RT Journal Article
SR Electronic
T1 New-onset refractory status epilepticus (NORSE)
JF Practical Neurology
JO Pract Neurol
FD BMJ Publishing Group Ltd
SP 119
OP 127
DO 10.1136/practneurol-2020-002534
VO 21
IS 2
A1 Laura Mantoan Ritter
A1 Lina Nashef
YR 2021
UL http://pn.bmj.com/content/21/2/119.abstract
AB New-onset refractory status epilepticus and its subcategory febrile infection-related epilepsy syndrome are rare devastating clinical presentations in those without pre-existing relevant history, often in schoolchildren or young adults, without a clear cause on initial investigations. A cause is later identified in up to half of adults, but in many fewer children. Patients often require protracted intensive care and are at significant risk of dying. Functional disability is common and subsequent chronic epilepsy is the norm, but some people do have good outcomes, even after prolonged status epilepticus. Patients need prompt investigations and treatment. Anaesthetic and antiseizure medications are supplemented by other treatment modalities, including the ketogenic diet. Despite limited evidence, it is appropriate to try to modify the presumed underlying pathogenesis with immune modulation early, with a more recent focus on using interleukin inhibitors. Optimising management will require concerted multicentre international efforts.