Francis Rohmer Neurology Epilepsy Units, National Institute of Health and Medical Research 1258, Federation of Translational Medicine of Strasbourg, Strasbourg University, Strasbourg, France.
New York University Grossman School of Medicine and NYU Langone Health, New York, New York, USA.
Epilepsia. 2022 Jun;63(6):1475-1499. doi: 10.1111/epi.17236. Epub 2022 May 3.
In 2017, the International League Against Epilepsy (ILAE) Classification of Epilepsies described the "genetic generalized epilepsies" (GGEs), which contained the "idiopathic generalized epilepsies" (IGEs). The goal of this paper is to delineate the four syndromes comprising the IGEs, namely childhood absence epilepsy, juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic-clonic seizures alone. We provide updated diagnostic criteria for these IGE syndromes determined by the expert consensus opinion of the ILAE's Task Force on Nosology and Definitions (2017-2021) and international external experts outside our Task Force. We incorporate current knowledge from recent advances in genetic, imaging, and electroencephalographic studies, together with current terminology and classification of seizures and epilepsies. Patients that do not fulfill criteria for one of these syndromes, but that have one, or a combination, of the following generalized seizure types: absence, myoclonic, tonic-clonic and myoclonic-tonic-clonic seizures, with 2.5-5.5 Hz generalized spike-wave should be classified as having GGE. Recognizing these four IGE syndromes as a special grouping among the GGEs is helpful, as they carry prognostic and therapeutic implications.
2017 年，国际抗癫痫联盟（ILAE）对癫痫分类进行了描述，其中包含了“特发性全面性癫痫”（IGE）。本文旨在阐述构成 IGE 的四个综合征，即儿童失神癫痫、青少年失神癫痫、青少年肌阵挛癫痫和仅有全面性强直-阵挛发作的癫痫。我们提供了这些 IGE 综合征的最新诊断标准，这些标准是由 ILAE 疾病分类和定义工作组（2017-2021 年）的专家共识意见以及我们工作组之外的国际外部专家确定的。我们结合了遗传、影像学和脑电图研究的最新知识，以及目前的癫痫发作和癫痫分类术语。如果患者不符合其中一种综合征的标准，但有一种或多种以下全面性发作类型：失神发作、肌阵挛发作、强直-阵挛发作和肌阵挛-强直-阵挛发作，伴有 2.5-5.5Hz 的全面性棘波，则应归类为具有 GGE。认识到这四种 IGE 综合征是 GGE 中的一个特殊分组是有帮助的，因为它们具有预后和治疗意义。
