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

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.

Highlights • New-onset refractory status epilepticus (NORSE) can present with a reversible splenial lesion. • Targeted temperature management and immunotherapy are useful for the treatment of a patient with NORSE who has a high fever. • Arterial spin labeling on MRI showed increased blood flow, assumed to be due to the persistent seizure activity.