cell 17, B cells and neutrophil-related cytokines appear to be upregulated intrathecally. The pathology of acute lesions is characterized by active inflammation demyelination with deposition of immunoglobulins and complements. Just like aquaporin 4 IgG-positive neuromyelitis optica spectrum disorders, some disease-modifying drugs for multiple sclerosis seem to be inefficacious in MOG-IgG-associated disease, and chronic immunosuppression is required to prevent relapse, especially in patients persistently positive for MOG-IgG. Our understanding of this newly recognized disease remains insufficient, and the progress of research is much expected.

Section: Clinical Phenotypes Of Mog‐igg‐associated Diseasementioning

confidence: 84%

Myelin oligodendrocyte glycoprotein immunoglobulin G‐associated disease: An overview

Fujihara

Sato

Nakashima

et al. 2018

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“…Wang et al 106 reported that 21% of MOG-IgG-positive patients presented with encephalitis, and one-third of these patients also showed anti-N-methyl d-aspartase receptor antibody. 105,107,108 Optic nerve lesions MRI abnormalities of the optic nerve have been found in 44-83% of patients. 105,107,108 Optic nerve lesions MRI abnormalities of the optic nerve have been found in 44-83% of patients.…”

Section: Brain Lesionsmentioning

confidence: 99%

Magnetic resonance imaging diagnosis of demyelinating diseases: An update

Miki

2019

Keywords acute disseminating encephalomyelitis (ADEM); demyelinating diseases; multiple sclerosis (MS); myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis; neuromyelitis optica spectrum disorder (NMOSD) Correspondence AbstractMajor demyelinating diseases include multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), acute disseminated encephalomyelitis (ADEM) and myelin oligodendrocyte glycoprotein (MOG) encephalomyelitis. As treatment strategies differ among these diseases, precise diagnosis of demyelinating diseases is crucial, and magnetic resonance imaging (MRI) plays a pivotal role in the diagnosis. In the present review, MRI appearances of characteristic lesions of these demyelinating diseases, and differentiation between MS and other demyelinating diseases based on MRI findings are discussed.

“…In contrast to ADEM/MDEM in children, there have been case reports of cerebral cortical encephalitis with MOG antibody in adults . The clinical features included generalized seizure, disturbance of consciousness, behavioral abnormalities and cortical focal signs, such as paraparesis seen in medial bifrontal cortical encephalitis.…”

Section: Mog Antibody‐associated Disseminated Demyelinating Diseasesmentioning

confidence: 99%

“…In contrast to ADEM/MDEM in children, there have been case reports of cerebral cortical encephalitis with MOG antibody in adults. 34,35 The clinical features included generalized seizure, disturbance of consciousness, behavioral abnormalities and cortical focal signs, such as paraparesis seen in medial bifrontal cortical encephalitis. The lesions distributions were characterized by either unilateral cerebral cortical or medial bifrontal cortical, and the lesions were best depicted as hyperintense areas by fluidattenuated inversion recovery images.…”

Section: Cerebral Cortical Encephalitis With Mog Antibodymentioning

confidence: 99%

Neuromyelitis optica spectrum and myelin oligodendrocyte glycoprotein antibody‐related disseminated encephalomyelitis

Misu

Fujihara

2018

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Neuromyelitis optica (NMO) is characterized by severe optic neuritis and transverse myelitis. The relationship of NMO to multiple sclerosis (MS) has long been debated. With the discovery of an NMO‐specific autoantibody to aquaporin 4 (AQP4), the clinical, radiological, and laboratory findings have clarified the differences between NMO and MS, and NMO spectrum disorders (NMOSD) have been proposed as the unifying term for the entire clinical entity including brain syndromes. Pathological studies in NMO showed loss of immunoreactivity to AQP4 and glial fibrillary acidic protein, but a relative preservation of myelin basic protein, especially at the lesions with perivascular deposition of immunoglobulins and complements. AQP4 antibody‐positive NMOSD is now considered an autoimmune astrocytopathic disease. In addition, the definite diagnosis should be made initially from the therapeutic viewpoint, because there have been several AQP4 antibody‐positive NMOSD cases exacerbated by disease‐modifying drugs for MS. In recent years, the antibody against myelin oligodendrocyte glycoprotein (MOG) has been studied for its association with other types of acute demyelinating diseases, such as acute or multiphasic disseminated encephalomyelitis, optic neuritis, NMOSD and brainstem or cerebral cortical encephalomyelitis. Recent brain biopsied MOG antibody‐positive case reports have suggested the dominance of humoral immunity, but it is not well elucidated whether the cellular immune responses against MOG could develop perivenous inflammatory demyelination like classical acute disseminated encephalomyelitis pathology. In the present review, we focus on two distinct diseases, aquaporin 4 antibody‐related NMOSD and MOG antibody‐related diseases, both of which were recently differentiated from MS by means of the disease‐specific autoantibodies and the distinct pathophysiologies.