Clinical spectrum of inflammatory central nervous system demyelinating disorders associated with antibodies against myelin oligodendrocyte glycoprotein.

Immunoglobulin G (IgG) antibodies against myelin oligodendrocyte glycoprotein (MOG) are detected in the serum of some patients with demyelinating diseases. These patients are known to show repeated clinical episodes of inflammatory demyelinating attacks in the central nervous system. Although the associated pathogenicity and mechanism of inflammatory demyelination remains inconclusive, it is known that patients with MOG-IgG antibodies have a different clinical spectrum from those with other demyelinating diseases, such as multiple sclerosis. Based on our database of 85 MOG-IgG positive (+) cases, the most frequently associated clinical episodes were isolated optic neuritis (67.5%), encephalitis (26.5%), and myelitis (19.3%). Optic neuritis in MOG-IgG (+) disease usually involves the long segment of optic nerves and sometimes happens bilaterally, but visual acuity usually recovers with proper treatment in the acute phase. Brain and brainstem lesions usually present vague and focal appearances with irregular margins, typically in subcortical or brainstem regions, but occasionally in the cortex or corpus callosum. Due to these characteristics, MOG-IgG (+) cases with brain or brainstem lesions are sometimes diagnosed with acute disseminated encephalomyelitis, meningitis, or symptomatic epilepsy. The myelitis in MOG-IgG (+) typically shows longitudinally extensive lesions as seen in neuromyelitis optica spectrum disorders. Acute treatment to reduce attack-related disability is recommended in MOG-IgG (+) disease, and long-term immunosuppression may be considered in patients with a high frequency of relapses and/or high risk of neurological disability.