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海马硬化相关的内侧颞叶癫痫中的错构瘤

Hamartia in hippocampal sclerosis-associated mesial temporal lobe epilepsy.

作者信息

Gawelek K L, Gales J M, Prayson R A

机构信息

Cleveland Clinic Department of Anatomic Pathology, Cleveland, OH, USA.

Cleveland Clinic Department of Anatomic Pathology, Cleveland, OH, USA.

出版信息

Ann Diagn Pathol. 2018 Feb;32:63-66. doi: 10.1016/j.anndiagpath.2017.08.004. Epub 2017 Sep 6.

Abstract

Hamartia are small collections of rounded glioneuronal cells that are thought to be due to aberrant cell migration. Their presence has been recognized in association with mesial temporal lobe epilepsy; their prevalence among cases of hippocampal sclerosis (HS) and any potential association with patient demographics and outcomes is unknown. This study examines hamartia in a series of 292 patients with pathologically confirmed HS. Medical records were reviewed for pertinent patient clinical information (follow-up mean 5years). Hamartia were identified in 96 cases (33%) and were seen primarily in the amygdala (n=88) and less commonly in the hippocampus (n=10) and temporal lobe (n=4). A statistically significant relationship was found between the presence of hamartia and male gender, younger age of seizure onset, and history of childhood febrile seizures and developmental delay. It is unclear if these associations represent a real association or are a result of the underlying pathologies related to chronic epilepsy. At follow-up, there were no significant differences between patients who had hamartia and those who lacked this finding. Hamartia were observed in all subtypes of HS and there was a significant difference found in subtype distribution as well as proportion of cases between subtypes, but no association with any specific subtype overall. The presence of hamartia was not associated with the coexistence of focal cortical dysplasia or any specific histologic pattern of dysplasia. Hamartia are a common concomitant finding in HS and indicates evidence of aberrant cell migration in the hippocampal and parahippocampal regions in these patients.

摘要

错构瘤是由圆形神经胶质神经元细胞构成的小集合体,被认为是细胞迁移异常所致。错构瘤的存在已被确认与内侧颞叶癫痫相关;其在海马硬化(HS)病例中的患病率以及与患者人口统计学特征和预后的任何潜在关联尚不清楚。本研究对292例经病理证实为HS的患者中的错构瘤进行了检查。查阅病历以获取相关患者临床信息(平均随访5年)。在96例(33%)患者中发现了错构瘤,主要见于杏仁核(n = 88),较少见于海马体(n = 10)和颞叶(n = 4)。发现错构瘤的存在与男性性别、癫痫发作起始年龄较小、儿童热性惊厥病史和发育迟缓之间存在统计学上的显著关系。尚不清楚这些关联是代表真正的关联还是与慢性癫痫相关的潜在病理结果。在随访中,有错构瘤的患者和没有此发现的患者之间没有显著差异。在HS的所有亚型中均观察到错构瘤,并且在亚型分布以及各亚型之间的病例比例方面发现了显著差异,但总体上与任何特定亚型均无关联。错构瘤 的存在与局灶性皮质发育异常的共存或任何特定的发育异常组织学模式均无关联。错构瘤是HS中常见的伴随发现,表明这些患者海马体和海马旁区域存在细胞迁移异常的证据。

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