Survey of the diagnostic and therapeutic approach to new-onset refractory status epilepticus

We conducted a survey of providers to assess for practice patterns in diagnosing and treating new-onset refractory status epilepticus (NORSE). NORSE is the occurrence of prolonged seizures that are not responsive to initial therapies in otherwise healthy individuals without obvious cause on initial presentation. This entity is thought to have multiple etiologies, including autoimmune.

A 29-question electronic survey was sent to providers included in the Neurocritical Care Society emailing list. Questions regarded responders' demographics, existing institutional practice protocols, diagnostic work-up, therapeutic management and expected outcomes in NORSE. Responses were collected from October 23, 2014, to November 25, 2014.

There were 107 respondents out of 1334 (8%). CT head, continuous EEG, lumbar puncture and microbe serologies were suggested within 24h of presentation as part of a diagnostic work-up. MRI brain, autoimmune work-up (systemic and anti-neuronal antibodies) and cytology/flow cytometry were favored later in the course. About 25% of providers would never perform an autoimmune work-up in this setting. Initial treatment included up to 3 anticonvulsants (including one anesthetic), followed by additional anticonvulsants/anesthetics along with antimicrobials, followed by steroids, plasma exchange, hypothermia and ketogenic diet. Many respondents would never use IV immunoglobulin or steroid-sparing immunosuppressants (29% and 42%, respectively) for NORSE.

This survey could serve as the foundation for development of a standardized approach for the diagnosis and treatment of NORSE.