关键词: 热性感染相关性癫痫综合征, 难治性癫痫, 诊治

Abstract: Objective To summarize the clinical manifestations， electroencephalogram and MRI features of febrile infection-related epilepsy syndrome（FIRES），and to explore its pathogenesis and current treatment progress. Methods The clinical manifestations，EEG and head MRI features of a child with FIRES in neurology department of Wuhan Children′s Hospital were analyzed. The literatures were reviewed；the clinical，EEG，head MRI features，and diagnosis and treatment progress were summarized. Results The child was school- age male with acute onset， who showed fever，disturbance of consciousness and drug- refractory status epilepticus. The EEG showed that the background was diffuse slow wave activity. In EEG，the partial episodes of left frontal and anterior temporal regions were monitored. There was no abnormality in the MRI signal of the brain. After three days of treatment with ketogenic diet（KD），the seizure was significantly reduced and the patient was transferred to the general ward. After discharge，KD and anti-epileptic drugs were continued. After one month，the return visit showed the child was occasionally convulsed and the consciousness improved，intelligence was significantly lower than that before onset. The literature shows that the disease mainly occurs in previously healthy school-age children，often induced by fever，followed by refractory status epilepticus or cluster-like seizures. FIRES has a poor prognosis. Survivors often have drug-refractory epilepsy and severe cognitive impairment. KD is generally considered to have a significant effect on patients with FIRES. Conclusion The cause of FIRES is unknown and may be caused by immune mediation. FIRES always occurs in school-age children，often induced by fever， its seizures are fulminant，it is drug-refractory，KD is effective in controlling acute seizures and improving prognosis.

Key words: febrile infection-related epilepsy syndrome（FIRES）, refractory epilepsy, diagnosis and treatment