Scheffer Ingrid E, Berkovic Samuel, Capovilla Giuseppe, Connolly Mary B, French Jacqueline, Guilhoto Laura, Hirsch Edouard, Jain Satish, Mathern Gary W, Moshé Solomon L, Nordli Douglas R, Perucca Emilio, Tomson Torbjörn, Wiebe Samuel, Zhang Yue-Hua, Zuberi Sameer M
Department of Medicine, The University of Melbourne, Austin Health, Melbourne, Victoria, Australia.
Department of Paediatrics, Royal Children's Hospital, The University of Melbourne, Melbourne, Victoria, Australia.
Epilepsia. 2017 Apr;58(4):512-521. doi: 10.1111/epi.13709. Epub 2017 Mar 8.
The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world. The new classification originates from a draft document submitted for public comments in 2013, which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type, where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized, and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome, where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis, as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self-limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century.
国际抗癫痫联盟(ILAE)的癫痫分类已更新,以反映自1989年上次批准的分类以来,随着重大科学进展,我们对癫痫及其潜在机制的认识有所增加。作为临床医生的关键工具,癫痫分类必须与思维变化相关且具有动态性,同时要稳健且能在全球所有地区通用。其主要目的是用于患者诊断,但对癫痫研究、抗癫痫疗法的开发以及全球范围内的交流也至关重要。新分类源自2013年提交供公众评论的一份文件草案,经过修订以纳入国际癫痫学界在多轮咨询中提供的大量反馈。它呈现出三个层次,首先是发作类型,在此假定患者正在经历如2017年新的国际抗癫痫联盟发作分类所定义的癫痫发作。在诊断出发作类型后,下一步是诊断癫痫类型,包括局灶性癫痫、全身性癫痫、全身性和局灶性合并癫痫,以及不明癫痫组。第三个层次是癫痫综合征,在此可以做出特定的综合征诊断。新分类在每个阶段都纳入了病因,强调在诊断的每个步骤都需要考虑病因,因为它通常对治疗有重大影响。病因分为六个亚组,因其潜在的治疗后果而被选中。引入了新术语,如发育性和癫痫性脑病。“良性”一词被“自限性”和“药物反应性”所取代,可在适当情况下使用。希望这个新框架将有助于改善21世纪的癫痫护理和研究。