Department of Pediatric Neurology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Department of Pathology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Epilepsy Behav. 2020 Nov;112:107380. doi: 10.1016/j.yebeh.2020.107380. Epub 2020 Sep 1.
The distribution of hippocampal sclerosis (HS) subtypes, according to the classification of the International League Against Epilepsy (ILAE), has been reported mainly in adult patients. We aimed to review the pathological findings in children who had anterior temporal lobectomy accompanied with amygdalohippocampectomy, in view of the current classification, and evaluate postsurgical outcome with respect to HS subtypes in childhood.
Seventy children who underwent temporal resections for treatment of medically refractory epilepsy, with a minimum follow-up of 2 years, were included; the surgical hippocampus specimens were re-evaluated under the HS ILAE classification.
Neuropathological evaluations revealed HS type 1 in 38 patients (54.3%), HS type 2 in 2 (2.8%), HS type 3 in 21 patients (30%), and no HS in 9 patients (12.9%). Of 70 patients, 23 (32.9%) had dual pathology, and the most common pattern was HS type 3 with low-grade epilepsy-associated brain tumors (LEAT). The distribution of HS types with respect to age revealed that HS type 3 and no HS subgroups had significantly more patients younger than 12 years, compared with those of HS type 1 (90.5%, 77.8% vs 47.4%, respectively). History of febrile seizures was higher in HS type 1. Prolonged/recurrent febrile seizures were most common in patients 12 years and older, whereas LEAT was the most common etiology in patients under 12 years of age (p < 0.001). Patients with HS type 1 had longer duration of epilepsy and an older age at the time of surgery compared with patients with HS type 3 and no HS (p: 0.031, p: 0.007). At final visit, 74.3% of the patients were seizure-free. Seizure outcome showed no significant difference between pathological subtypes.
Our study presents the distribution of HS ILAE subtypes in an exclusively pediatric series along with long-term seizure outcome. The study reveals that the leading pathological HS subgroup in children is HS type 1, similar with adult series. Hippocampal sclerosis type 2 is significantly less in children compared with adults; however, HS type 3 emerges as the second most predominant group because of dual pathology, particularly LEAT. Further studies are required regarding clinicopathological features of isolated HS in pediatric cohort. Seizure-free outcome was favorable and similar in all HS types in children. The proportion of HS types may be better defined in pediatric patients with temporal resections, as the current HS ILAE classification becomes more widely used, and may help reveal the surgical and cognitive outcome with respect to HS types.
根据国际抗癫痫联盟（ILAE）的分类，海马硬化（HS）亚型的分布主要在成年患者中报道。我们旨在根据当前分类，回顾接受颞叶切除术伴杏仁核-海马切除术的儿童的病理发现，并评估儿童 HS 亚型的术后结果。
纳入 70 例因药物难治性癫痫接受颞叶切除术的儿童，随访时间至少 2 年，对手术海马标本进行 HS ILAE 分类重新评估。
神经病理学评估显示 38 例（54.3%）为 HS 1 型，2 例（2.8%）为 HS 2 型，21 例（30%）为 HS 3 型，9 例（12.9%）为无 HS。70 例患者中有 23 例（32.9%）存在双重病理，最常见的模式是 HS 3 型伴低级别与癫痫相关的脑肿瘤（LEAT）。HS 类型与年龄的分布显示，HS 3 型和无 HS 亚组中 12 岁以下的患者明显多于 HS 1 型（分别为 90.5%、77.8%和 47.4%）。HS 1 型患者热性惊厥病史较高。12 岁及以上患者中，长时间/反复发作热性惊厥最为常见，而 12 岁以下患者中，LEAT 是最常见的病因（p<0.001）。与 HS 3 型和无 HS 型相比，HS 1 型患者癫痫发作持续时间更长，手术时年龄更大（p：0.031，p：0.007）。末次随访时，74.3%的患者无癫痫发作。病理亚型之间无显著的癫痫发作结果差异。
本研究在专门的儿科系列中呈现了 ILAE 海马硬化亚型的分布，以及长期的癫痫发作结果。研究表明，儿童中主要的病理 HS 亚组是 HS 1 型，与成人系列相似。与成人相比，儿童中 HS 2 型明显较少；然而，由于双重病理，尤其是 LEAT，HS 3 型成为第二大主要群体。需要进一步研究儿科孤立性 HS 的临床病理特征。在儿童中，无癫痫发作的结果是有利的，且在所有 HS 类型中相似。随着 ILAE 分类的广泛应用，在接受颞叶切除术的儿科患者中，HS 类型的比例可能得到更好的定义，这可能有助于揭示 HS 类型与手术和认知结果的关系。
