Monographic Research • Diagnosis and Treatment of Epilepsy in Children
Diagnosis and treatment of 12 cases of febrile infection-related epilepsy syndrome
Wang Guoli, Deng Xiaolu, Peng Jing, Wang Xia, Wu Liwen, Zhang Ciliu, Yang Lifen, Yin Fei, He Fang
Published 2019-09-20
Cite as J Chin Physician, 2019,21(9): 1297-1301. DOI: 10.3760/cma.j.issn.1008-1372.2019.09.004
Abstract
ObjectiveTo investigate the clinical features, diagnosis and treatment of febrile infection-related epilepsy syndrome (FIRES).
MethodsThe 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.
ResultsOf 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.
ConclusionsFIRES 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.
Key words:
Acute febrile encephalopathy; Status epilepticus; Drug resistant epilepsy; Ketogenic diet; Child
Contributor Information
Wang Guoli
Department of Pediatrics, Xiangya Hospital, Central South University, Changsha 410008, China
Deng Xiaolu
Peng Jing
Wang Xia
Wu Liwen
Zhang Ciliu
Yang Lifen
Yin Fei
He Fang