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.
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