2023 Volume 40 Issue 4 Pages 606-609
Myelin oligodendrocyte glycoprotein (MOG) antibody associated disease (MOGAD) is an inflammatory demyelinating disease of the central nervous system. The disease entity encompasses a group of disorders in which autoantibodies (MOG antibodies) are directed against MOG, one of the myelin sheath proteins of the central nervous system, and which present with a variety of symptoms, including optic neuritis, myelitis, acute disseminated encephalomyelitis (ADEM), brainstem encephalitis and cerebral cortical encephalitis. MOG antibodies are thought to be involved in the pathogenesis of these diseases through autoimmune mechanisms, and the same genetic predisposition is expected as in other autoantibody associated diseases. The sensitivity and specificity of MOG antibodies have increased with the development of assay technology and the use of cell–based assays. Although patient serum is commonly used to measure MOG antibodies, MOG antibodies can be positive in cerebrospinal fluid alone.
The pathogenesis of MOGAD is variable, with some MOGAD patients having a monophasic course without relapse. Patients presenting with attacks of optic neuritis or transverse myelitis respond better to acute treatment than patients with aquaporin–4 antibody–positive neuromyelitis optica spectrum disorder, often recovering to their original level of ADL. In children, ADEM is more common and often associated with seizures. Other forms of encephalomyelitis, such as brainstem encephalitis and cortical encephalitis, are less common. Some are very refractory, with recurrent inflammatory attacks and brain atrophy. Retrospective studies have shown that patients with myelitis tend to have a monophasic course. MOG antibody positive–to–negative seroconversion has been reported to be a significant predictor of a monophasic course in children, but it does not necessarily correlate with a monophasic course in adult cases.
Treatment of MOGAD includes steroid pulses in the acute attack phase, as in other CNS inflammatory diseases. There is no drug that is approved in Japan for the prevention of relapses. This review discusses the pathogenesis and treatment of MOGAD.
