New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome: Developmental Medicine and Child Neurology

N. Specchio, N. Pietrafusa

Research output: Contribution to journalArticlepeer-review

Abstract

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are relatively rare clinical presentations. They are characterized by de novo onset of refractory status epilepticus (RSE) without clearly identifiable acute or active cause (structural, toxic, or metabolic). We reviewed the literature using PubMed reports published between 2003 and 2019 and summarized the clinical, neurophysiological, imaging, and treatment findings. Focal motor seizures, which tend to evolve into status epilepticus, characterize the typical presentation. Disease course is biphasic: acute phase followed by chronic phase with refractory epilepsy and neurological impairment. Aetiology is unknown, but immune-inflammatory-mediated epileptic encephalopathy is suspected. Electroencephalograms show variety in discharges (sporadic or periodic, focal, generalized, or more frequently bilateral), sometimes with a multifocal pattern. About 70% of adult NORSE have abnormal magnetic resonance imaging (MRI); in paediatric series of FIRES, 61.2% of patients have a normal brain MRI at the beginning and only 18.5% during the chronic phase. No specific therapy for FIRES and NORSE currently exists; high doses of barbiturates and ketogenic diet can be used with some effectiveness. Recently, anakinra and tocilizumab, targeting interleukin pathways, have emerged as potential specific therapies. Mortality rate is around 12% in children and even higher in adults (16–27%). © 2020 Mac Keith Press
Original languageEnglish
Pages (from-to)897-905
Number of pages9
JournalDev. Med. Child Neurol.
Volume62
Issue number8
DOIs
Publication statusPublished - 2020

Keywords

  • anakinra
  • barbituric acid derivative
  • tocilizumab
  • anticonvulsant therapy
  • brain disease
  • clinical feature
  • differential diagnosis
  • disease course
  • drug megadose
  • drug resistant epilepsy
  • electroencephalogram
  • epilepsy
  • epileptic state
  • fever
  • human
  • infection
  • ketogenic diet
  • laboratory test
  • medical history
  • mortality rate
  • neuroimaging
  • neuropathology
  • neurophysiology
  • new onset refractory status epilepticus
  • nuclear magnetic resonance imaging
  • outcome assessment
  • pathophysiology
  • priority journal
  • Review
  • seizure
  • complication
  • Epileptic Syndromes
  • Fever
  • Humans
  • Infections
  • Status Epilepticus

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