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. Symptoms of MOGAD may include vision loss, muscle weakness, stiffness or paralysis, confusion, seizures, myelin oligodendrocyte glycoprotein, and headaches. These symptoms can be sometimes myelin oligodendrocyte glycoprotein with other diseases such as multiple sclerosis.

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.

Myelin oligodendrocyte glycoprotein

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. As a member of the immunoglobulin superfamily, MOG is highly immunogenic [ 2 ]. The function of MOG has not been fully elucidated, but it may act as a cell adhesion molecule, regulate microtubule stability, and modulate myelin immune interactions [ 2 ]. These induce demyelination in experimental autoimmune encephalomyelitis animal models immunized with MOG [ 3 ].

Relapses can have long-term effects on the central nervous system, including gait and vision challenges. Idiopathic inflammatory-demyelinating diseases of the central nervous system.

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.

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. MOGAD causes painful swelling, known as inflammation. Symptoms are caused by attacks from:.

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. 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.

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Infrequently, seizures may lead to status epilepticus and the need for temporary mechanical ventilation [ 48,49 ]. Myelin oligodendrocyte glycoprotein antibodies in neurological disease. Notably, an examination of serum samples obtained from out of the ONTT participants revealed that only 3 1. Characteristic attacks — While none of the clinical features of MOGAD are disease-specific, some are highly characteristic. Acute myelitis may be associated with LETM on MRI lesions that extend over 3 contiguous segments or 3 contiguous segments of focal spinal cord atrophy. NMDA antibodies have been shown to coexist on occasion with MOG antibodies, and patients can manifest with combinations of features from both disorders, either concurrently or sequentially. See "Differential diagnosis of acute central nervous system demyelination in children", section on 'Distinguishing ADEM and multiple sclerosis'. The first attack is usually the worst, but each attack can cause more damage. In partnership with Amit Bar-Or, MD, Director of the NeuroImmune Program at the Hospital of the University of Pennsylvania, we provide an age-span program that offers novel precision neuroimmunology discovery research. MOGAD lesions are often bilateral poorly defined and appear large with deep gray matter involvement. Limited data suggest that bilateral cerebral cortical encephalitis in children is associated with more fulminant presentations, including critical illness, severe encephalopathy, seizures, and worse outcomes [ 47 ]. The majority up to 80 percent are unilateral with symptoms referable to one hemisphere and are accompanied radiologically by cortical swelling, T2 hyperintensity, and leptomeningeal enhancement see 'MRI brain' below. Overlapping demyelinating syndromes and anti—N-methyl-D-aspartate receptor encephalitis. T2 hypointensity in the adjacent white matter has also been reported [ 45 ]. This positive predictive value is dependent on the test-ordering practices and disease prevalence.

Skip to content. What is myelin oligodendrocyte glycoprotein antibody disease?

Am J Neuroradiol. The differential diagnosis for uveitis is broad, and many cases remain idiopathic. Instead, these patients may present with altered consciousness, seizures, or a brainstem syndrome. While persistent MOG-IgG seropositivity is associated with an increased risk of relapse, it is difficult to tailor treatment to MOG-IgG titers as patients may remain seropositive while relapse-free for years. Attack recovery can take weeks to months. Some of them are not-inflammatory, such as adrenoleukodystrophy , vanishing white matter disease , and Rubella induced mental retardation. This differs from AQP4-IgG, in which, when using cell-based assays, false positives are extremely rare [ 85 ]. In some cases, the enhancement involves the optic nerve sheath in isolation image 2 termed optic perineuritis [ 68 ] or can extend into the orbital fatty tissues image 2 [ 19 ]. Banwell B, et al. Next Steps Contact Us. Mult Scler ;