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 in the optic nerves, brain and spinal cord, 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.

Myelin oligodendrocyte glycoprotein

Skip to content. What is myelin oligodendrocyte glycoprotein antibody disease? Myelin oligodendrocyte glycoprotein MOG is a protein found on the covering of nerves in the central nervous system. While the precise function of MOG is not fully understood, it likely plays a role in myelin maturation, myelin integrity, and cell surface interactions. MOG antibody disease affects males and females almost equally and are more prevalent in children than adults. Patients who show the symptoms listed above will undergo a serum blood test. MRIs of the brain, orbits and spine may also reveal lesions or white matter changes suggestive of an inflammatory process. As appropriate, our Neuroimmune specialists will also rule out other conditions that may look similar to MOG antibody disease. At the Neuroscience Center , our goal is to stop symptoms and restore a child's normal growth and development with as few treatment-related side effects as possible. Most patients respond well to intravenous steroids, with some also requiring oral prednisone for one to four weeks. Complete recovery from the onset attack is common in children, and in general the outcome is favorable. However, patients who experience more severe attacks and relapsing MOGAD are at risk for more severe deficits. Relapses can occur. Relapses can have long-term effects on the central nervous system, including gait and vision challenges.

Investigations — The evaluation of suspected MOGAD on initial presentation entails the following imaging and laboratory studies:.

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 ]. Subsequently, newer generation cell-based assays were developed, and the MOGAD phenotype became clearer, with patients now recognized to have episodes of optic neuritis, ADEM, transverse myelitis, or other central nervous system CNS manifestations, either alone or in combination. Overall, the pathologic features support an antibody-mediated central nervous system CNS demyelinating disease distinct from multiple sclerosis MS.

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. MOG is a molecule detected on the outer membrane of myelin sheaths and expressed primarily within the brain, spinal cord and also the optic nerves. Its function is not fully understood but this glycoprotein may act as a cell surface receptor or cell adhesion molecule. The specific outmost location of myelin makes it a potential target for autoimmune antibodies and cell-mediated responses in demyelinating processes. Optic neuritis seems to be the most frequent presenting phenotype in adults and ADEM in children. In adults, the disease course is multiphasic and subsequent relapses increase disability.

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 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.

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See 'Cerebrospinal fluid' above. Orthologs Species Human. Chromosome 17 mouse [2]. Multiple studies have shown that maintenance therapy with oral corticosteroids has been associated with a reduction in relapse rate [ 64 , 82 , 83 ]. This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. When bilateral it can be mistaken for papilledema [ 19 ]. During attacks, CSF may show evidence of a pleocytosis particularly in patients with myelitis, brainstem, and multifocal relapses, more so than optic neuritis events. Most patients respond well to intravenous steroids, with some also requiring oral prednisone for one to four weeks. The detection of optic nerve lesions relies upon dedicated orbital imaging, with fat suppressed T2 images and gadolinium enhanced T1W views or short tau inversion recovery sequences. Instead, these patients may present with altered consciousness, seizures, or a brainstem syndrome. The role of optical coherence tomography in the diagnosis of afferent visual pathway problems: A neuroophthalmic perspective. Am J Neuroradiol. Mult Scler J. Optic neuritis and autoimmune optic neuropathies: advances in diagnosis and treatment.

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.

See a doctor or other healthcare professional if you experience any of the above symptoms for unknown reasons. Sufficiently powered prospective studies with extended follow-up would enable a more comprehensive understanding of the natural history of MOGAD, identify predictors of its course and establish guidelines for its treatment. In MOGAD, enhancement of the optic nerve most often involves the anterior optic nerve pathway extending up to the fundus image 2 , which may explain the frequent optic disc edema accompanying it [ 19 ]; isolated involvement of the chiasm is more suggestive of aquaporinIgG seropositive neuromyelitis optica spectrum disorder AQP4-IgG NMOSD [ 67 ]. MOGAD lesions are often bilateral poorly defined and appear large with deep gray matter involvement. The symptoms include the subacute onset of paraparesis or quadriparesis with loss of sensation below the lesion and a sensory level across the trunk [ 31 ]. Cases of bilateral cerebral cortical encephalitis are also described but are less common [ 46,47 ]. Recurrent or isolated attacks of ON, myelitis, ADEM, cerebral monofocal or multifocal deficits, brainstem or cerebellar deficits, cerebral cortical encephalitis often with seizures. J Neuroinflammation Comparison of inflammatory demyelinating diseases of the central nervous system. Tools Tools. N Engl J Med. J Neurovirol. Eur J Paediatr Neurol ; Acta Neurol Scand. The latter is characterized by any combination of transverse myelitis and optic neuritis, sometimes with other CNS regions involved.

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