RT Journal Article SR Electronic T1 MOG antibody–positive, benign, unilateral, cerebral cortical encephalitis with epilepsy JF Neurology - Neuroimmunology Neuroinflammation JO Neurol Neuroimmunol Neuroinflamm FD Lippincott Williams & Wilkins SP e322 DO 10.1212/NXI.0000000000000322 VO 4 IS 2 A1 Ogawa, Ryo A1 Nakashima, Ichiro A1 Takahashi, Toshiyuki A1 Kaneko, Kimihiko A1 Akaishi, Tetsuya A1 Takai, Yoshiki A1 Sato, Douglas Kazutoshi A1 Nishiyama, Shuhei A1 Misu, Tatsuro A1 Kuroda, Hiroshi A1 Aoki, Masashi A1 Fujihara, Kazuo YR 2017 UL http://nn.neurology.org/content/4/2/e322.abstract AB Objective: To describe the features of adult patients with benign, unilateral cerebral cortical encephalitis positive for the myelin oligodendrocyte glycoprotein (MOG) antibody.Methods: In this retrospective, cross-sectional study, after we encountered an index case of MOG antibody–positive unilateral cortical encephalitis with epileptic seizure, we tested for MOG antibody using our in-house, cell-based assay in a cohort of 24 consecutive adult patients with steroid-responsive encephalitis of unknown etiology seen at Tohoku University Hospital (2008–2014). We then analyzed the findings in MOG antibody–positive cases.Results: Three more patients, as well as the index case, were MOG antibody–positive, and all were adult men (median age 37 years, range 23–39 years). The main symptom was generalized epileptic seizure with or without abnormal behavior or consciousness disturbance. Two patients also developed unilateral benign optic neuritis (before or after seizure). In all patients, brain MRI demonstrated unilateral cerebral cortical fluid-attenuated inversion recovery hyperintense lesions, which were swollen and corresponded to hyperperfusion on SPECT. CSF studies showed moderate mononuclear pleocytosis with some polymorphonuclear cells and mildly elevated total protein levels, but myelin basic protein was not elevated. A screening of encephalitis-associated autoantibodies, including aquaporin-4, glutamate receptor, and voltage-gated potassium channel antibodies, was negative. All patients received antiepilepsy drugs and fully recovered after high-dose methylprednisolone, and the unilateral cortical MRI lesions subsequently disappeared. No patient experienced relapse.Conclusions: These MOG antibody–positive cases represent unique benign unilateral cortical encephalitis with epileptic seizure. The pathology may be autoimmune, although the findings differ from MOG antibody–associated demyelination and Rasmussen and other known immune-mediated encephalitides.ADEM=acute disseminated encephalomyelitis; AMPA=α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor; AQP4=aquaporin-4; CASPR2=contactin-associated protein 2; CBA=cell-based assay; CBC=complete blood count; CFF=critical flicker frequency; DWI=diffusion-weighted imaging; EAE=experimental autoimmune encephalomyelitis; FLAIR=fluid-attenuated inversion recovery; GABAB=γ-aminobutyric acid receptor type B receptor; GAD=glutamic acid decarboxylase; GdT1WI=gadolinium enhancement on T1-weighted imaging; HIMP=high-dose IV methylprednisolone; HSV=herpes simplex virus; IgG=immunoglobulin G; IL-6=interleukin-6; LETM=longitudinally extensive transverse myelitis; LGI1=leucine-rich glioma-inactivated protein 1; MBP=myelin basic protein; MOG=myelin oligodendrocyte glycoprotein; MS=multiple sclerosis; NMDAR=NMDA receptor; NMOSD=neuromyelitis optica spectrum disorder; ON=optic neuritis; PSL=prednisolone; RE=Rasmussen encephalitis; T2WI=T2-weighted imaging; Tg=thyroglobulin; TPO=thyroid peroxidase; VA=visual acuity; VEP=visual evoked potential