Reversible splenial lesion in a patient with new-onset refractory status epilepticus (NORSE)

Akane Unno Mizutani; Akihiro Shindo; Shigeo Arikawa; Takuya Shimada; Keita Matsuura; Kaoru Ikejiri; Kei Suzuki; Hiroshi Imai; Hidekazu Tomimoto

doi:10.1016/j.ensci.2019.100220

Reversible splenial lesion in a patient with new-onset refractory status epilepticus (NORSE)

eNeurologicalSci. 2019 Dec 24:18:100220. doi: 10.1016/j.ensci.2019.100220. eCollection 2020 Mar.

Authors

Akane Unno Mizutani¹, Akihiro Shindo¹, Shigeo Arikawa¹, Takuya Shimada¹, Keita Matsuura¹, Kaoru Ikejiri², Kei Suzuki², Hiroshi Imai², Hidekazu Tomimoto¹

Affiliations

¹ Department of Neurology, Mie University Graduate School of Medicine, Tsu, Mie, Japan.
² Mie University Hospital, Emergency and Critical Care Center, Tsu, Mie, Japan.

Abstract

New-onset refractory status epilepticus (NORSE) is a rare neurological emergency condition with poor prognosis. A 30-year-old male suddenly had tonic-clonic convulsions seven days after a preceding fever and diarrhea. MRI showed a reversible splenial lesion, and he developed refractory multifocal and generalized seizures in spite of anticonvulsant medication. He was diagnosed with NORSE and received a combination treatment with immunotherapy and targeted temperature management (TTM), which effectively decreased his seizures. This case suggests that even for patients with reversible splenial lesions, NORSE should be considered, and that treatment with immunotherapy and TTM may be effective.

Keywords: **, discontinued due to granulocytopenia.; *, discontinued due to liver failure; Acute encephalitis; Acute encephalopathy; CBZ, carbamazepine; CHDF, continuous hemodiafiltration; DZP, diazepam; IVIg, intravenous immunoglobulin; Immunotherapy; KBr, potassium bromide; LCM, lacosamide; LEV, levetiracetam; PB, phenobarbital; PHT, phenytoin; PLEX, plasma exchange; PMP, perampanel; Status epilepticus; Targeted temperature management; ZNS, zonisamide; mPSL, methylprednisolone pulse.