Two Japanese cases of anti-MOG antibody-associated encephalitis that mimicked neuro-Behçet's disease

Abstract Recently, we documented two Japanese cases of myelin-oligodendrocyte glycoprotein (MOG) antibody-associated relapsing encephalitis among patients who had been diagnosed with probable neuro-Behcet's disease (NBD). They presented partial systemic BD symptoms, brainstem lesions, and the human leukocyte antigen (HLA) B51 allele and responded well to steroid therapy. Our cases suggest that we need to differentiate anti-MOG antibody-associated encephalitis from probable NBD because both disorders can present with brainstem or cerebral lesions, CSF pleocytosis, and elevated levels of CSF IL-6 and respond to steroid treatment. Furthermore, oral ulceration, skin lesions, and HLA-B51 might be observed nonspecifically in patients with anti-MOG antibody-associated encephalitis.