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pmid33620842      StatPearls-/-ä 2024 ; ä (ä): ä
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  • New Onset Refractory Status Epilepticus #MMPMID33620842
  • Bhatia K; De Jesus O
  • StatPearls-/-ä 2024[Jan]; ä (ä): ä PMID33620842show ga
  • The International League Against Epilepsy (ILAE) defines status epilepticus (SE) as continuous clinical and/or electrographic seizure activity, or recurrent seizure activity without recovery to baseline, lasting for >/= 5 mins. If the seizure activity continues despite treatment with adequate doses of an initial benzodiazepine and an acceptable second-line intravenous (IV) antiepileptic medication, the patient is considered to have progressed into refractory status epilepticus (RSE). Super refractory status epilepticus (SRSE) is defined as RSE that continues or recurs despite the use of IV anesthetic agents for >/= 24 hours, including those cases that recur upon withdrawal or cessation of anesthetic agents. RSE is seen in approximately 9% to 43% of all SE cases, with a reported in-hospital mortality of 15% to 33%. It has been observed that approximately 4% to 12% of patients with SE eventually progress to SRSE, with in-hospital mortality reported to be as high as 40% to 54%. The term new-onset refractory status epilepticus (NORSE) was first used by Wilder-Smith et al. in 2005 to describe cases of SRSE with no associated past medical history of epilepsy and with no identifiable cause despite initial evaluation. In 2018 an international group of experts proposed a consensus definition of NORSE and related conditions to allow a more standardized terminology to improve patient management and clinical research. NORSE is defined as a "clinical presentation characterized by new onset of refractory status epilepticus, in a patient without active epilepsy or other preexisting relevant neurological disorder (acute strokes, brain masses, drug overdoses, etc.), and without a clear acute or active structural, toxic or metabolic cause." A closely related condition, febrile infection-related epilepsy syndrome (FIRES), has been defined as a subset of NORSE, associated with a prior febrile infection, with fever starting between 2 weeks to 24 hours before the onset of RSE. In contrast, patients with NORSE may or may not be associated with fever before the start of RSE.
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