CAJ | 학술논문

国外学者报道了一组既往正常的儿童,在发热后出现难治性部分性癫(癎)或癫(癎)持续状态,预后存在认知功能障碍以及药物抵抗性癫(癎)的临床病例,将其命名为热性感染相关性癫(癎)综合征.目前该病病因不明,病情危重,预后差,脑脊液、脑活检和神经影像学未见特异性改变,尚无明确有效的治疗方法,有报道生酮饮食可能有效.该文就热性感染相关性癫(癎)综合征的临床特点进行综述.
국외학자보도료일조기왕정상적인동,재발열후출현난치성부분성전(간)혹전(간)지속상태,예후존재인지공능장애이급약물저항성전(간)적림상병례,장기명명위열성감염상관성전(간)종합정.목전해병병인불명,병정위중,예후차,뇌척액、뇌활검화신경영상학미견특이성개변,상무명학유효적치료방법,유보도생동음식가능유효.해문취열성감염상관성전(간)종합정적림상특점진행종술.
The scholars abroad have reported a group of previously normal children,who develop intractable focal epilepsy or status epilepticus after fever,followed by drug-resistant partial epilepsy and severe cognitive impairment,named febrile infection-related epilepsy syndrome (FIRES).There are unknown etiology,critical condition,poor prognosis and absent of specific changes on cerebrospinal fluid,biopsies and neuroimagings.No therapeutic agent is efficacious and ketogenic diet may be an alternative therapy in some reports.This study describes the clinical characteristics of FIRES.