A 16-Year-Old Boy with Meningoencephalitis with Auto-Antibody against Glutamate Receptor

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We herein report a 16-year-old boy who presented with fever and generalized convulsion. His symptoms progressively worsened, and electroencephalography (EEG) showed status with bi-frontal focus. One month after the initial presentation, examination of the cerebrospinal fluid showed pleocytosis, an increase in the IgG index and a positive oligoclonal IgG band. Brain MRI suggested slightly high signal intensity in the cerebral gray matter on FLAIR images. We considered that the patient had autoimmune meningoencephalitis, and therefore treated him with methyl-predonisolone pulse therapy, which substantially improved both his clinical condition and EEG findings. Before the therapy, the IgM and IgG auto-antibodies against glutamate receptor (GluR)ε2 and δ2 were positive, however they became negative after the therapy. In conclusion, some cases of meningoencephalitis with auto-antibodies against GluR may show an electrical status, mild brain MRI findings, and a good response to methylprednisolone pulse therapy. These clinical features were quite different from those observed for cases of Rasmussen's encephalitis.
一般社団法人日本小児神経学会の論文