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

Overview

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.


Sources

1. Gaspard N, Hirsch LJ, Sculier C et al. New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES): state of the art and perspectives. Epilepsia 2018; 59(4): 1– 8. doi: 10.1111/ epi.14022.

2. Marashly A, Lew S, Koop J. Succes­sful surgical management of new onset refractory status epilepticus (NORSE) present­­ing with gelastic seizure in a 3 year old girl. Epilepsy Behav Case Rep 2017; 8: 18– 26. doi: 10.1016/ j.ebcr.2017.05.002.

3. Gaspard N, Brandon PF, Alvarez V et al. New-onset refractory status epilepticus. Etiology, clinical features, and outcome. Neurology 2015; 85(18): 1604– 1613. doi: 10.1212/ WNL.0000000000001940.

4. Gal CRE, Jum­ma O, Mohanraj R. Five cases of new onset refractory status epilepticus (NORSE) syndrome: Outcome with early im­munother­apy. Seizure 2013; 22(3): 217– 220. doi: 10.1016/ j.seizure.2012.12.016.

5. Marečková I, Vojtěch Z, Procházka T et al. Případ de novo vzniklého super-refrakterního epileptického statu. Neurol Praxi 2015; 16(6): 366– 369.

6. Lizuka T, Kanazawa N, Kaneko J et al. Cryptogenic NORSE. Its distinctive clinical features and response to im­munother­apy. Neurol Neuroim­munol Neuroinflamm 2017; 4(6): e396. doi: 10.1212/ NXI.0000000000000396.

7. Wilder-Smith EP, Lim EC, Teoh HL et al. The NORSE (new-onset refractory status epilepticus) syndrome: defin­­ing a dis­ease entity. Ann Acad Med Singapore 2005; 34(7): 417– 420.

8. NORSE Institute. Norse dia­gnostic checklist. [online]. Available from URL: https:/ / static1.squarespace.com/ static/ 56433eade4b0b66656c0301c/ t/ 58b5dfa36b8f5b9a4 2283592/ 1488314276155/ An­notated+NORSE+Dia­gnos­tic+Checklist+pdf.pdf .

9. van Baalen A, Hausler M, Plecko-Startinig B et al. Febrile infection-related epilepsy syndrome without derectable autoantibodies and response to im­munother­apy: a case series and discus­sion of epileptogenesis in FIRES. Neuropediatrics 2012; 43(4): 209– 216. doi: 10.1055/ s-0032-1323848.

10. Fox K, Wel­ls ME, Ten­nison M et al. Febrile infection-related epilepsy syndrome (FIRES): a literature review and case study. Neuroradiogn J 2017; 57(3): 224– 233. doi: 10.1080/ 21646821.2017.1355181.

11. Aulická Š, Aulický P, Česká K et al. Management konvulzivního status epilepticus v dětském věku. Anesteziol Intenziv Med. In press 2018.

12. Li J, Saldivar Ch, Maganti RK. Plasma exchange in cryptogenic new onset refractory status epilepticus. Seizure 2013; 22(1): 70– 73. doi: 10.1016/ j.seizure.2012.09.011.

13. Kantanen AM, Reinikainen M, Parvianen I et al. Long-term outcome of refractory status epilepticus in adults: a retrospectice population –  based study. Epilepsy Research 2017; 133: 13– 21. doi: 10.1016/ j.eplepsyres.2017.03.009.

Labels
Paediatric neurology Neurosurgery Neurology

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Czech and Slovak Neurology and Neurosurgery

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