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Published on: September 20, 2024
Comparative clinical outcomes in children with Febrile Infection-Related Epilepsy Syndrome (FIRES)
Jia Hui Teo1, Wai Chen Lim2, Mae Shyan Wong3
1Neurology Service, Department of Paediatrics, KK Women's and Children's Hospital, Singapore.
Insights
Children with Febrile Infection-Related Epilepsy Syndrome (FIRES) often experience long-term behavioral and emotional issues. Early screening for aggression, inattention, and mood disorders is crucial for all recovering children.
Area of Science:
- Pediatric Neurology
- Neuroinflammation
- Epilepsy Syndromes
Background:
- Febrile Infection-Related Epilepsy Syndrome (FIRES) is a severe, rare encephalopathy in children.
- Understanding the long-term functional, emotional, and behavioral outcomes is critical for comprehensive patient care.
- Treatment advancements include biologics and ketogenic diets (KD), necessitating outcome evaluation.
Purpose of the Study:
- To compare functional, emotional, and behavioral outcomes in children with FIRES.
- To assess outcomes in patients receiving conventional treatments versus those also treated with biologics and/or KD.
- To analyze outcomes over a 24-month period post-diagnosis.
Main Methods:
- Retrospective chart review of pediatric patients (<18 years) diagnosed with FIRES.
- Analysis of two cohorts based on biologic availability (pre-2018 vs. 2018 onwards).
- Functional assessment using modified Rankin Scale (mRS) and WeeFIM; behavioral screening via MOAS, ADHD, anxiety, and depression diagnoses.
Main Results:
- Children requiring biologics and/or KD showed lower functional scores (WeeFIM) at discharge and 24 months.
- Aggression was more prevalent (53.5%) and severe (higher MOAS scores) in the later period (2018-2023).
- Inattention and mood disorders occurred later than aggression, with similar proportions across treatment groups.
Conclusions:
- Behavioral and emotional sequelae are common in FIRES, irrespective of advanced treatments like biologics or KD.
- Aggression typically manifests earlier, while inattention and mood disorders appear later in the disease course.
- Routine screening for long-term emotional and behavioral issues is essential for all children recovering from FIRES.
Background And Aims:
We evaluated functional, emotional, and behavioural outcomes in children with FIRES in those who received only conventional treatments versus those who received biologics and/or ketogenic diet.
Methods:
A retrospective chart review of children (<18 years) diagnosed with FIRES at two paediatric hospitals. Children were analysed according to two time periods according to availability of biologics (from 2018 onwards). Functional outcomes were assessed via modified Rankin Scale (mRS) and paediatric functional independence measure (WeeFIM). Aggression symptoms (screened with modified overt aggression scale (MOAS)), diagnoses of attention deficit/ hyperactivity disorder (ADHD), and anxiety and depression were also recorded. These outcome measures were analysed in intervals, up to 24 months post diagnosis.
Results:
There were 30 children (mean age 8.9 ± 4.2 years) diagnosed with FIRES. Median mRS at 12 months post FIRES was 2 (IQR 1-5). WeeFIM score was lower at discharge (p = 0.053) to 24 months follow up (p = 0.030) in children who required biologics and/or KD. Aggression was more prevalent (53.5 %) in the latter period (2018-2023) with median MOAS scores higher at discharge (p = 0.019) and follow up at 24 months (p = 0.10). Inattention (33.3 %) and mood disorders (20 %) were observed in similar proportions of children across both time periods. Aggression appeared earlier (median 1.8 months, IQR 1-4.5) whilst inattention and mood disorder were noted later (median 4 months, IQR 1.8-10.5 and median 27 months, IQR 12 = 39 respectively).
Conclusion:
All children recovering from FIRES need to be screened for potential long-term emotional and behavioural sequelae.
What This Paper Adds:
(1) Behavioural and emotional sequelae were observed in FIRES regardless of whether biologics and/or Ketogenic Diet treatment was given. (2) Aggression symptoms appeared earlier, whilst inattention and mood disorder were noted later during follow up. (3) All children post FIRES should be screened for long-term emotional and behavioural sequelae.
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