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Purpose <p>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).</p> Methods <p>A retrospective analysis was performed on inpatients with a diagnosis of FIRES. They were treated with either VNS + KD (with an interval &lt; 5&#xa0;days) or KD alone, in addition to standard therapy.</p> Results <p>Eighteen children (nine females, nine males) with a median onset age of 8.1&#xa0;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&#xa0;days, respectively. Four children received VNS + KD, and 14 received KD alone. The median time to intervention was 16.5&#xa0;days after onset, and the median follow-up duration was 7.1&#xa0;years. The VNS + KD group showed a significant reduction in average seizure frequency per hour (<i>P</i> = 0.002). However, no significant differences were observed in ESD or duration of status epilepticus (SE) (<i>P</i> &gt; 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 (<i>P</i> &gt; 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 (<i>P</i> = 0.002). Younger age of onset was associated with increased odds of a poor outcome (<i>P</i> = 0.045).</p> Conclusion <p>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.</p>

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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

  • 期刊论文

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  • 发表日期：

    2025年10月6日

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  • Tianshuang Wang,
  • Xiaotian Chen,
  • Renqing Zhu,
  • Yixue Wang,
  • Xiaoyan Gong,
  • Lifei Yu,
  • Yi Wang,
  • Yuanfeng Zhou

摘要

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.