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 - PubMed
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FIRES patients experienced severe seizure burdens in the acute phase and had poor long-term prognoses. Combining VNS and KD treatments in the acute phase may represent a viable therapeutic strategy for reducing seizures and improving long-term function for pediatric FIRES.

Childs Nerv Syst. 2025 Oct 6;41(1):305. doi: 10.1007/s00381-025-06957-4.

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 a 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 (nine females, nine males) with a median onset age of 8.1 years were studied. Before KD/VNS treatment, the average number of seizures (AS) per hour was 3.0 and the percentage of total duration of electrographic seizures (ESD) per hour was 65.5%. Median duration of anesthesia, duration of mechanical ventilation, and duration in ICU and hospital were 58.5, 39.5, 65.5, and 74.5 days, respectively. Four children received VNS + KD, and 14 received KD alone. The median time to intervention was 16.5 days after onset, and the median follow-up duration was 7.1 years. The VNS + KD group showed a significant reduction in average seizure frequency per hour (P = 0.002). However, no significant differences were observed in ESD or duration of status epilepticus (SE) (P > 0.05). And there were no significant differences between the groups in terms of duration of anesthesia, duration of mechanical ventilation, and duration in ICU and hospital (P > 0.05). At the last follow-up, 55.6% had moderate to severe disabilities, and 16.7% had died. The VNS + KD group exhibited significantly better functional outcomes as measured by the modified Rankin scale (P = 0.002). Younger age of onset was associated with increased odds of a poor outcome (P = 0.045).

CONCLUSION: FIRES patients experienced severe seizure burdens in the acute phase and had poor long-term prognoses. Combining VNS and KD treatments in the acute phase may represent a viable therapeutic strategy for reducing seizures and improving long-term function for pediatric FIRES.

PMID:41047417 | DOI:10.1007/s00381-025-06957-4

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