Patients with temporal lobe epilepsy (TLE) due to mesial temporal sclerosis (MTS) are eligible candidates for resective epilepsy surgery. We report on 2 male patients aged 4 years with suspected TLE due to MTS who were referred for presurgical evaluation. Both patients came to medical attention within the first year of life suffering from febrile status epileptici and subsequent unprovoked seizures. The following years, moderate developmental delay was present. High-resolution magnetic resonance imaging confirmed hippocampal sclerosis. Continuous EEG video monitoring revealed seizure patterns contralateral to the MTS in both patients. Genetic analysis was performed as both the clinical presentation of the patients and EEG video monitoring findings were not consistent with the presence of the hippocampal sclerosis alone and revealed de novo mutations within exon of the SCN1A gene. Resective surgical strategies were omitted due to the genetic findings. In conclusion, both patients suffered from a dual pathology syndrome with (a) TLE related to MTS resulting most likely from recurrent febrile status in early childhood and (b) Dravet syndrome, which is most likely the cause of the febrile convulsions leading to the MTS in these 2 patients.

中文翻译：

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SCN1A 相关癫痫中的内侧颞叶硬化：两个长期脑电图案例研究

由于颞叶内侧硬化 (MTS) 导致的颞叶癫痫 (TLE) 患者适合进行癫痫切除手术。我们报告了 2 名 4 岁男性患者，他们因 MTS 疑似 TLE，被转诊进行术前评估。两名患者在出生后的第一年内都因发热性癫痫持续状态和随后的无端癫痫发作而就医。接下来的几年，出现了中度发育迟缓。高分辨率磁共振成像证实了海马硬化。连续 EEG 视频监测显示两名患者的 MTS 对侧癫痫发作模式。进行基因分析是因为患者的临床表现和 EEG 视频监测结果与单独的海马硬化症的存在不一致，并揭示了 SCN1A 基因外显子内的新生突变。由于遗传发现，切除手术策略被省略。总之，这两名患者都患有双重病理综合征，其中 (a) 与 MTS 相关的 TLE 最有可能是由儿童早期反复发热状态引起的，以及 (b) Dravet 综合征，这很可能是导致 MTS 的热性惊厥的原因在这 2 例患者中。