Instruction manual for the ILAE 2017 operational classification of seizure types

This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail. Key signs and symptoms of seizures (semiology) are used as a basis for categories of seizures that are focal or generalized from onset or with unknown onset. Any focal seizure can further be optionally characterized by whether awareness is retained or impaired. Impaired awareness during any segment of the seizure renders it a focal impaired awareness seizure. Focal seizures are further optionally characterized by motor onset signs and symptoms: atonic, automatisms, clonic, epileptic spasms, or hyperkinetic, myoclonic, or tonic activity. Nonmotor‐onset seizures can manifest as autonomic, behavior arrest, cognitive, emotional, or sensory dysfunction. The earliest prominent manifestation defines the seizure type, which might then progress to other signs and symptoms. Focal seizures can become bilateral tonic–clonic. Generalized seizures engage bilateral networks from onset. Generalized motor seizure characteristics comprise atonic, clonic, epileptic spasms, myoclonic, myoclonic–atonic, myoclonic–tonic–clonic, tonic, or tonic–clonic. Nonmotor (absence) seizures are typical or atypical, or seizures that present prominent myoclonic activity or eyelid myoclonia. Seizures of unknown onset may have features that can still be classified as motor, nonmotor, tonic–clonic, epileptic spasms, or behavior arrest. This “users’ manual” for the ILAE 2017 seizure classification will assist the adoption of the new system.     

ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology

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.     

Epileptic seizures and epilepsy: definitions proposed by the International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE)

The International League Against Epilepsy (ILAE) and the International Bureau for Epilepsy (IBE) have come to consensus definitions for the terms epileptic seizure and epilepsy. An epileptic seizure is a transient occurrence of signs and/or symptoms due to abnormal excessive or synchronous neuronal activity in the brain. Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures and by the neurobiologic, cognitive, psychological, and social consequences of this condition. The definition of epilepsy requires the occurrence of at least one epileptic seizure.     

癫癎及癫癎综合征国际分类(1989年)的临床应用分析

目的探讨各类癫和癫综合征在就诊人群中的分布,以利于癫患者的诊治。方法按国际抗癫联盟(ILAE)1989年推荐的癫和癫综合征的分类方案对门诊患者进行调查。结果1191例患者中,部分性癫和综合征766例(64·3%),全面性癫和综合征240例(20·2%),不能确定为部分性或全面性的癫及综合征79例(6·7%),特殊综合征16例(1·3%),其他非癫疾患90例(7·6%)。在部分性癫和综合征中,症状性者占了绝大多数,而全面性癫和综合征中,特发性者占绝大多数。结论正确地进行癫分类对临床有重要的指导意义,各种癫综合征的分布是有规律可循的。     

国际抗癫(癎)联盟国际癫(癎)综合征分类可分类率比较及架构分析

目的 比较国际抗癫(癎)联盟(International League Against Epilepsy,ILAE)1989、2001、2006年癫(癎)综合征分类的可分类率并分析架构变化.方法 先后依据3个分类对2007年8月1日至2008年3月31日连续就诊于北京协和医院癫(癎)中心的1356例癫(癎)患者进行癫(癎)综合征诊断,并进行统计学分析.结果 3个分类的可分类率分别为75.5 % 、89.0 % 和88.1 % ,2001及2006年分类明显优于1989年分类(x2=116.3,P<0.01);对特异综合征的可分类率分别为11.6 % (157例)、12.0 % (162例)及11.9 % (160例),差异无统计学意义(x2=0.09,P>0.05).结论 ILAE 2006年癫(癎)综合征分类引入了较科学的分类模式和评价体系,提高了癫(癎)的可分类率,可尝试应用于临床及基础研究.     

Experience with the International League Against Epilepsy Proposals for Classification of Epileptic Seizures and the Epilepsies and Epileptic Syndromes in a Pediatric Outpatient Epilepsy Clinic

The International League Against Epilepsy proposals for classification of epileptic seizures (1981) and of the epilepsies and epileptic syndromes (1985) have been used in daily practice in a pediatric epilepsy clinic in Bogota, Colombia. Most patients can be classified by these schemes, and the classifications are useful in everyday diagnosis and management. However, there are some drawbacks and difficulties with the classifications. Some syndromes are unnecessarily separated as different entities, artificially contributing to the complexity of the Classification.     

国际癫癎发作分类建议(2001年)在部分性发作中的应用

目的探讨国际抗癫联盟关于癫发作分类的建议(2001年)在部分性癫发作中的应用价值和特点。方法收集1996年6月至2004年10月在我院癫中心就诊,经过录像脑电监测捕捉到发作的128例部分性癫患者的发作情况,应用1981、2001年两种癫发作分类方案,由3位受过正规癫和脑电图训练的神经科医生进行分类。结果128例患者共检测到298次发作。按照1981年分类标准,97次(32%)属于简单部分性发作,105次(35%)为复杂部分发作,12次(4%)部分性发作难以准确划分为简单或者复杂性,81次(27%)为继发全面性发作,3次(1%)为部分性癫持续状态;应用2001年分类建议,有295次发作为自限性局灶性发作,其中67次(22%)为局灶性感觉性发作,140次(47%)为局灶性运动性发作,包括81次(27%)伴随颞叶自动症的局灶运动性发作,81次(27%)为继发全面性发作,3次为局灶性癫持续状态发作。2001年分类方案中,发作类型体现了特征性的临床症状,与发作期的癫性放电的解剖部位有紧密的对应,但是,高级皮层的发作症状在一定程度上受到了忽略。结论2001年国际癫发作分类体现了良好的发作症状、发作类型、解剖部位之间的联系,可以试用于临床,但需要进一步的完善。     

国际癫痫发作分类建议（2001年）在部分性发作中的应用

目的 探讨国际抗癫痫联盟关于癫痫发作分类的建议（2001年）在部分性癫痫发作中的应用价值和特点。方法收集1996年6月至2004年10月在我院癫痫中心就诊，经过录像脑电监测捕捉到发作的128例部分性癫疴患者的发作情况，应用1981、2001年两种癫痫发作分类方案，由3位受过正规癫痫和脑电图训练的神经科医生进行分类。结果128例患者共检测到298次发作。按照1981年分类标准，97次（32％）属于简单部分性发作，105次（35％）为复杂部分发作，12次（4％）部分性发作难以准确划分为简单或者复杂性，81次（27％）为继发全面性发作，3次（1％）为部分性癫痫持续状态；应用2001年分类建议，有295次发作为自限性局灶性发作，其中67次（22％）为局灶性感觉性发作，140次（47％）为局灶性运动性发作，包括81次（27％）伴随颞叶自动症的局灶运动性发作，81次（27％）为继发全面性发作，3次为局灶性癫痫持续状态发作。2001年分类方案中，发作类型体现了特征性的临床症状，与发作期的癫痫性放电的解剖部位有紧密的对应，但是，高级皮层的发作症状在一定程度上受到了忽略。结论2001年国际癫痫发作分类体现了良好的发作症状、发作类型、解剖部位之间的联系，可以试用于临床，但需要进一步的完善。     

国际抗癫(痈)联盟2017年发布的癫(痈)发作和癫(痈)新分类介绍

文中主要介绍国际抗癫(痈)联盟2017年发布的新的癫(痈)分类及术语.     

国际抗癫痫联盟关于发作和癫痫分类框架术语及概念最新修订版的解读

国际抗癫痫联盟(International League Against Epilepsy,ILAE)分类和术语委员会2010年制定的发作和癫痫分类框架术语及概念修订版旨在进一步建立和规范发作和癫痫分类术语及概念的国际共识,反映术语与分类的基本关系,以指导临床实践,尤其是指导制定更合适的治疗方案。对于以往的全面性发作和局灶性发作分类法,目前强调依据网络的起源方式重新进行定义,其中双侧起源为全面性发作,局限于一侧为局灶性发作。此外,建议使用遗传性、结构性/代谢性和未知病因这3个术语以替代原来的特发性、症状性和隐源性。新的报告还首次使用了一些特定的术语,如电-临床综合征、相对明确的癫痫群体和癫痫性脑病等。本文对ILAE关于发作和癫痫分类框架术语及概念最新修订版进行了解读。     

Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009

The International League Against Epilepsy (ILAE) Commission on Classification and Terminology has revised concepts, terminology, and approaches for classifying seizures and forms of epilepsy. Generalized and focal are redefined for seizures as occurring in and rapidly engaging bilaterally distributed networks (generalized) and within networks limited to one hemisphere and either discretely localized or more widely distributed (focal). Classification of generalized seizures is simplified. No natural classification for focal seizures exists; focal seizures should be described according to their manifestations (e.g., dyscognitive, focal motor). The concepts of generalized and focal do not apply to electroclinical syndromes. Genetic, structural–metabolic, and unknown represent modified concepts to replace idiopathic, symptomatic, and cryptogenic. Not all epilepsies are recognized as electroclinical syndromes. Organization of forms of epilepsy is first by specificity: electroclinical syndromes, nonsyndromic epilepsies with structural–metabolic causes, and epilepsies of unknown cause. Further organization within these divisions can be accomplished in a flexible manner depending on purpose. Natural classes (e.g., specific underlying cause, age at onset, associated seizure type), or pragmatic groupings (e.g., epileptic encephalopathies, self‐limited electroclinical syndromes) may serve as the basis for organizing knowledge about recognized forms of epilepsy and facilitate identification of new forms.     

Disparities in epilepsy: Report of a systematic review by the North American Commission of the International League Against Epilepsy

Purpose:   We undertook a systematic review of the evidence on disparities in epilepsy with a focus on North American data (Canada, United States, and the English-speaking Caribbean).
Methods:   We identified and evaluated: access to and outcomes following medical and surgical treatment, disability, incidence and prevalence, and knowledge and attitudes. An exhaustive search (1965–2007) was done, including: (1) disparities by socioeconomic status (SES), race/ethnicity, age, or education of subgroups of the epilepsy population; or (2) disparities between people with epilepsy (PWE) and healthy people or with other chronic illnesses.
Results:   From 1,455 citations, 278 eligible abstracts were identified and 44 articles were reviewed. Comparative research data were scarce in all areas. PWE have been shown to have lower education and employment status; among PWE, differences in access to surgery have been shown by racial/ethnic groups. Aboriginals, women, and children have been shown to differ in use of health resources. Poor compliance has been shown to be associated with lower SES, insufficient insurance, poor relationship with treating clinicians, and not having regular responsibilities.
Discussion:   Comprehensive, comparative research on all aspects of disparities in epilepsy is needed to understand the causes of disparities and the development of any policies aimed at addressing health disparities and minimizing their impact.     

解读国际抗癫痫联盟分类和术语委员会对发作和癫痫分类框架术语及概念修订的最新报告(2010)

自1964年Gastaut[1]开始尝试对癫痫进行国际化的分类以来,国际抗癫痫联盟(international league against epilepsy,ILAE)任命的每一届分类和学术委员会(以下简称委员会)都会顺应时代的要求,对癫痫的国际分类和术语进行修订,力求使新修订的分类和术语能完全反映癫痫领域内基础和临床研究的最新进展,例如ILAE 1981年癫痫发作临床和脑电图的分类[2]、1989年癫痫和癫痫综合征分类[3]以及2001年癫痫发作和癫痫诊断方案的建议[4]等,这些文件都已由国内的学者翻译并介绍给了大家.     

Idiopathic Generalized Epilepsies Recognized by the International League Against Epilepsy

Summary:  There are eight syndromes currently recognized by the International League Against Epilepsy (ILAE) that would fit the original operational definition of idiopathic generalized epilepsy (IGE) syndromes, including benign myoclonic epilepsy in infancy; generalized epilepsy with febrile seizures plus, an entity in evolution; epilepsy with myoclonic absences; epilepsy with myoclonic-astatic seizures; childhood absence epilepsy; juvenile absence epilepsy; juvenile myoclonic epilepsy; and epilepsy with generalized tonic–clonic seizures only. All of these syndromes can be easily diagnosed when distinctive features are present. In some cases, such features are not present or only appear later in the course of the disease, making it challenging to distinguish the various syndromes. Electroencephalogram (EEG) is the most helpful laboratory test and often will strongly support the diagnosis of IGE, but may not be very helpful in discriminating between several of the syndromes with overlapping features. The same applies for genetic testing, although it is expected that further research exploring the genotype-phenotype relationships will enhance our abilities to make definitive diagnoses. At the current time, clinical features are still the cornerstone of accurate classification, and accurate classification, in turn, is the best predictor of outcome.     

Relative Frequency of Different Types of Epilepsy: A Study Employing the Classification of the International League Against Epilepsy

Approximately three-quarters of epileptic patients seen in consultation by specialists can easily be assigned to one of the diagnostic categories of the International League against Epilepsy. When patient age is taken into account, such a distribution shows that partial epilepsy is more common than generalized epilepsy (62% versus 38%) in patients of all ages, especially in those over 15 years of age (78% versus 22%). In contrast, generalized epilepsy is more frequent in subjects under 15 than partial epilepsy (55% versus 45%). In this younger age group the rarity of partial epilepsy is primarily due to the infrequency of temporal lobe epilepsy (21% of cases, as opposed to 56% in subjects over 15), whereas both primary and secondary generalized epilepsy contribute to the frequency of generalized epilepsy in this group.