New-onset SUPER refractory status epilepticus: A case series of 26 patients.

Objective: To better understand the heterogeneous population of patients with new-onset refractory status epilepticus (NORSE), we studied the most severe cases who presented with new-onset super refractory status epilepticus (NOSRSE). Methods: We report a retrospective case series of 26 adults admitted to the Columbia University Irving Medical Center Neurological Intensive Care Unit (NICU) from 2/2009-2/2016 with NOSRSE. We evaluated demographics, diagnostic studies, and treatment course. Outcomes were modified Rankin Score (mRS) at hospital discharge and most recent follow-up visit (minimum of 2 months post discharge), NICU and hospital length of stay, and long-term anti-epileptic drug (AED) use. Results: Of the 252 patients with refractory status epilepticus, 27/252 had NORSE and 26/27 of those had NOSRSE. Age was bimodally distributed with peaks at 27 and 63 years. The vast majority (96%) had an infectious and/or psychiatric prodrome. Etiology was cryptogenic in 73%, autoimmune in 19%, and infectious in 8%. Seven patients (27%) underwent brain biopsy, autopsy or both; 3 (12%) were diagnostic (herpes simplex encephalitis, candida encephalitis, and acute demyelinating encephalomyelitis). On discharge, 6 patients (23%) had good or fair outcome (mRS 0-3). Of the patients with long-term follow-up data (median 9 months, interquartile range 2-22 months), 12 patients (71%) had mRS 0-3. Conclusion: Among our cohort, nearly all patients with NORSE had NOSRSE. The majority were cryptogenic with few antibody-positive cases identified. Neuropathology was diagnostic in 12% of cases. Although only 23% of patients had good or fair outcome on discharge, 71% met these criteria at follow-up.