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脑电图在癫痫综合征诊断和分类中的作用:国际抗癫痫联盟神经生理学特别工作组的临床实践工具(第1部分)

The role of EEG in the diagnosis and classification of the epilepsy syndromes: a tool for clinical practice by the ILAE Neurophysiology Task Force (Part 1).

作者信息

Koutroumanidis Michalis, Arzimanoglou Alexis, Caraballo Roberto, Goyal Sushma, Kaminska Anna, Laoprasert Pramote, Oguni Hirokazu, Rubboli Guido, Tatum William, Thomas Pierre, Trinka Eugen, Vignatelli Luca, Moshé Solomon L

机构信息

GSTT, Clin Neurophysiology and Epilepsies, St Thomas' Hospital, London, UK.

University Hospitals of Lyon, Department of Clinical Epileptology, Sleep Disorders and Functional Neurology in Children, Lyon, France.

出版信息

Epileptic Disord. 2017 Sep 1;19(3):233-298. doi: 10.1684/epd.2017.0935.

Abstract

The concept of epilepsy syndromes, introduced in 1989, was defined as "clusters of signs and symptoms customarily occurring together". Definition of epilepsy syndromes based on electro-clinical features facilitated clinical practice and, whenever possible, clinical research in homogeneous groups of patients with epilepsies. Progress in the fields of neuroimaging and genetics made it rapidly clear that, although crucial, the electro-clinical description of epilepsy syndromes was not sufficient to allow much needed development of targeted therapies and a better understanding of the underlying pathophysiological mechanisms of seizures. The 2017 ILAE position paper on Classification of the Epilepsies recognized that "as a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking". The concept of "epilepsy syndromes" evolved, incorporating issues related to aetiologies and comorbidities. A comprehensive update (and revision where necessary) of the EEG diagnostic criteria in the light of the 2017 revised terminology and concepts was deemed necessary. The work was commissioned by the Neurophysiology Task Force of the ILAE Committee on the Diagnostic Methods. Diagnostic criteria and recording procedures were developed by group consensus, reached through an "informal", internal decision-making process. Each working group member was allocated a number of syndromes, and a standard structured template was used. International literature was extensively reviewed. We developed a simple diagnostic system that is applicable to all epilepsy syndromes which allows the physician (i) to rate the strength of EEG diagnosis (degree of diagnostic certainty) by weighting EEG findings in relation to the available clinical information or the specific clinical question, and ii) to suggest further EEG diagnostics where conclusive diagnostic evidence is lacking. We also propose a system of syndrome-specific recording protocols that, used with the relevant clinical presentation or specific clinical question, may maximize activation of epileptic discharges and ultimately help with standardization of EEG recording across departments, worldwide. Because recording methodology also depends on available resources, a two-tier system was developed to embrace clinical EEG services in resource-limited and industrialized countries. A clinical practice statement for each of the epilepsy syndromes discussed underscores the crucial role of the clinical information with regards to both the optimization of the EEG recording and mainly its meaningful interpretation. Part I covers Genetic (Idiopathic) generalized epilepsies and syndromes, Reflex epilepsies, structural and genetic focal (lobar) syndromes and Progressive Myoclonus Epilepsies [Published with educational EEG plates on www.epilepticdisorders.com].

摘要

癫痫综合征的概念于1989年提出,被定义为“通常一起出现的一组体征和症状”。基于电临床特征对癫痫综合征进行定义有助于临床实践,并在可能的情况下,推动对癫痫患者同质性群体的临床研究。神经影像学和遗传学领域的进展很快表明,尽管癫痫综合征的电临床描述至关重要,但不足以推动急需的靶向治疗的发展,也无法更好地理解癫痫发作的潜在病理生理机制。2017年国际抗癫痫联盟(ILAE)关于癫痫分类的立场文件认识到,“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”。“癫痫综合征”的概念不断演变,纳入了与病因和共病相关的问题。鉴于2017年修订的术语和概念,对脑电图诊断标准进行全面更新(必要时进行修订)被认为是必要的。这项工作由ILAE诊断方法委员会神经生理学特别工作组委托进行。诊断标准和记录程序通过“非正式”的内部决策过程达成小组共识而制定。每个工作组成员被分配了一些综合征,并使用了标准的结构化模板。对国际文献进行了广泛综述。我们开发了一个适用于所有癫痫综合征的简单诊断系统,该系统使医生能够:(i)通过权衡脑电图结果与可用临床信息或特定临床问题的关系,对脑电图诊断的强度(诊断确定性程度)进行评分;(ii)在缺乏确凿诊断证据时,建议进一步的脑电图诊断。我们还提出了一个针对特定综合征的记录方案系统,该系统与相关临床表现或特定临床问题一起使用,可能会最大限度地激活癫痫放电,并最终有助于全球各部门脑电图记录的标准化。由于记录方法也取决于可用资源,因此开发了一个两级系统,以涵盖资源有限国家和工业化国家的临床脑电图服务。针对所讨论的每种癫痫综合征的临床实践声明强调了临床信息在优化脑电图记录以及主要在其有意义的解释方面的关键作用。第一部分涵盖遗传性(特发性)全身性癫痫和综合征、反射性癫痫、结构性和遗传性局灶性(叶性)综合征以及进行性肌阵挛癫痫[已在www.epilepticdisorders.com上发布带有教学脑电图图谱]。

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