AERRPS, DESC, NORSE, FIRES: multi-labeling or distinct epileptic entities?

In this review, we report a case of an adolescent girl presenting with epileptic encephalopathy preceded by febrile illness, demarcate the clinical phenotypic homogeneity among previously reported cases, and hypothesize on potential mechanisms based on current experimental evidence. Our literature review revealed >249 cases that share several main features: febrile illness with no preceding condition, negative laboratory studies including cerebrospinal fluid (CSF) analysis, status epilepticus refractory to conventional pharmacotherapy, and long-term developmental delays. This condition appears to have many names, the most recent of which is "FIRES" (fever-induced refractory epileptic encephalopathy). It seems likely that the described cases are representing the same entity. The possibility of a genetic or acquired channelopathy can be raised in light of negative infectious, autoimmune, microscopic, and gross pathology findings.
AuthorsFatima Y Ismail, Eric H Kossoff
JournalEpilepsia (Epilepsia) Vol. 52 Issue 11 Pg. e185-9 (Nov 2011) ISSN: 1528-1167 [Electronic] United States
PMID22004046 (Publication Type: Case Reports, Journal Article, Review)
CopyrightWiley Periodicals, Inc. © 2011 International League Against Epilepsy.
  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Electroencephalography
  • Epilepsy (classification, diagnosis)
  • Epilepsy, Complex Partial (classification, diagnosis)
  • Epilepsy, Tonic-Clonic (classification, diagnosis)
  • Female
  • Humans
  • Middle Aged
  • Seizures, Febrile (classification, diagnosis)
  • Status Epilepticus (classification, diagnosis)
  • Terminology as Topic
  • Young Adult

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