Abstract
Febrile infection–related epilepsy syndrome (FIRES) is a devastating epileptic encephalopathy with limited treatment options and unclear etiology. Vagus nerve stimulation (VNS) is an FDA-approved therapy for refractory epilepsy that has been shown to decrease the frequency and severity of seizures. There is a growing interest in alternate non-pharmaceutical therapies for managing super-refractory status epilepticus (SRSE). We present a 29-month-old case, diagnosed with FIRES, whose seizures were successfully controlled by utilization of VNS after ineffective response to intensive pharmacotherapy and ketogenic diet treatment. The VNS was planted after 14 days of refractory seizure activity with a following rapid parameter titration for 42 days without evident side effect, which finally controlled the seizure in the acute phase. VNS may be a potential candidate for the treatment of SRSE in FIRES.
References
Hirsch LJ, Gaspard N, van Baalen A et al (2018) Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia 59(4):739–744. https://doi.org/10.1111/epi.14016
Wheless JW, Gienapp AJ (2018) Phillippe Ryvlin (2018) Vagus nerve stimulation (VNS) therapy update. Epilepsy Behav 88S:2–10. https://doi.org/10.1016/j.yebeh.2018.06.032
Zeiler FA, Zeiler KJ, Teitelbaum J, Gillman LM, West M (2015) VNS for refractory status epilepticus. Epilepsy Res 112:100–113. https://doi.org/10.1016/j.eplepsyres.2015.02.014
Specchio N, Ferretti A, Pietrafusa N, Trivisano M, Calabrese C, Pavia GC, Benedictis De A, Marras CE, de Palma L , Vigevano F (2020) Refractory status epilepticus in genetic epilepsy-is vagus nerve stimulation an option. Front Neurol 11:443. https://doi.org/10.3389/fneur.2020.00443
Ganesana SL, Hahn CD (2019) Electrographic seizure burden and outcomes following pediatric status epilepticus. Epilepsy Behav 101(Pt B):106409. https://doi.org/10.1016/j.yebeh.2019.07.010
Elliott RE, Rodgers SD, Bassani L, Morsi A, Geller EB, Carlson C et al (2011) Vagus nerve stimulation for children with treatment-resistant epilepsy: a consecutive series of 141 cases. J Neurosurg Pediatr 7(5):491–500. https://doi.org/10.3171/2011.2.PEDS10505
Dibué-Adjei M, Brigo F, Yamamoto T, Vonck K, Trinka E (2019) Vagus nerve stimulation in refractory and super-refractory status epilepticus-a systematic review. Brain stimul 12(5):1101–1110. https://doi.org/10.1016/j.brs.2019.05.011
Grioni D, Landi A, Fiori L, Pietro SF (2018) Does emergent implantation of a vagal nerve stimulator stop refractory status epilepticus in children? Seizure 61:94–97. https://doi.org/10.1016/j.seizure.2018.08.008
Clarkson BDS, LaFrance-Corey RG, Kahoud RJ, Farias-Moeller R, Pne ET, Howe CL (2019) Functional defificiency in endogenous interleukin-1 receptor antagonist in patients with febrile infection-related epilepsy syndrome. ANN NEUROL 85:526–537. https://doi.org/10.1002/ana.25439
Kramer U, Chi CS, Lin KL, Specchio N, Sahin M et al (2011) Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children. Epilepsia 52:1956–1965. https://doi.org/10.1111/j.1528-1167.2011.03250.x
Sakuma H, Tanuma N, Kuki I, Takahashi Y, Shiomi M, Hayashi M (2015) Intrathecal overproduction of proinflammatory cytokines and chemokines in febrile infection-related refractory status epilepticus. J Neurol Neurosurg Psychiatry 86:820–822. https://doi.org/10.1136/jnnp-2014-309388
Funding
This paper is funded by the Shanghai Municipal Science and Technology Major Project (Grant No. 2017SHZDZX01) and Research Project of Shanghai Municipal Health Commission (Grant No. 201940351).
Author information
Authors and Affiliations
Contributions
TL is the first author who analyzed the data and drafted the manuscript. YXW and GPL conceived the idea and supervised the study. YFZ and YW are the corresponding authors who revised and are responsible for the manuscript content. All the authors participated in the proofreading. All the authors have read and approved the final manuscript.
Corresponding authors
Ethics declarations
Ethics approval
The study was performed with the approval of the Children’s Hospital of Fudan University Ethics Board (No. 2016 [117]).
Consent to participate
Written informed voluntary consent was obtained from the parents of the child.
Consent for publication
We confirm that we have read the Journal’s position on issues involved in the publication and affirm that this report is consistent with those guidelines.
Conflict of 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.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Luo, T., Wang, Y., Lu, G. et al. Vagus nerve stimulation for super-refractory status epilepticus in febrile infection–related epilepsy syndrome: a pediatric case report and literature review. Childs Nerv Syst 38, 1401–1404 (2022). https://doi.org/10.1007/s00381-021-05410-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-021-05410-6