癫痫性脑病(Epileptic encephalopathy,EE)是指癫痫活动对脑功能的不良影响超过其潜在的病因。对其潜在的机制知之甚少,但最近的研究表明癫痫发作和发作间期癫痫样放电可以破坏暂时和永久支撑认知功能的神经网络分布。EE只是癫痫中影响发育的多种因素之一。EE所致的认知损害通常难以与潜在的病因或是抗癫痫药物的不良反应区分。这种困难导致越来越多地使用EE来概括“严重的”癫痫综合征或是与严重的癫痫以及智力障碍相关的病因,而不管癫痫影响认知功能的证据。文献中不但使用EE这个术语来描述癫痫活动导致认知损害的过程,也将其归为一类严重的癫痫综合征,这导致了混淆。提议EE这一术语应该严格用于普遍的癫痫过程影响发育这个中心概念,而应该避免使用EE作为分类方式。需要另一个术语来概括广泛且异质的有严重癫痫和智力障碍的患者,其机制可能是未知的,但是通常与潜在的遗传性、代谢性或结构性病因密切相关提高对EE致病机制的认识至关重要,可能有利于明确生物标志物用于早期诊断和治疗。 Epileptic encephalopathy (EE) refers to the adverse effect of epileptic activity on brain function beyond its underlying cause. Little is known about its underlying mechanisms, but recent studies show that epileptic seizures and interictal epileptiform discharges can disrupt neural network distributions that temporarily and permanently support cognitive functions. EE is only one of the many factors that affect development in epilepsy. Cognitive impairment due to EE is often difficult to distinguish from underlying causes or adverse reactions to antiepileptic drugs. This difficulty has led to the increasing use of EE to summarize “severe” epilepsy syndromes or causes associated with severe epilepsy and mental retardation regardless of the evidence that epilepsy affects cognitive function. The literature not only uses the term EE to describe the process of epilepsy activity leading to cognitive impairment, but also classifies it as a type of severe epilepsy syndrome that leads to confusion. The term EE should be used strictly as a central concept of the general epilepsy process that affects development, and EE should be avoided as a taxonomy. There is a need for another term to outline a broad and heterogeneous group of patients with severe epilepsy and mental retardation whose mechanisms may not be known, but are often associated with underlying genetic, metabolic or structural causes that increase the risk of EE pathogenicity Awareness is crucial and may be useful for making clear the use of biomarkers for early diagnosis and treatment.