title: [Fever-induced refractory epileptic encephalopathy in school-aged children: clinical features and outcome-a multicenter study on 13 children].
abstract: OBJECTIVE
  To analyze the clinical features and outcomes of fever-induced refractory epileptic encephalopathy in school-aged children (FIRES) in China.
  
  
  METHODS
  A retrospective multicenter study on 13 FIRES patients was conducted. By collecting the medical data and follow up research of these patients, the clinical manifestations, clinical courses, investigations, treatments and outcomes of FIRES patients were analyzed.
  
  
  RESULTS
  The 13 enrolled patients were previously healthy. The median age at onset was 8.3 years. The male-to-female ratio was 6:7. They exhibited with prolonged refractory status epilepticus after fever onset. All the patients presented with partial seizures, 9 of them had secondary generalized epilepsy; 12 patients had EEG data, 6 of them showed asymmetric diffused slow wave. Seven patients presented with focal or multifocal discharges mostly located in frontotemproal areas. n acute period, many seizures (up to 100) could occur each day. Between seizures, the patients were drowsy or even comatose. Neuroimaging demonstrated normal findings in 10 children. Classical anti-epileptic drugs (AED, 3-7 kinds, median 5) were ineffective; 3 children received anesthesia treatment.Eight patients were admitted to ICU, 6 of them needed mechanical ventilation. One patient died 1 week after discharge. n follow up period, most patients were in a stable and chronic condition. Seizures were controlled by AED only in 1 child. Parents of 1 patient gave up all treatment but the patient still had seizure. Other 10 patients had refractory epilepsy that could not be controlled by 3-4 AEDs. We collected neuroimaging data of 11 patients in follow up period, 1 of the 9 previously normal patients exhibited temporal lobe atrophy, others did not show any change compared with that in acute phase. Only 3 of the 12 surviving patients retained normal cognitive level but all of them could not recover to pre-morbidity status.
  
  
  CONCLUSIONS
  FIRES is a devastating epileptic encephalopathy with unknown cause. Different kinds of anti-epileptic treatments were given without satisfactory effect, even extensive treatment efforts including intensive care were unsuccessful. Thus the outcome is disastrous in most of the previously healthy children.
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D002648:Child; D004569:Electroencephalography; D005260:Female; D005334:Fever / Q000150:complications*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D011859:Radiography; D012189:Retrospective Studies; D003294:Seizures, Febrile / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D013226:Status Epilepticus / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants