Myelin oligodendrocyte glycoprotein

Myelin oligodendrocyte glycoprotein antibody-associated disease, also known as MOGAD, is a rare inflammatory disease that affects the central nervous system. In MOGADthe immune system attacks the fatty substance that protects nerve fibers myelin oligodendrocyte glycoprotein the optic nerves, myelin oligodendrocyte glycoprotein, brain and spinal cord. Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, and headaches. These symptoms can be sometimes confused with other diseases such as multiple sclerosis.

Contributor Disclosures. Please read the Disclaimer at the end of this page. The disease has a predilection for children. Treatment and prognosis are reviewed separately. In a study using MOG self-antigen tetramers detected by a radioimmunoassay technique, MOG antibodies were detected in a subset of patients with acute disseminated encephalomyelitis ADEM but rarely in adult patients with MS [ 1 ].

Myelin oligodendrocyte glycoprotein

Federal government websites often end in. The site is secure. Data Availaiblity statement is not applicable as this review article is based exclusively on published work. New diagnostic criteria for myelin oligodendrocyte glycoprotein antibody-associated disease MOGAD have recently been proposed, distinguishing this syndrome from other inflammatory diseases of the central nervous system. Seropositivity status for MOG-IgG autoantibodies is important for diagnosing MOGAD, but only in the context of robust clinical characterization and cautious interpretation of neuroimaging. For this reason, possible alternative diagnoses need to be considered, and low MOG-IgG titers need to be carefully weighted. Key challenges to the current understanding of MOGAD are also highlighted, including uncertainty regarding the specificity and pathogenicity of MOG autoantibodies, the need to identify immunopathologic targets for future therapies, the quest to validate biomarkers that facilitate diagnosis and detect disease activity, and the importance of deciphering which patients with MOGAD require long-term immunotherapy. Myelin oligodendrocyte glycoprotein antibody-associated disease MOGAD is a relatively new addition to the category of central nervous system CNS inflammatory demyelinating diseases [ 1 , 2 ]. CNS inflammatory demyelinating conditions, including multiple sclerosis MS and neuromyelitis optica spectrum disorders NMOSD , are differentiated based on severity, clinical phenotype, imaging, laboratory, and pathological findings [ 2 ] Table 1. The current estimated range of incidence in the pediatric population is 3. Notably, these numbers, along with the estimated worldwide prevalence of 20 million [ 5 ], are likely to increase with growing recognition of the disease and improved availability of serological testing. Yet, disease manifestations may occur after prodromal infections, particularly those caused by viral pathogens, such as influenza, Epstein—Barr virus, herpes simplex virus, and severe acute respiratory syndrome coronavirus 2 SARS-CoV-2 , to name a few [ 3 ]. Occasionally, patients with MOGAD have an overlap syndrome with anti-NMDA receptor encephalitis, characterized by clinical features of demyelination associated with encephalopathy, seizures, dyskinesias, or psychosis [ 5 ]. As the clinical spectrum of MOGAD continues to expand, so too does our appreciation for diagnostic and management challenges associated with this enigmatic condition. Key areas of ongoing research include determining the specificity and pathogenicity of MOG autoantibodies, identifying immunopathologic targets for future therapies, discovering and validating biomarkers that detect disease activity, and deciphering which patients with MOGAD require long-term immunotherapy.

Mechanisms of raised intracranial pressure include inflammation, cerebral venous sinus thrombosis, mass effect or coincidental IIH, myelin oligodendrocyte glycoprotein. Relevance of antibodies to myelin oligodendrocyte glycoprotein in CSF of seronegative cases. Remission of myasthenia gravis following plasma-exchange.

Myelin oligodendrocyte glycoprotein MOG is a glycoprotein believed to be important in the myelination of nerves in the central nervous system CNS. In humans this protein is encoded by the MOG gene. MOG's cDNA coding region in humans have been shown to be "highly homologous" [9] to rats, mice, and bovine, and hence highly conserved. This suggests "an important biological role for this protein". The gene for MOG, found on chromosome 6 p The crystal structure of myelin oligodendrocyte glycoprotein was determined by x-ray diffraction at a resolution of 1.

Myelin oligodendrocyte glycoprotein antibody-associated disease, also known as MOGAD, is a rare inflammatory disease that affects the central nervous system. In MOGAD , the immune system attacks the fatty substance that protects nerve fibers in the optic nerves, brain and spinal cord. Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, and headaches. These symptoms can be sometimes confused with other diseases such as multiple sclerosis. However, there are treatments to help speed the recovery from attacks, manage symptoms and reduce the likelihood of symptoms returning.

Myelin oligodendrocyte glycoprotein

Federal government websites often end in. The site is secure. Myelin oligodendrocyte glycoprotein MOG -associated disease MOGAD is a rare, antibody-mediated inflammatory demyelinating disorder of the central nervous system CNS with various phenotypes starting from optic neuritis, via transverse myelitis to acute demyelinating encephalomyelitis ADEM and cortical encephalitis. Even though sometimes the clinical picture of this condition is similar to the presentation of neuromyelitis optica spectrum disorder NMOSD , most experts consider MOGAD as a distinct entity with different immune system pathology.

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In some children, a progressive worsening accompanied by a leukodystrophy-like MRI pattern has been described after one or more ADEM episodes [ 35,59 ]. Formulary drug information for this topic. Corresponding author. MOGAD lesions are often bilateral poorly defined and appear large with deep gray matter involvement. The New England Journal of Medicine. JAMA Neurol ; International Business Collaborations. Thus, in patients with features suggestive of and anti-NMDA receptor encephalitis orofacial dyskinesias, psychosis, seizures , testing cerebrospinal fluid for NMDA receptor antibodies should be considered [ 51,52 ]. The structure and function of myelin oligodendrocyte glycoprotein. Other causes of OMS including tumors, paraneoplastic conditions, and autoimmune processes — all of which need to be excluded. In contrast to MS, a burden of asymptomatic spinal cord lesions is relatively uncommon. Optical coherence tomography — Optical coherence tomography OCT is an imaging tool that can provide quantitative information of the size of the various layers of the retina using near infrared light. Part 2: Results from lumbar punctures in 80 pediatric patients. Neurol - Neuroimmunol Neuroinflammation 9:e

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Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Treatment of myelin oligodendrocyte glycoprotein immunoglobulin G—associated disease. Symptoms are caused by attacks from:. Features include rapid multidirectional conjugate eye movement, ataxia, and myoclonus. The disease has a predilection for children. J Ophthalmol. Overlapping demyelinating syndromes and anti—N-methyl-D-aspartate receptor encephalitis. Neurol—Neuroimmunol Neuroinflammation 9:e MRI changes accompanying the unilateral cortical encephalitis phenotype of myelin oligodendrocyte glycoprotein antibody-associated disease MOGAD. Examples of mono-focal or multifocal deficits may be accompanied by MRI T2 hyper-intense lesions in the middle cerebellar peduncle, around the fourth ventricle, supratentorial white matter, cortical juxtacortical regions, and deep gray nuclei. Furthermore, findings by Cobo-Calvo et al. Investigations — The evaluation of suspected MOGAD on initial presentation entails the following imaging and laboratory studies:. See 'MRI orbits' above. Relapses can occur.