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Case Reports
New-onset Refractory Status Epilepticus Involving the Limbic System, Spinal Cord, and Peripheral Nerves.
- Kensuke Daida, Kenya Nishioka, Masashi Takanashi, Manami Kobayashi, Keisuke Yoshikawa, Susumu Kusunoki, Kazumasa Yokoyama, and Nobutaka Hattori.
- Department of Neurology, Juntendo University School of Medicine, Japan.
- Intern. Med. 2020 Jan 15; 59 (2): 267-270.
AbstractA healthy 28-year-old woman presented suddenly with intractable status epilepticus: a focal seizure evolved into a generalized seizure preceded by a high fever. Brain magnetic resonance imaging indicated bilateral hyperintensities in the hippocampus on T2-weighted imaging. Electroencephalograms continuously demonstrated diffuse sharp waves and poly-spikes. Comprehensive immunomodulation therapies and anti-epileptic drugs did not lead to any improvements. We therefore diagnosed her with cryptogenic limbic encephalitis and new-onset refractory status epilepticus (NORSE). We detected positive anti-ganglioside antibodies, IgG-GQ1b, GD1a, and GT1b, which were negative at six months after the onset. We emphasize the heterogeneous pathogenesis and intractable conditions of NORSE.
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