In these two cases, we describe NORSE with newly diagnosed Autoimmune encephalitis. The clinical courses for each were different.

Case 1, a 30 yo M with no pmh was admitted to Epilepsy Monitoring Unit (EMU). Patient was in focal status with LP CSF findings consistent with AIE. Patient had conventional treatment for AIE: steroids and IVIG. Patient was discharged upon clinical improvement. He was admitted a week later for worsening seizures. Patient’s seizures were treated with 5 PLEX sessions followed by chemotherapy (Actemra and Rituxan) administration in addition to high doses of antiseizure medication.

Case 2, 32 yo M with no pmh presents with 11 days of generalized weakness, cognitive slowing/confusion. Patient had 6 generalized tonic clonic seizures. Patient was in the ICU and intubated. Various agents were used for seizure control such as Vimpat, Keppra, Depakote and Ketamine. Day 4, was started on steroids and IVIG. Day 11, started on 5 sessions of PLEX followed by chemotherapy (Actemra and Rituxan) with significant improvement.

NORSE is a diagnosis with high rate of refractoriness to treatment and severe outcome. Early initiation of immunological therapy provide the most benefit.First line therapy is based on clinical presentation and expert opinion.These two cases illustrate better outcome with early initiation of plasmapheresis in rapidly progressing refractory focal status epilepticus of autoimmune etiology. Its been shown PLEX is better economically and less potential side effects as compared to IVIG. Rapid seizure control with combination of high dose new anti-seizure and immunological treatment in a timely manner are key for better outcomes.