Vagus Nerve Stimulation Combined with Ketogenic Diet Versus Ketogenic Diet Alone in the Treatment of Febrile Infection-Related Epilepsy Syndrome in Children: Efficacy and Long-Term Prognosis Study

Abstract Purpose To evaluate the clinical efficacy and long-term functional prognosis of vagus nerve stimulation (VNS) combined with ketogenic diet (KD) in the treatment of children with febrile infection-related epilepsy syndrome (FIRES). Methods A retrospective analysis was performed on inpatients with diagnosis of FIRES. They were treated with either VNS + KD (with an interval < 5 days) or KD alone, in addition to standard therapy. Results Eighteen children (9 boys) with a median onset age of 7.35 years were studied. Before KD/VNS treatment, the average number of seizures (AS) per hour was 4.34 and the percentage of total duration of electrographic seizures (ESD) per hour was 65.5%. Median anesthesia, ICU stay, and hospitalization durations were 58.5, 65.5, and 74.5 days, respectively. Four children received VNS + KD, and 14 received KD alone. The median intervention time was 16.5 days, and follow-up lasted 5.41 years. No significant differences were found between the groups in terms of anesthesia duration, mechanical ventilation, ICU stay, and hospitalization (P > 0.05). The VNS + KD group showed a significant AS reduction (P = 0.002) but no significant differences in ESD or SE duration (P > 0.05). At the last follow-up, 55.56% had moderate to severe disabilities, and 16.67% had died. The VNS + KD group had a significantly better modified Rankin scale than the KD group (P = 0.002). A younger onset age was linked to poorer outcomes (P = 0.038). Conclusion FIRES patients experience severe seizure burdens in acute phase and have poor long-term prognoses. Combining VNS and KD treatments appears promising for reducing seizures and improving long-term function for FIRES patients.