Myoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?

Brain Dev. 2023 May;45(5):293-299. doi: 10.1016/j.braindev.2023.01.004. Epub 2023 Jan 30.


Background: Febrile infection-related epilepsy syndrome (FIRES) is a rare and catastrophic clinical syndrome occurring in previously healthy patients. Aetiology is still unknown and outcome usually poor. We describe a case of myoclonic prolonged super refractory status epilepticus (P-SRSE) in FIRES in a patient admitted to the paediatric intensive care unit of Padova, Italy.

Case report: A previously healthy 14-year-old girl with onset of myoclonic status epilepticus after a mild febrile illness was admitted to our hospital with a diagnosis of FIRES. Extensive diagnostic work-up was inconclusive. Status epilepticus and electroclinical seizures recurred every time weaning from anaesthetic agents was attempted. Eventually, a vagal nerve stimulator (VNS) was implanted and cannabidiol (CBD) administered, 43 days and 70 days after P-SRSE onset, respectively. Two days after CBD introduction, status epilepticus weaned and the girl rapidly regained complete consciousness showing a brilliant and unexpected recovery. At last follow-up, 12 months later, she is 8-months seizure free on multiple antiseizure medications, has only mild neuropsychological impairment with no neurological and intellective deficit.

Conclusions: To our knowledge, this represents a unique case with an extremely favourable evolution with a possible effect of the association of VNS and CBD to traditional antiseizure medications.

Keywords: Cannabidiol; FIRES; Febrile infection-related epilepsy syndrome; Paediatrics; Status epilepticus; Vagus nerve stimulation.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Cannabidiol* / therapeutic use
  • Child
  • Drug Resistant Epilepsy* / diagnosis
  • Encephalitis* / complications
  • Female
  • Humans
  • Immune System Diseases* / complications
  • Seizures / complications
  • Status Epilepticus* / diagnosis
  • Status Epilepticus* / drug therapy
  • Vagus Nerve Stimulation*


  • Cannabidiol