To explore clinical manifestations, electroencephalogram and medical imaging characteristics of children with febrile infection-related epilepsy syndrome (FIRES).

A total of five children (patient 1-5) with FIRES were selected as research subjects, who were enrolled in West China Second University Hospital, Sichuan University from August 2016 to September 2019. Their clinical data, including clinical manifestations, laboratory tests results, electroencephalogram, imaging results, treatments and follow-up results, etc. were analyzed retrospectively. The procedures in this study followed the requirements of Helsinki Declaration of World Medical Association revised in 2013.

The results of these 5 children with FIRES were as follows. ①General clinical data: their age was 4-9 years old, the ratio of male to female was 2∶3, interval between fever and first seizure was 4-7 d, all were in coma when admitted to hospital. The modified Glasgow coma score was 3-7 scores. ②Electroencephalogram results: electroencephalogram background showed rhythms slowed and multifocal epileptiform discharges. ③Imaging results: results of cranial imaging in early stage were normal. Later, their brain atrophy could be found in the later stage, and abnormal signal shadows could be found in frontal, parietal, occipital cortex or subcortex, and periventricular white matter areas. ④Therapeutic results: they were treated by multiple antiepileptic drugs, hormones, gamma globulin, plasma exchange and other treatments and all these treatments were ineffective, but clinical seizures were significantly reduced after treatment by ketogenic diet (KD) in 1-14 d. ⑤Follow-up results: after followed up for 1-6 months, epileptic seizures were completely controlled in 2 cases (patient 3, 5), epileptic seizures were obviously controlled in 1 case (patient 1), 2 cases (patient 2, 4) remained intractable seizures. Other than 1 case (patient 3) remained mild learning disabilities, 4 cases (patient 1-2, 4-5) had moderate to severe cognitive impairment.

FIRES is a severe epileptic encephalopathy, and most children with FIRES may remain severe cognitive impairment and refractory seizures after treatment. The prognosis is poor, the effects of drug treatments such as conventional antiepileptic drugs, anesthetics, immune modulation and others are not ideal, KD treatment may help to control seizures.