Multifocal multilobar focal cortical dysplasia type IIa in Febrile Infection Related Epilepsy Syndrome (FIRES)

Clin Neurol Neurosurg. 2022 Jun:217:107247. doi: 10.1016/j.clineuro.2022.107247. Epub 2022 Apr 11.

Abstract

A seven-year-old girl with history of type I diabetes and no history of seizures presented for altered mental status with convulsions nearly one week after a febrile illness. Serum and laboratory studies were normal with EEG showing biparietal fast activity and seizures originating from right occipital lobe consistent with FIRES. A collaborative decision was ultimately made to withdraw care. Post-mortem whole brain histopathological examination revealed diffuse abnormalities in multiple areas including both parietal lobes and the right parieto-occipital junction consistent with focal cortical dysplasia type IIa. We believe this to be the first report that describes focal cortical dysplasia type IIa co-localizing with epileptogenic areas on EEG in a case of FIRES, and recommend that focal cortical dysplasia be considered as an etiology early in the course of FIRES.

Keywords: Cortical malformations; Epilepsy syndromes; Refractory epilepsy.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Drug Resistant Epilepsy* / etiology
  • Drug Resistant Epilepsy* / surgery
  • Electroencephalography
  • Encephalitis* / complications
  • Epilepsy
  • Epileptic Syndromes* / complications
  • Female
  • Humans
  • Magnetic Resonance Imaging / adverse effects
  • Malformations of Cortical Development* / complications
  • Malformations of Cortical Development* / diagnostic imaging
  • Malformations of Cortical Development, Group I
  • Seizures / etiology
  • Treatment Outcome

Supplementary concepts

  • Focal cortical dysplasia of Taylor