Diagnosis and treatment of 12 cases of febrile infection-related epilepsy syndrome
10.3760/cma.j.issn.1008-1372.2019.09.004
- VernacularTitle: 发热感染相关性癫痫综合征12例诊治分析及治疗进展
- Author:
Guoli WANG
1
;
Xiaolu DENG
;
Jing PENG
;
Xia WANG
;
Liwen WU
;
Ciliu ZHANG
;
Lifen YANG
;
Fei YIN
;
Fang HE
Author Information
1. Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
- Publication Type:Journal Article
- Keywords:
Acute febrile encephalopathy;
Status epilepticus;
Drug resistant epilepsy;
Ketogenic diet;
Child
- From:
Journal of Chinese Physician
2019;21(9):1297-1301
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES).
Methods:The clinical data of 12 children with FIRES admitted to Xiangya Hospital of Central South University from 2015 to 2018 were retrospectively analyzed. The basic information, clinical manifestations, electroencephalogram, imaging examination, treatment and prognosis were analyzed.
Results:Of the 12 patients, 7 were male and 5 were female. The age of onset was (7.0±3.7)years (1.3 year to 13 years). The average hospitalization time (34-86 days, median 52 days). Twelve patients were healthy before the disease, and had fever before convulsion. The interval between fever and seizure was (3.5±1.7)days (1-7 days). The status epilepticus and consciousness deficit were the main clinical manifestations. The electrogram of 8 patients showed status epilepticus when admitted. 12 patients had disturbance of consciousness; the acute episodes were focal seizures (100%, 12/12) and generalized tonic-clonic seizures (41.7%, 5/12). All patients used 3-5 antiepileptic drugs (median 4), all treated with hormones and gamma globulin. 4 patients with ketogenic diet (KD) were treated within 2 weeks of onset, and the average duration from onset to electroencephalogram (EEG) improvement was (19.2±5.0)days. In 8 patients who did not use KD within 2 weeks of onset, the average duration from onset to EEG improvement was (29.9±9.6)days.
Conclusions:FIRES is more common in normal children with school age. The main manifestation is refractory status epilepticus in the days after acute fever, focal episodes of seizures, anti-epileptic drug resistance. Early initiation of KD produces a favorable prognosis.