| Objective: To investigate the clinical characteristics of children with febrile infections-related epilepsy syndrome(FIRES)and severe viral encephalitis(SVE),therefore to provide clinical experience for the early differential diagnosis and early treatment.Methods: Twenty three children patients with FIRES and twenty four children patients with SVE which caused by definite pathogen were enrolled in the study,in Children's Hospital of Chongqing Medical University from January 2016 to June 2019.The acute clinical features,EEG,MRI,treatment and prognosis were retrospectively analyzed and compared between the two groups.Results: 1.24 children in the SVE had positive virus-specific antibody IgM in CSF,including 12 cases of EB virus,6 cases of herpes simplex virus,3 cases of measles virus,and 2 cases of enterovirus and adenovirus.There were no abnormalities virus-specific antibodies in CSF of 23 Children with FIRES.2.Clinical characteristics:1)General Information: 23 cases of FIRES were 2.3 men for every 1 woman,24 cases of SVE were 1.4 men for every 1 woman,both more boy,with no gender difference.Compared with SVE,patients with FIRES have an elder average age of onset(7.39±3.0y vs.3.83±3.57y),Prodromal infection was common in both groups(73.9% vs 91.7%),with gender difference.2)Clinical features: Compared with SVE,the first seizure was SE rarely in FIRSE(13.0% vs 42.9%),but quickly occur frequent partial seizure are more common(73.9% vs 40.0%)and SE last longer(19.09±9.76 d vs 4.05±3.68d).The interval between first fever and first seizure was longer(3.70±1.30 d vs 2.38±1.80d),The duration of fever in FIRES was longer(40.74±34.08 d vs 15.50±10.91d),and the period of unconsciousness was longer(39.00 d vs21.5d).All comparative items mentioned above were statistically significant(P<0.05).2.CSF: Compared with SVE,FIRES's normal cerebrospinal fluid examination is more than SVE(69.6%vs20.8%).WBC count [6.00(2.00,8.00)*10^6/L] and protein concentration [0.31(0.25,0.44)mmol/L] in few FIRES are only mild elevated,but WBC count [21.00(2.75,54.00)*10^6/L] and protein concentration [0.56(0.28,1.07)mmol/L] in SVE are only mild to moderately elevated,All comparative items mentioned above were statistically significant(P<0.05).3.EEG:compared with SVE,patients with FIRES have more multifocal discharges on the attack stage(40.0% vs 0%),and more frequent frontal discharges while interictal(40.0% vs 9.1%)on EEG during the acute phase.All comparative items mentioned above were statistically significant(P <0.05).5.Head MRI: Compared with SVE,normal brain MRI of FIRES is more common(68.4% vs 22.2%)the comparative item mentioned above was statistically significant(P<0.05).50.0% of SVE are multiple lesions of brain MRI,involving temporal lobe(50.0%),insular lobe(44.4%)frontal lobe(38.9%)and thalamus(38.9%).6.Treatment: Both diseases need comprehensive treatments,and the types of AEDs(4.48±1.31 vs 1.38±0.92)and BSC(1.91±0.52 vs 1.42±0.78)are more used in the acute phase of FIRES.Longer treatment of ICU and assisted ventilation,All comparative items mentioned above were statistically significant(P<0.05).7.Prognosis: Followed up the two groups of children from 3 months to 3.5 years,the mortality rate of FIRES and SVE were both high(10.0% vs 20.0%),and they were both left serious neurological sequelae(22.2% vs 31.3%),no difference.Compared with SVE,the epilepsy in the FIRES group is more difficult to treat,the types of AEDs(3.44 ± 0.96 vs 1.71 ± 0.95)are significantly more,and KD treatment in chronic phase has a certain effect.Conclusion:(1)When patients occur on the onset of school age,the interval between fever and first seizure is relatively long.The first seizure is mostly a non-convulsive state,but refractory focal seizures gradually appear,and the frontal area is predominantly focal discharges of EEG,with multifocal discharges during the seizure period.There are no obvious abnormalities in cerebrospinal fluid and head MRI,and the duration of convulsions,fever duration and unconsciousness are longer(mostly more than 2 weeks),and FIRES should be highly vigilant.(2)When convulsions appear soon after fever,especially the status of convulsions,CSF only white blood cell count and protein concentration are mild to moderately elevated,and MRI is more heavily involved,which is multifocal brain lesions,which mainly involve the frontal and temporal lobe,insular lobe,and thalamus,the condition gradually stabilized around 2 weeks,and the tendency to consider the diagnosis of SVE.(3)Compared with SVE,FIRES need more types of AEDs and BSCs in the acute phase of treatment.ICU stays in hospital and ventilator use are longer,means requiring longer intensive care and respiratory support,and the remaining epilepsy is more difficult to treat,KD treatment has a certain effect. |
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