Researchers studied a rare and serious seizure condition in children called FIRES that happens after a fever or infection. They tested whether combining two treatments—a special nerve stimulation device and a high-fat diet—worked better than the diet alone. The study followed 18 children for over 7 years. The combination treatment helped reduce how often seizures happened, and kids who got both treatments had better long-term outcomes. However, this condition is still very serious, and many children experienced lasting challenges. This research suggests that using both treatments together might be a helpful approach for doctors treating this rare condition.

The Quick Take

  • What they studied: Whether combining vagus nerve stimulation (a device that sends gentle electrical signals to a nerve in the neck) with a special high-fat diet works better than just the diet alone for treating a rare, severe seizure condition in children.
  • Who participated: 18 children (9 boys and 9 girls) with an average age of about 8 years old who had a rare seizure condition called FIRES that started after a fever or infection. Four children received the combination treatment, and 14 received only the diet treatment.
  • Key finding: Children who received both the nerve stimulation device and the special diet had significantly fewer seizures per hour compared to those who only received the diet. Additionally, these children had better long-term functioning and recovery outcomes.
  • What it means for you: If your child has this rare condition, doctors may consider combining these two treatments to help reduce seizures and improve long-term outcomes. However, this is a very serious condition that requires specialized medical care, and results vary from child to child.

The Research Details

This was a retrospective study, meaning researchers looked back at medical records of children who had already been treated for FIRES. They compared two groups: children who received both vagus nerve stimulation and a ketogenic diet (a special high-fat, low-carb diet), and children who received only the ketogenic diet. The vagus nerve stimulation device is a small implant that sends gentle electrical signals to help control seizures. All children also received standard seizure medications and intensive care. Researchers followed these children for an average of 7 years after treatment to see how well they recovered.

The study measured several things: how many seizures happened each hour, how long the seizure activity lasted, how long children needed to stay on breathing machines and in the hospital, and how well they functioned in daily life years later. The researchers used a special scale called the modified Rankin scale to measure long-term recovery, which looks at things like whether children could walk, talk, and care for themselves.

This type of study is useful for rare conditions because it’s hard to find enough patients to do larger, more controlled studies. However, because it looks back at past records rather than carefully controlling all the details, it has some limitations in what we can conclude.

FIRES is an extremely rare and life-threatening condition where children suddenly develop severe, continuous seizures after a fever or infection. Standard seizure medications often don’t work well for this condition. Understanding which treatment combinations work best is crucial because doctors need effective options to help these children survive and recover as well as possible. This research helps doctors know whether adding the nerve stimulation device to the special diet might improve outcomes.

This study has some important strengths and weaknesses. The strength is that it followed children for a long time (over 7 years), which helps show real-world outcomes. However, the study is small (only 18 children total, with only 4 in the combination treatment group), which means the results might not apply to all children with this condition. The study was not randomized, meaning doctors chose which treatment each child received rather than randomly assigning them, which could affect the results. Additionally, because this is a rare condition, it’s difficult to do larger, more controlled studies.

What the Results Show

Children who received both the nerve stimulation device and the special diet had significantly fewer seizures per hour compared to those who received only the diet. Before treatment, children were having an average of 3 seizures per hour. The combination treatment group showed much better improvement in reducing these seizures.

However, the study found no significant difference between the two groups in other measures, such as how long the seizure activity lasted, how long children needed to stay on breathing machines, or how long they stayed in the hospital. This suggests that while the combination treatment helped reduce seizure frequency, it didn’t dramatically change the overall hospital course.

The most important finding was about long-term outcomes. Children who received the combination treatment had significantly better functioning in their daily lives years later, as measured by how well they could walk, talk, and care for themselves. This suggests that the combination approach may help children recover better in the long run.

Overall, the study found that FIRES is a very serious condition: 55.6% of all children studied had moderate to severe disabilities at the last follow-up, and 16.7% had died. Younger children (those who developed the condition at a younger age) tended to have worse outcomes.

The study found that younger age at the start of the condition was associated with worse long-term outcomes. This was surprising and suggests that age may be an important factor in how this condition affects children. The researchers also noted that the time between when the condition started and when treatment began was about 16.5 days on average, which is important because earlier treatment might potentially lead to better outcomes.

This research adds to a growing body of evidence that the ketogenic diet (a high-fat, low-carb diet) can help control seizures in children with FIRES when standard medications don’t work. The new finding is that combining this diet with vagus nerve stimulation may provide additional benefits, particularly for long-term recovery. Previous research has shown that both treatments individually can help, but this study suggests that using them together might be even better. However, because FIRES is so rare, there isn’t a large amount of previous research to compare this to.

This study has several important limitations. First, it’s very small with only 18 children total, and only 4 received the combination treatment. This small number means the results might not apply to all children with FIRES. Second, the study was not randomized, meaning doctors chose which treatment each child received based on their judgment, which could have influenced the results. Third, because this is a rare condition, it was done at a single hospital, so the results might be different in other places. Fourth, the study looked back at past medical records rather than carefully controlling all aspects of the treatment. Finally, the long follow-up period (7 years) is good for seeing real-world outcomes, but some information from medical records might be incomplete or missing.

The Bottom Line

For children with FIRES, doctors should consider combining vagus nerve stimulation with the ketogenic diet as a treatment strategy, as this combination appears to reduce seizures and improve long-term outcomes. However, this recommendation is based on a small study, so more research is needed. This should only be done under the care of specialized neurologists and neurosurgeons who have experience with this rare condition. Standard seizure medications should continue to be used alongside these treatments. Confidence level: Moderate—the results are promising but based on a small number of patients.

This research is most relevant to children diagnosed with FIRES, their families, and the specialized doctors who treat this rare condition (pediatric neurologists and neurosurgeons). Parents of children with FIRES should discuss these findings with their medical team. This does not apply to children with other types of seizures or epilepsy. Healthcare providers treating FIRES should consider this as one option among several treatment approaches.

The combination treatment showed benefits relatively quickly in terms of reducing seizures (within days to weeks of starting treatment). However, the most important benefits—better long-term functioning and recovery—take much longer to see, typically months to years. Parents should understand that even with the best treatment, recovery from FIRES is often slow and challenging.

Want to Apply This Research?

  • For families managing FIRES, track the number of seizures per day or per hour (as medical teams measure), the duration of seizure activity, medication changes, and functional milestones (such as when the child can sit up, speak, or move independently). This data helps doctors see if treatments are working.
  • Work closely with your medical team to ensure the ketogenic diet is being followed correctly if prescribed, keep detailed records of seizure patterns to share with doctors, attend all follow-up appointments, and monitor for side effects of medications or the nerve stimulation device.
  • Maintain a seizure diary documenting frequency and duration of seizures, track functional abilities over time using simple observations (can the child move, speak, eat independently?), keep records of all medications and device settings, and have regular check-ins with the medical team to adjust treatment as needed. Long-term monitoring is essential as FIRES can have lasting effects.

This research describes a very rare and serious medical condition (FIRES) that requires specialized medical care. This information is for educational purposes only and should not replace professional medical advice. If your child has been diagnosed with FIRES or experiences severe, continuous seizures, seek immediate care from a pediatric neurologist or neurosurgeon. Treatment decisions should be made in consultation with your child’s medical team based on their individual situation. The findings from this small study suggest a potential treatment approach but should not be considered definitive proof. More research is needed to fully understand the benefits and risks of combining these treatments.