Myelin oligodendrocyte glycoprotein (MOG) is a minor myelin protein localized at the outermost layer of the myelin sheath. Cell-based assays to detect conformation-sensitive MOG-immunoglobulin G (IgG) have identified a unique group of patients with optic neuritis, acute/multiphasic disseminated encephalomyelitis, encephalitides (brainstem and cerebral cortical), myelitis and aquaporin-4 IgG-negative neuromyelitis optica spectrum disorders. MOG-IgG-associated disease affects both children and adults, and the female : male ratio is almost 1:1. Cerebrospinal fluid (CSF) examination shows pleocytosis of mononuclear or polymorphonuclear cells during acute exacerbation, but oligoclonal IgG bands are usually negative. CSF-myelin basic protein levels are high, but CSF-glial fibrillary acidic protein is not elevated, suggesting demyelination. CSF-cytokines related to T helper cell-17, B cells and neutrophils are remarkably upregulated, which is similar to aquaporin-4 IgG-positive neuromyelitis optica spectrum disorders, but distinct from multiple sclerosis. As for treatment of MOG-IgG-associated disease, high-dose intravenous methylprednisolone is commonly used in the acute phase, but it has not been established which patients require long-term immunosuppression and which drugs should be chosen to prevent relapse. There have been some recent studies to support the pathogenicity of MOG-IgG in vitro and in vivo. Taken together, MOG-IgG-associated disease seems to be a unique inflammatory demyelinating disease of the central nervous system, but further research is required to clarify the clinical and epidemiological features, treatment strategy, and the underlying pathology.
- acute disseminated encephalomyelitis
- myelin oligodendrocyte glycoprotein
- neuromyelitis optica spectrum disorder
ASJC Scopus subject areas
- Neuroscience (miscellaneous)
- Immunology and Microbiology (miscellaneous)
- Clinical Neurology