Myelin oligodendrocyte glycoprotein immunoglobulin G‐associated disease: An overview

Abstract

Myelin oligodendrocyte glycoprotein (MOG) is localized at the outermost layer of the myelin sheath and is accessible for autoantibodies. Although experimental autoimmune encephalitis induced with MOG immunization has been studied for 30 years, the results of previous reports on MOG immunoglobulin G (IgG) detection with enzyme-linked immunosorbent assay and Western blot are confusing. However, after the development of human MOG-transfected cell-based assay to detect conformational-sensitive MOG-IgG, unique groups of patients have been found seropositive, and MOG-IgG-associated disease has become a hot topic in clinical neuroimmunology. Currently, the clinical spectrum of MOG-IgG-associated disease includes idiopathic optic neuritis, acute disseminated encephalomyelitis, encephalitides (brainstem and cerebral cortical), idiopathic myelitis, atypical multiple sclerosis, aquaporin-4 IgG-negative neuromyelitis optica spectrum disorders and others. MOG-IgG-associated 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. T helper cell 17, B cells and neutrophil-related cytokines appear to be upregulated intrathecally. The pathology of acute lesions is characterized by active inflammation demyelination with deposition of immunoglobulins and complements. Just like aquaporin 4 IgG-positive neuromyelitis optica spectrum disorders, some disease-modifying drugs for multiple sclerosis seem to be inefficacious in MOG-IgG-associated disease, and chronic immunosuppression is required to prevent relapse, especially in patients persistently positive for MOG-IgG. Our understanding of this newly recognized disease remains insufficient, and the progress of research is much expected.

Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology

“…MOG-IgG is found in a subset of patients with optic neuritis, acute disseminated encephalomyelitis/multiphasic disseminated encephalomyelitis, myelitis, AQP4-IgG–seronegative NMOSD, or encephalitis. 49 Although MOG-IgG–seropositive and AQP4-IgG–seropositive NMOSD share a similar serum and CSF cytokine profile characterized by the coordinated upregulation of multiple cytokines, especially Th17-related, these diseases have different pathologies. 50 AQP4-IgG–seropositive NMOSD is primarily an astrocytopathic disease, whereas MOG-IgG–seropositive NMOSD is a demyelinating disease.…”

Perivenous demyelination: Association with anti‐myelin oligodendrocyte glycoprotein antibody

“…After the discovery of conformation‐sensitive MOG antibody by human MOG‐transfected cell‐based assay, we could identify the specific features of some MOG antibody–positive cases and could consider the therapeutic strategies (Table 2). 6,16,17 We can identify several phenotypes such as ON (recurrent, bilateral), ADEM, MDEM, transverse myelitis, atypical MS, brainstem encephalitis, cortical encephalitis with seizures, and NMOSD 7,8,18‐20 . In addition, there are several cases of recurrent MOG antibody–positive ADEM or autoimmune encephalitis with another autoantibodies such as NMDA receptor antibody, 21 suggesting the existence of combined diseases with disease‐specific antibodies.…”

Case Report: Anti-MOG Antibody Seroconversion Accompanied by Dimethyl Fumarate Treatment

“…Although therapy for MOGAD is yet to be established, according to consensus among international experts ( 6 , 7 ), immunosuppressive therapy (e.g., corticosteroids, azathioprine, tacrolimus, mycophenolate mofetil, and methotrexate) is the mainstay for treatment to prevent relapse. In particular, B cell depleting therapies such as rituximab have shown good therapeutic responses, but relapse occurs immediately after B cell recovery.…”

“…These data strongly suggest that suppression of humoral immunity is likely the key therapeutic strategy for MOGAD. DMDs for MS such as interferon-β, glatiramer acetate, and natalizumab might not be efficacious, but the effectiveness of fingolimod remains uncertain ( 6 , 7 ). Although a recent report mentioned that DMF is ineffective but not harmful in a patient with MOGAD ( 8 ), the effectiveness of DMF has yet to be determined.…”