New-onset refractory status epilepticus and considered spectrum disorders (NORSE/ FIRES)

Authors: K. Česká;  O. Horák;  H. Ošlejšková;  Š. Aulická
Authors‘ workplace: Klinika dětské neurologie LF MU a FN Brno, Centrum vysoce specializované péče pro farmakorezistentní epilepsie MZ ČR, Brno
Published in: Cesk Slov Neurol N 2018; 81(6): 658-662
Category: Review Article
doi: 10.14735/amcsnn2018658


New-onset refractory status epilepticus (NORSE) is defined as status epilepticus in a patient without active epilepsy or other preexist­­ing relevant neurological disorder. It is characterized by refractory epileptic seizures, without a clear active structural, toxic, or metabolic cause. In most cases, the dis­ease is preceded by mild febrile il­lnes­s. Very frequently, the ethiology of NORSE can not be proven (cryptogenic NORSE). In a minority of cases is causal autoim­mune encephalitis and viral infection of CNS. Conventional antiepileptic medications show very limited succes­s. Ther­apy includes im­munother­apy, ketogenic diet or another non pharmacological options. The long-term prognosis of patients is variable, in most cases cognitive impairments and epilepsy are present.

Key words:

new-onset refractory status epilepticus – clinical presentation – diagnostic protocol – therapy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manu­script met the ICMJE “uniform requirements” for biomedical papers.


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Paediatric neurology Neurosurgery Neurology

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Czech and Slovak Neurology and Neurosurgery

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