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Case Report| Volume 45, ISSUE 5, P293-299, May 2023

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

  • Author Footnotes
    1 These authors contributed equally.
    Claudia Maria Bonardi
    Footnotes
    1 These authors contributed equally.
    Affiliations
    Paediatric Intensive Care Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Padua, Italy
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  • Author Footnotes
    1 These authors contributed equally.
    Giulia Melinda Furlanis
    Footnotes
    1 These authors contributed equally.
    Affiliations
    Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy
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  • Irene Toldo
    Affiliations
    Paediatric Neurology and Neurophysiology Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Padua, Italy
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  • Brando Guarrera
    Affiliations
    Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy
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  • Concetta Luisi
    Affiliations
    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
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  • Andrea Pettenazzo
    Affiliations
    Paediatric Intensive Care Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Padua, Italy
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  • Margherita Nosadini
    Affiliations
    Paediatric Neurology and Neurophysiology Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Padua, Italy
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  • Clementina Boniver
    Affiliations
    Paediatric Neurology and Neurophysiology Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Padua, Italy
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  • Author Footnotes
    2 Stefano Sartori and Andrea Landi share the last authorship.
    Stefano Sartori
    Correspondence
    Corresponding author at: Paediatric Neurology and Neurophysiology Unit, Department of Woman’s and Child’s Health, Padua University Hospital, Via Giustiniani 3, 35128, Padua, Italy.
    Footnotes
    2 Stefano Sartori and Andrea Landi share the last authorship.
    Affiliations
    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
    Search for articles by this author
  • Author Footnotes
    2 Stefano Sartori and Andrea Landi share the last authorship.
    Andrea Landi
    Footnotes
    2 Stefano Sartori and Andrea Landi share the last authorship.
    Affiliations
    Paediatric and Functional Neurosurgery Unit, Padua University Hospital, Padua, Italy

    Department of Neurosciences (DNS), University of Padua, Padua, Italy
    Search for articles by this author
  • Author Footnotes
    1 These authors contributed equally.
    2 Stefano Sartori and Andrea Landi share the last authorship.
Published:January 30, 2023DOI:https://doi.org/10.1016/j.braindev.2023.01.004
Myoclonic super-refractory status epilepticus with favourable evolution in a teenager with FIRES: Is the association of vagus nerve stimulation and cannabidiol effective?
Previous ArticleClinical value of therapeutic drug monitoring for levetiracetam in pediatric patients with epilepsy
Next ArticleA novel INPP4A mutation with pontocerebellar hypoplasia, myoclonic epilepsy, microcephaly, and severe developmental delay

      Abstract

      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

      Abbreviations:

      CBD (cannabidiol), FIRES (febrile infection-related epilepsy syndrome), KD (ketogenic diet), NORSE (new-onset refractory status epilepticus), RSE (refractory status epilepticus), VNS (vagal nerve stimulation)
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      Article info

      Publication history

      Published online: January 30, 2023
      Accepted: January 16, 2023
      Received in revised form: January 11, 2023
      Received: September 18, 2022

      Identification

      DOI: https://doi.org/10.1016/j.braindev.2023.01.004

      Copyright

      © 2023 Published by Elsevier B.V. on behalf of The Japanese Society of Child Neurology. All rights reserved.

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