癫痫性脑病癫痫脑病定义

癫痫性脑病

癫痫性脑病

●Introduction

Definition

Explanation

●Examples

●Concluding Remarks

癫痫性脑病

Epileptic Encephalopathy

●脑病： Encephalo-pathy

●癫痫性脑病：

癫痫 Epileptic + 脑病

癫痫所致（相关）的脑病

X脑病所致（相关）的癫痫

ILAE 2001

ILAE 2001

Epileptic Encephalopathy

A condition in which the epileptiform

abnormalities themselves are believed to contribute to the progressive disturbance

in cerebral function.

●脑功能障碍，进展性

●癫痫性异常（放电本身）所致

ILAE 2001

Summary of key recommendations from the ILAE 2009

Commission on Classification and Terminology

– For Discussion at the ICE in Budapest

Epileptic Encephalopathy

●Embodies the notion that

epileptic activity lead to severe cognitive and behavioral impairment above and beyond what from the underlying pathology (e.g. cortical malformation) alone

（癫痫样活动本身的意义，而不是基础病因）

seizures themselves contribute to the cognitive impairments

（发作本身的意义）

●Although may occur at any age, most common and severe in the developing brain

（发育期）

Epileptic Encephalopathy

●Inherent in this concept is the idea that

by suppressing or preventing the epileptic activity, one

may improve the cognitive and behavioral outlook of the

disorder. （干预的意义）

In the developing brain, rapid effective intervention should and can be used before the abnormal epileptic activity

interferes irrevocably with normal processes of brain

development. （快速有效的干预）

●The best known and most common of these are West, LGS, Dravet, LKS-CSWS, and Doose syndromes.

（包括多个综合征-有待更多研究）

癫痫性脑病

Definition

Explanation

●癫痫性脑病

具有相对独特而复杂的临床脑电特征

精神行为认知损伤

●癫痫样活动本身所致

●独立于基础脑疾病

包括一组预后不良的综合征

●West, LGS, Dravet, LKS-CSWS, and Doose, etc.

及时干预可能改善认知的转归

多见于发育时期

癫痫性脑病

●Introduction to Epileptic Encephalopathy ●Examples: Epileptic Encephalopathies

Ohtahara Syndrome

Early myoclonic encephalopathy （EME） Dravet Syndrome

Lennox-Gastaut Syndrome

●Concluding Remarks

癫痫和癫痫综合征分类

（简化，ILAE 2001）

将各种癫痫综合征分成8组

●婴儿和儿童特发性局灶性癫痫(4)

●家族性（常染色体显性遗传）局灶性癫痫(5)●症状性（或可能为症状性）局灶性癫痫(5+)●特发性全面性癫痫(8+)

●反射性癫痫(3+)

●癫痫性脑病(8)

●进行性肌阵挛癫痫

●可不诊断为癫痫的发作(8)