Department of Pediatric Neurology, Referral Center for Rare Epilepsies, Necker Infants Maladies Hospital, APHP, 149 rue de Sévres, Paris, France.
Epilepsia. 2013 Sep;54 Suppl 6:54-6. doi: 10.1111/epi.12278.
Idiopathic hemiconvulsion hemiplegia and epilepsy syndrome (IHHE) and febrile infection-related epilepsy syndrome (FIRES) are rare epileptic syndromes characterized by the occurrence of status epilepticus in a previously healthy child during or closely after a febrile episode. In both syndromes, there is no evidence of central nervous system infection (encephalitis) and the etiology remains unclear. Treatment is disappointing, particularly in FIRES, except for a response to ketogenic diet (KD) in half of patients. In IHHS, children develop hemispheric brain atrophy with contralateral hemiplegia, epilepsy, and a variable degree of cognitive deficit. Patients with FIRES develop refractory epilepsy with severe cognitive deficit affecting the temporal and frontal lobe functions. The role of inflammation is hypothesized with a vicious circle involving inflammation and seizure activity facilitated by brain maturation putting them under the concept of "acute encephalopathy with inflammation-mediated status epilepticus."
特发性偏瘫性癫痫伴偏瘫痉挛发作综合征（IHHE）和热性感染相关性癫痫持续状态（FIRES）是两种罕见的癫痫综合征，其特征是在先前健康的儿童发热期间或发热后不久发生癫痫持续状态。在这两种综合征中，均无中枢神经系统感染（脑炎）的证据，其病因仍不清楚。治疗效果不佳，FIRES 更是如此，除了有一半患者对生酮饮食（KD）有反应。在 IHHS 中，儿童会出现半球性脑萎缩伴对侧偏瘫、癫痫和不同程度的认知缺陷。FIRES 患者会出现难治性癫痫和严重认知缺陷，影响颞叶和额叶功能。炎症的作用被假设为一个恶性循环，包括炎症和癫痫活动，这是由大脑成熟促进的，使它们处于“伴有炎症介导的癫痫持续状态的急性脑病”的概念之下。
