Neuropaediatrics Department, Necker Hospital, APHP, Paris, INSERM U663, Paris, and Faculty of Medicine, Descartes University, Paris, France.
Lancet Neurol. 2011 Jan;10(1):99-108. doi: 10.1016/S1474-4422(10)70214-3.
Fever-induced refractory epileptic encephalopathy in school-aged children (FIRES), and idiopathic hemiconvulsion-hemiplegia syndrome (IHHS) are both triggered by fever, although evidence for a causal microorganism or an autoimmune phenomenon is lacking. FIRES begins in school age with status epilepticus lasting several weeks, involves perisylvian areas including mesial temporal structures, and is followed by pharmacoresistant epilepsy with major cognitive deterioration. IHHS begins in infancy with unilateral clonic status epilepticus and is followed by hemiplegia with pharmacoresistant epilepsy. The aetiology of FIRES and IHHS remains unknown, although clinical features and experimental models point to a likely vicious cycle involving inflammation and seizure activity that depends on the stage of brain maturation. We therefore propose to group these conditions under the concept of acute encephalopathy with inflammation-mediated status epilepticus. In addition to preliminary but encouraging clinical observations, there are theoretical reasons to consider the ketogenic diet as an early means to control both seizures and inflammation.
发热相关性难治性癫痫性脑病（FIRES）和特发性偏瘫性惊厥-偏瘫综合征（IHHS）均由发热引发，尽管缺乏针对其致病微生物或自身免疫现象的确凿证据。FIRES 起病于学龄期，表现为持续数周的癫痫持续状态，累及包括内侧颞叶结构在内的侧脑室周围区域，随后出现药物难治性癫痫和严重认知功能障碍。IHHS 起病于婴儿期，表现为单侧惊厥性癫痫持续状态，随后出现偏瘫和药物难治性癫痫。FIRES 和 IHHS 的病因仍不清楚，尽管临床特征和实验模型提示可能存在涉及炎症和癫痫活动的恶性循环，该循环依赖于大脑成熟阶段。因此，我们建议将这些病症归为伴有炎症介导的癫痫持续状态的急性脑病。除了初步但令人鼓舞的临床观察外，还有理论依据认为生酮饮食可作为早期控制癫痫发作和炎症的手段。
