Effective plasmapheresis treatment of intractable visual symptoms of MOG antibody-related disorders

<p>  Myelin oligodendrocyte glycoprotein (MOG) antibody-positive disorders are generally considered to have a good response to steroids ; however, no treatment policy has been established for patients who do not respond to initial treatment or whose symptoms do not improve sufficiently. We report the case of a 12-year-old boy who experienced the onset of photophobia in the left eye, difficulty in moving facial muscles, and dysuria. Fluid-attenuated inversion recovery images of the brain showed multiple lesions in the cerebral cortex and subcortical white matter. Hence, we diagnosed acute disseminated encephalomyelitis. However, visual symptoms did not improve after 3 courses of methylprednisolone pulse (each course : 1 g/day for 3 days) and high-dose immunoglobulin (1 g/kg/dose) therapies. Subsequently, MOG antibodies were found in the serum and cerebrospinal fluid samples (serum titer, 1 : 2,048 ; cerebrospinal fluid titer, 1 : 64), and thus an anti-MOG antibody-related disease was confirmed. Plasmapheresis was performed thrice, followed by one course of methylprednisolone pulse therapy, following which the visual symptoms gradually improved. To prevent recurrence, this treatment was followed by oral prednisolone, which was gradually tapered over the next 7 months. In the 20 months since the onset of symptoms, the patient has exhibited no relapse. In MOG antibody-related disorders, visual symptoms are a common residual disability. In our patient, the improvement of visual symptoms was observed after plasmapheresis ; therefore, plasmapheresis should be considered while referring to the flicker value when the therapeutic effect of intravenous methylprednisolone is poor in patients with optic nerve inflammation.</p>