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

Autor: Bonardi CM; Paediatric Intensive Care Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy., Furlanis GM; Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy., Toldo I; Paediatric Neurology and Neurophysiology Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy., Guarrera B; Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy., Luisi C; Department of Neurosciences (DNS), University of Padua, Padua, Italy; Rare and Complex Epilepsy Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Full Member of European Reference Network EpiCARE, Rome, Italy., Pettenazzo A; Paediatric Intensive Care Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy., Nosadini M; Paediatric Neurology and Neurophysiology Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy., Boniver C; Paediatric Neurology and Neurophysiology Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy., Sartori S; Paediatric Neurology and Neurophysiology Unit, Department of Woman's and Child's Health, Padua University Hospital, Padua, Italy; Department of Neurosciences (DNS), University of Padua, Padua, Italy. Electronic address: stefano.sartori@unipd.it., Landi A; Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy; Department of Neurosciences (DNS), University of Padua, Padua, Italy.
Jazyk: angličtina
Zdroj: Brain & development [Brain Dev] 2023 May; Vol. 45 (5), pp. 293-299. Date of Electronic Publication: 2023 Jan 30.
DOI: 10.1016/j.braindev.2023.01.004
Abstrakt: 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.
Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2023 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.)
Databáze: MEDLINE
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