Übersicht über erstmaliges Auftreten eines therapierefraktären Status epilepticus: aktuelle Konzepte, Diagnose und Behandlung

Abstract

New-onset refractory status epilepticus (NORSE) is a rare and devastating condition presenting with refractory status epilepticus and often evolving into super-refractory status epilepticus. The poor outcome with a significant mortality rate and a high rate of morbidity among survivors of NORSE warrants awareness and early identification of the condition so as to initiate appropriate investigations and management. Although the pathophysiological mechanisms are unknown, inflammatory mechanisms including activation of innate immunity are likely involved. Treatment should therefore include aggressive escalation of antiseizure medications followed by early initiation of a ketogenic diet and immunomodulation. First-line immunotherapy should be initiated within 72 h of seizure onset, with a ketogenic diet and second-line immunomodulation being started in the first week if seizures remain. This article aims to review current knowledge on NORSE and to highlight specific areas including recent consensus definitions and Delphi-based recommendations as well as open registries for future research.