Contributions
Abstract: 279
Type: Educational Session
Abstract Category: N/A
Myelin oligodendrocyte glycoprotein (MOG) is localized in the extracellular surface of oligodendrocytes and the outermost lamellae of myelin sheath and thus accessible for autoantibodies. Experimental autoimmune encephalomyelitis induced with MOG peptide immunization has been studies for more than 30 years and are known to mimic human NMO to some extent.
The findings in previous studies on MOG-antibody detected with ELISA and Western blot were confusing due to low specificity. However, following the development of human MOG-transfected cell-based assay to detect conformational-sensitive MOG-antibody, unique groups of patients have been found seropositive for MOG-antibody. At this point, a combination of full-length human MOG-transfected cells and secondary antibody specific to Fc or IgG1 is relatively good to detect unique patient groups. But false-positive and false-negative results can occur, and further efforts should be made to establish a highly-reliable standard assay for MOG-antibody.
The clinical spectrum of MOG-IgG-associated disease includes idiopathic optic neuritis (ON), acute disseminated encephalomyelitis (ADEM)/multiphasic demyelinating encephalomyelitis (MDEM), encephalitides (brainstem and cerebral cortical), idiopathic myelitis, atypical multiple sclerosis (MS), aquaporin 4 (AQP4)-antibody-negative neuromyelitis optica spectrum disorders (NMOSD) and others. MOG-antibody 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. Th17 (IL-6, etc), B cell and neutrophil-related cytokines are upregulated intrathecally. Although acute lesions in a small number of brain-biopsied cases with MOG-antibody disease has been reported to be characterized by active inflammation demyelination with deposition of immunoglobulins and complements, more extensive analyses including cellular immunity are needed to elucidate the neuropathological features. As in AQP4-antibody-positive NMOSD, some disease modifying drugs for MS appear to be inefficacious in MOG-antibody disease and chronic immunosuppression is needed to prevent relapse, especially in patients persistently positive for MOG-antibody.
In this presentation, updated information of the newly recognized inflammatory demyelinating disease and the features distinct from those in MS and AQP4-antibody positive NMOSD will be presented.
Disclosure: Kazuo Fujihara: KF serves on the scientific advisory boards for Bayer, Biogen, Mitsubishi Tanabe, Novartis, Chugai, Alexion, and MedImmune; has received funding for speaker honoraria and/or research support from Bayer, Biogen, Eisai, Mitsubishi Tanabe, Novartis, Astellas, Takeda, Asahi Kasei Medical, Chemo-Sero-Therapeutic Research Institute, Teva, Teijin, Chugai, Ono, and Genzyme; is funded by KAKENHI from the Ministry of Education, Science and Technology of Japan and by the Grants-in-Aid for Scientific Research from the Ministry of Health, Welfare and Labor of Japan
Abstract: 279
Type: Educational Session
Abstract Category: N/A
Myelin oligodendrocyte glycoprotein (MOG) is localized in the extracellular surface of oligodendrocytes and the outermost lamellae of myelin sheath and thus accessible for autoantibodies. Experimental autoimmune encephalomyelitis induced with MOG peptide immunization has been studies for more than 30 years and are known to mimic human NMO to some extent.
The findings in previous studies on MOG-antibody detected with ELISA and Western blot were confusing due to low specificity. However, following the development of human MOG-transfected cell-based assay to detect conformational-sensitive MOG-antibody, unique groups of patients have been found seropositive for MOG-antibody. At this point, a combination of full-length human MOG-transfected cells and secondary antibody specific to Fc or IgG1 is relatively good to detect unique patient groups. But false-positive and false-negative results can occur, and further efforts should be made to establish a highly-reliable standard assay for MOG-antibody.
The clinical spectrum of MOG-IgG-associated disease includes idiopathic optic neuritis (ON), acute disseminated encephalomyelitis (ADEM)/multiphasic demyelinating encephalomyelitis (MDEM), encephalitides (brainstem and cerebral cortical), idiopathic myelitis, atypical multiple sclerosis (MS), aquaporin 4 (AQP4)-antibody-negative neuromyelitis optica spectrum disorders (NMOSD) and others. MOG-antibody 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. Th17 (IL-6, etc), B cell and neutrophil-related cytokines are upregulated intrathecally. Although acute lesions in a small number of brain-biopsied cases with MOG-antibody disease has been reported to be characterized by active inflammation demyelination with deposition of immunoglobulins and complements, more extensive analyses including cellular immunity are needed to elucidate the neuropathological features. As in AQP4-antibody-positive NMOSD, some disease modifying drugs for MS appear to be inefficacious in MOG-antibody disease and chronic immunosuppression is needed to prevent relapse, especially in patients persistently positive for MOG-antibody.
In this presentation, updated information of the newly recognized inflammatory demyelinating disease and the features distinct from those in MS and AQP4-antibody positive NMOSD will be presented.
Disclosure: Kazuo Fujihara: KF serves on the scientific advisory boards for Bayer, Biogen, Mitsubishi Tanabe, Novartis, Chugai, Alexion, and MedImmune; has received funding for speaker honoraria and/or research support from Bayer, Biogen, Eisai, Mitsubishi Tanabe, Novartis, Astellas, Takeda, Asahi Kasei Medical, Chemo-Sero-Therapeutic Research Institute, Teva, Teijin, Chugai, Ono, and Genzyme; is funded by KAKENHI from the Ministry of Education, Science and Technology of Japan and by the Grants-in-Aid for Scientific Research from the Ministry of Health, Welfare and Labor of Japan