Aims: To summarize the clinical features and outcomes in children with FIRES and review current evidence. Methods: A retrospective cohort on children 1-16 years with a diagnosis of FIRES in a district hospital in south India over 1 year and literature review following consensus on FIRES in 2018. Results: We had 12 children (70% male) presenting with refractory seizures on day 3 or 4. The onset was generalized tonic-clonic seizures (GTCS) in 75% with focal seizures, encephalopathy, or extrapyramidal symptoms in 25%. Baseline blood and MRI were normal. CSF was negative. Electroencephalogram (EEG) was abnormal in 91%. Benzodiazepine and steroids were used in all whilst ketogenic diet could not be tried. Mortality was 16% and others had neurological sequelae at 1-year follow-up. Discussion: Acute refractory seizures preceded by febrile illness in children between 5 and 13 years define the condition with male preponderance. The incidence is reported to be 1: 1,000,000 whilst we had 12 children in a single center in 1 year. Focal followed by GTCS has been mentioned as the common presentation however, 83% of our cohort presented with GTCS. No specific EEG pattern identified. Ketogenic diet and immunomodulation have been tried both of which were limited due to resources. Mortality ranges from 12-30% with early management playing a key role. Conclusion: FIRES is a diagnostic dilemma despite advances. Early clinical diagnosis with a structured multi-centric approach might help opening a new horizon.

Abinaya Seenivasan
Royal Manchester Children's Hospital
United Kingdom

Nandakumar Nambiyappan
Nambiyappan hospitals
India