Superfast VNS Titration for the Treatment of SRSE in the Acute Phase of FIRES: A Pediatric Case Report and Literature Review

Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe epileptic encephalopathy that predominantly affects children, characterized by the sudden onset of superrefractory status epilepticus (SRSE) following a febrile infection. The management of SRSE in the acute phase of FIRES remains a major clinical challenge, as conventional antiseizure medications often yield unsatisfactory outcomes. Vagus nerve stimulation (VNS) is an established adjunctive therapy for refractory epilepsy, yet its application in the acute setting of FIRES with superfast titration has been rarely reported. Herein, we present a pediatric case of FIRES complicated by SRSE that was successfully managed with an accelerated VNS titration protocol. Our findings indicate that superfast VNS titration may be a feasible and effective therapeutic option for pediatric patients with FIRES-related SRSE who are unresponsive to conventional treatments. However, because of the limited number of cases and the lack of randomized controlled trials, large-scale, multicenter studies are warranted to validate the optimal titration regimen and long-term outcomes of this approach. This case report and literature review aim to provide clinical insights for the management of this devastating disease and highlight the potential value of early and accelerated VNS titration in the acute phase of FIRES.

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Journal
Journal of Child Neurology
Published
2026-06-09
DOI
https://doi.org/10.1177/08830738261454181
Primary Topic
Vagus Nerve Stimulation Research
Type
article
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article

Superfast VNS Titration for the Treatment of SRSE in the Acute Phase of FIRES: A Pediatric Case Report and Literature Review

Xing Lv, Jianguo Shi, Wandong Hu, Lin Wang et al.
Journal of Child Neurology
Vagus Nerve Stimulation Research
article

Superfast VNS Titration for the Treatment of SRSE in the Acute Phase of FIRES: A Pediatric Case Report and Literature Review

Xing Lv, Jianguo Shi, Wandong Hu, Lin Wang, Yao Meng, Guifu Geng
article en

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe epileptic encephalopathy that predominantly affects children, characterized by the sudden onset of superrefractory status epilepticus (SRSE) following a febrile infection. The management of SRSE in the acute phase of FIRES remains a major clinical challenge, as conventional antiseizure medications often yield unsatisfactory outcomes. Vagus nerve stimulation (VNS) is an established adjunctive therapy for refractory epilepsy, yet its application in the acute setting of FIRES with superfast titration has been rarely reported. Herein, we present a pediatric case of FIRES complicated by SRSE that was successfully managed with an accelerated VNS titration protocol. Our findings indicate that superfast VNS titration may be a feasible and effective therapeutic option for pediatric patients with FIRES-related SRSE who are unresponsive to conventional treatments. However, because of the limited number of cases and the lack of randomized controlled trials, large-scale, multicenter studies are warranted to validate the optimal titration regimen and long-term outcomes of this approach. This case report and literature review aim to provide clinical insights for the management of this devastating disease and highlight the potential value of early and accelerated VNS titration in the acute phase of FIRES.

Journal of Child Neurology
Children's Hospital (TN)
Good health and well-being
Openalex Percentile: Top 8%
Vagus Nerve Stimulation Research
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