Abstract

A 54-year-old man developed altered mental state and generalised tonic-clonic seizures after 1 week of upper respiratory tract symptoms and diarrhoea, having been previously well. His MR scan of brain showed multifocal progressive T2 cortical signal changes. He was diagnosed with new-onset refractory status epilepticus (NORSE), initially treated as being secondary to autoimmune/paraneoplastic limbic encephalitis, although subsequent investigations were negative. His seizures and electrographic epileptiform activity continued despite escalating doses of antiseizure medications, immunosuppression with corticosteroids, immunoglobulins, plasma exchange and rituximab, and thereafter anaesthetic agents. A vagus nerve stimulator (VNS) was implanted 6 weeks after admission and its voltage rapidly increased over 4 days; his seizure activity resolved in the third week after VNS implantation. This case highlights the role of VNS in the early management of NORSE.