Comparative clinical outcomes in children with Febrile Infection-Related Epilepsy Syndrome (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.