NORSE is defined as a new onset of refractory status epilepticus without a clear active structural, metabolic or toxic cause in a patient without active epilepsy. In up to half the cases a possible or probable cause is found- most commonly autoimmune or paraneoplastic encephalitis while infectious causes are less common.

A 64-year-old female was presented with sudden onset altered behavior consisting of reduced word output and unresponsiveness. She had intermittent jerky movements of the head and eyelid fluttering. EEG monitoring demonstrated non-convulsive status epilepticus originating from the right frontotemporal region. Antiepileptic drugs (AEDs) given included Ativan, Keppra, and Vimpat. She was later started on Midazolam infusion and Depakote, Clobazam and Topamax were added. The seizures persisted and she was placed in pentobarbital coma. Brain CT, CT angiogram and MRI were within normal. CSF revealed positive oligoclonal bands. Workup for infectious, inflammatory and autoimmune/paraneoplastic disease was negative except for markedly elevated serum GAD 65 Antibody at >250 units/ml (normal <5) and the levels have remained elevated throughout her hospitalization. The patient received multiple immunosuppressive medications including high-dose daily prednisone, intravenous immunoglobulin, rituximab, and plasma exchange without significant improvement in status epilepticus. Attempts to wean pentobarbital have been followed by recurrence of seizures and this medication has been continued.