18F-FDG PET reveals frontotemporal dysfunction in children with fever-induced refractory epileptic encephalopathy

Michel Mazzuca1, Isabelle Jambaque, Lucie Hertz-Pannier

  • 1APHP, Neuropediatric Department, Hospital Necker-Enfants Malades, and Inserm, U663-University Paris Descartes, Paris, France.

Abstract

Insights

Fever-induced refractory epileptic encephalopathy in school-age children (FIRES) is a rare epilepsy. 18F-FDG PET scans reveal widespread brain dysfunction, explaining severe cognitive and behavioral issues in affected children.

Area of Science:

  • Neuroscience
  • Pediatric Neurology
  • Epileptology

Background:

  • Fever-induced refractory epileptic encephalopathy in school-age children (FIRES) is a distinct epileptic condition with unknown causes.
  • Standard MRI often shows limited brain abnormalities that do not correlate with severe neuropsychological deficits.

Purpose of the Study:

  • To investigate neocortical dysfunction in FIRES using 18F-FDG PET.
  • To identify common patterns of brain metabolism abnormalities in FIRES patients.

Main Methods:

  • Eight school-aged children (6-13 years) with FIRES underwent 18F-FDG PET scans.
  • Voxel-based analysis of cerebral glucose metabolism was performed using statistical parametric mapping, comparing patients to an age-matched control group.

Main Results:

  • Group analysis identified a widespread hypometabolic network in the temporoparietal and orbitofrontal cortices.
  • Individual analyses correlated hypometabolic areas with EEG foci and deficits in language, memory, and behavior.

Conclusions:

  • 18F-FDG PET imaging reveals a consistent network dysfunction in FIRES patients, despite clinical variability.
  • This brain network dysfunction likely underlies the significant neurological sequelae observed in FIRES.