A case of Vogt-Koyanagi-Harada disease associated with non-herpetic acute limbic encephalitis with autoantibodies against glutamate receptor ε2 in the cerebrospinal fluid

Abstract

We report a case of Vogt-Koyanagi-Harada (VKH) disease associated with non-herpetic acute limbic encephalitis with autoantibodies against glutamate receptor ε2 in the cerebrospinal fluid. A 42-year-old woman developed a complaint of visual distortion, visual disturbance, headache and mild psychiatric symptoms, such as anxiety and depression. She was diagnosed as VKH through the fidings of fluorescein fundus angiography, which revealed patchy hypofluorescence associated with delayed choroidal filling at early fluorescein angiographic phase, and spotted choroidal hyperfluorescence and pooling of dye at late phase. Analysis of the cerebrospinal fluid (CSF) showed slight increase of leukocyte count (49/μ/, mononuclear cells) and immunoglobulin (Ig) G index. An anti-GluRε2 IgM antibody was positive in CSF. Brain magnetic resonance imaging (MRI) showed a monofocal hyperintensity lesion in the left parahippocampal gyrus on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images. We diagnosed her VKH disease associated with non-herpetic acute limbic encephalitis. She was treated with oral prednisone, 70 mg day and her symptoms have gradually improved. To our knowledge, meningoencephalitis in VKH disease is extremely rare and the analysis of anti-GluRε2 IgM antibody in CSF has not been reported. We speculate that a certain immunologic mechanism, including the anti-GluRε2 IgM antibody, contributes to the pathogenesis of the VKH disease with non-herpetic acute limbic encephalitis.