Gales Jordan M, Jehi Lara, Nowacki Amy, Prayson Richard A
Cleveland Clinic Department of Anatomic Pathology, Cleveland, OH, 44195; Cleveland Clinic Lerner College of Medicine of Case Western Reserve University School of Medicine, Cleveland, OH 44198.
Cleveland Clinic Department of Neurology, Division of Epilepsy, Cleveland, OH 44198.
Hum Pathol. 2017 May;63:79-88. doi: 10.1016/j.humpath.2017.02.013. Epub 2017 Mar 18.
Hippocampal sclerosis (HS) and focal cortical dysplasia (FCD) are among the most common neuropathological findings in those undergoing surgery for refractory mesial temporal lobe epilepsy. Existing data regarding differences among the most recent International League Against Epilepsy (ILAE) HS subtypes remain limited. This study sought to characterize the roles of HS subtype and coexistent FCD. Epilepsy surgery pathologic specimens in 307 cases of temporal lobe epilepsy with HS were reviewed (mean age±SD, 37±15years; 56% women). HS and coexistent FCD were classified according to ILAE guidelines. Medical records were reviewed for data on seizure recurrence and seizure burden (clinical follow-up mean duration ± SD, 5±4years). Cases of typical HS (ILAE type I) predominated (ILAE type Ia: 41%, Ib: 47%, II: 11%, and III: 0.7%]. The HS subtypes shared similar demographic and etiologic characteristics, as well as associated pathology and postoperative seizure outcomes. Individuals with type Ib HS were more likely to remain seizure free at long-term follow-up when compared with other subtypes, and they had a later age of seizure onset. Two hundred forty-three cases (79%) demonstrated FCD within the adjacent temporal lobe. Its presence was associated with a significantly decreased risk of seizure recurrence (P=.02). When present, FCD was predominantly type I (98%). HS subtype does not appear to affect epilepsy surgery outcomes despite some clinical differences between the subgroups. FCD is often observed in association with HS in mesial temporal lobe epilepsy; the finding of FCD was associated with better postoperative outcomes.
海马硬化（HS）和局灶性皮质发育不良（FCD）是难治性内侧颞叶癫痫患者手术中最常见的神经病理学发现。关于最新国际抗癫痫联盟（ILAE）HS亚型之间差异的现有数据仍然有限。本研究旨在描述HS亚型和共存FCD的作用。回顾了307例患有HS的颞叶癫痫患者的癫痫手术病理标本（平均年龄±标准差，37±15岁；56%为女性）。HS和共存FCD根据ILAE指南进行分类。查阅病历以获取癫痫复发和发作负担的数据（临床随访平均持续时间±标准差，5±4年）。典型HS（ILAE I型）病例占主导（ILAE Ia型：41%，Ib型：47%，II型：11%，III型：0.7%）。HS各亚型具有相似的人口统计学和病因学特征，以及相关病理学和术后癫痫发作结果。与其他亚型相比，Ib型HS患者在长期随访中更有可能无癫痫发作，且癫痫发作起始年龄较晚。243例（79%）在相邻颞叶显示有FCD。其存在与癫痫复发风险显著降低相关（P = 0.02）。当存在时，FCD主要为I型（98%）。尽管各亚组之间存在一些临床差异，但HS亚型似乎并不影响癫痫手术结果。在内侧颞叶癫痫中，FCD常与HS相关；FCD的发现与更好的术后结果相关。
