Fortini Sebastian, Espeche Alberto, Galicchio Santiago, Cersósimo Ricardo, Chacon Santiago, Gallo Adolfo, Gamboni Beatriz, Adi Javier, Fasulo Lorena, Semprino Marcos, Cachia Pedro, Caraballo Roberto H
Hospital del Niño Jesús, Tucumán, Argentina.
Hospital Público Materno Infantil de Salta, Argentina.
Epilepsy Res. 2021 Nov;177:106768. doi: 10.1016/j.eplepsyres.2021.106768. Epub 2021 Sep 17.
We describe the evolution of the electroclinical picture of patients with different types of self-limited epilepsy of childhood (SLEC) occurring at the same or at different times with or without atypical evolutions as well as patients with SLEC associated with childhood absence epilepsy (CAE).
A multicenter, retrospective, descriptive study was conducted evaluating patients with SLEC who had focal seizures of different types of SLEC including atypical evolutions as well as SLEC associated with absence epilepsy seen at eight Argentinian centers between April 2000 and April 2019. Of 7705 patients with SLEC, aged between 2 and 14 years (mean, 7.5 years), of whom 2013 were female and 5692 male (ratio, 1:2.8), 5068 patients had SLECTS, 2260 patients had self-limited childhood occipital epilepsy Panayiotopoulos type (SLE-P), 356 had self-limited childhood occipital epilepsy Gastaut type (SLE-G), and 21 had self-limited epilepsy with affective seizures (SLEAS). Electroclinical features typical of more than one SLEC syndrome were recognized in 998 (13 %) children.
We recognized three well-defined groups of patients. The most frequent association was SLE-P and SLECTS, the paradigmatic type, but associations of SLE-P and SLE-G, SLECTS and SLE-G, and SLEAS and SLE-P or SLECTS were also recognized. The second-most-common association was SLEC and an atypical evolution. In this group, the most frequent combination was SLECTS with its atypical evolution, opercular status epilepticus, epileptic encephalopathy with continuous spike-and-waves during slow sleep, or Landau-Kleffner syndrome. SLE-P and SLE-G associated with an atypical evolution were also identified. The third, less-frequent group had SLECTS, SLE-P, or SLE-G associated with CAE. These cases support the concept that the different types of SLEC are part of a self-limited childhood seizure susceptibility syndrome.
Our study demonstrated that 13 % of our patients with SLEC have with different types of SLEC occurring at the same or at different times with or without atypical evolutions - i.e. CSWSS - as well as patients with SLEC associated with CAE, supporting the concept of the self-limited childhood seizure susceptibility syndrome.
我们描述了不同类型儿童自限性癫痫（SLEC）患者的临床电生理特征演变情况，这些患者在相同或不同时间发病，有或无非典型演变，以及与儿童失神癫痫（CAE）相关的SLEC患者。
进行了一项多中心、回顾性、描述性研究，评估2000年4月至2019年4月期间在阿根廷八个中心就诊的SLEC患者，这些患者有不同类型SLEC的局灶性发作，包括非典型演变情况以及与失神癫痫相关的SLEC。在7705例年龄在2至14岁（平均7.5岁）的SLEC患者中，2013例为女性，5692例为男性（比例为1:2.8），其中5068例患有SLECTS，2260例患有儿童自限性枕叶癫痫Panayiotopoulos型（SLE-P），356例患有儿童自限性枕叶癫痫Gastaut型（SLE-G），21例患有伴情感性发作的自限性癫痫（SLEAS）。998例（13%）儿童具有不止一种SLEC综合征的典型临床电生理特征。
我们识别出三组明确的患者。最常见的关联是SLE-P和SLECTS，即典型类型，但也识别出SLE-P和SLE-G、SLECTS和SLE-G以及SLEAS和SLE-P或SLECTS的关联。第二常见的关联是SLEC和非典型演变。在这组中，最常见的组合是SLECTS及其非典型演变、岛盖部癫痫持续状态、慢波睡眠期持续棘慢波癫痫性脑病或Landau-Kleffner综合征。也识别出与非典型演变相关的SLE-P和SLE-G。第三组较少见，包括与CAE相关的SLECTS、SLE-P或SLE-G。这些病例支持了不同类型SLEC是儿童自限性癫痫易感性综合征一部分的概念。
我们的研究表明，13%的SLEC患者有不同类型的SLEC在相同或不同时间发病，有或无非典型演变——即慢波睡眠期持续棘慢波癫痫性脑病——以及与CAE相关的SLEC患者，支持了儿童自限性癫痫易感性综合征的概念。
