Cleveland Clinic Epilepsy Center, Cleveland, OH, USA.
Mod Pathol. 2013 Aug;26(8):1051-8. doi: 10.1038/modpathol.2013.52. Epub 2013 Apr 5.
Patients with magnetic-resonance-imaging (MRI)-negative (or 'nonlesional') pharmacoresistant focal epilepsy are the most challenging group undergoing presurgical evaluation. Few large-scale studies have systematically reviewed the pathological substrates underlying MRI-negative epilepsies. In the current study, histopathological specimens were retrospectively reviewed from MRI-negative epilepsy patients (n=95, mean age=30 years, 50% female subjects). Focal cortical dysplasia cases were classified according to the International League Against Epilepsy (ILAE) and Palmini et al classifications. The most common pathologies found in this MRI-negative cohort included: focal cortical dysplasia (n=43, 45%), gliosis (n=21, 22%), hamartia+gliosis (n=12, 13%), and hippocampal sclerosis (n=9, 9%). The majority of focal cortical dysplasia were ILAE type I (n=37) or Palmini type I (n=39). Seven patients had no identifiable pathological abnormalities. The existence of positive pathology was not significantly associated with age or temporal/extratemporal resection. Follow-up data post surgery was available in 90 patients; 63 (70%) and 57 (63%) attained seizure freedom at 6 and 12 months, respectively. The finding of positive pathology was significantly associated with seizure-free outcome at 6 months (P=0.035), but not at 12 months. In subgroup analysis, the focal cortical dysplasia group was not significantly correlated with seizure-free outcome, as compared with the negative-pathology groups at either 6 or 12 months. Of note, the finding of hippocampal sclerosis had a significant positive correlation with seizure-free outcome when compared with the negative-pathology group (P=0.009 and 0.004 for 6- and 12-month outcome, respectively). Absence of a significant histopathology in the resected surgical specimen did not preclude seizure freedom. In conclusion, our study highlights the heterogeneity of epileptic pathologies in MRI-negative epilepsies, with focal cortical dysplasia being the most common finding. The existence of positive pathology in surgical specimen may be a good indication for short-term good seizure outcome. There is a small subset of cases in which no pathological abnormalities are identified.
磁共振成像(MRI)阴性(或“非病变性”)药物难治性局灶性癫痫患者是接受术前评估最具挑战性的群体。很少有大规模的研究系统地回顾了 MRI 阴性癫痫的病理基础。在目前的研究中,回顾性地分析了 95 例 MRI 阴性癫痫患者的组织病理学标本(平均年龄 30 岁,50%为女性)。局灶性皮质发育不良病例根据国际抗癫痫联盟(ILAE)和 Palmini 等人的分类进行分类。在这个 MRI 阴性队列中发现的最常见的病理包括:局灶性皮质发育不良(n=43,45%)、胶质增生(n=21,22%)、错构瘤+胶质增生(n=12,13%)和海马硬化(n=9,9%)。大多数局灶性皮质发育不良为 ILAE 1 型(n=37)或 Palmini 1 型(n=39)。7 例患者无明显的病理异常。阳性病理的存在与年龄或颞叶/颞外切除术无关。90 例患者术后随访资料;63 例(70%)和 57 例(63%)在 6 个月和 12 个月时无癫痫发作。6 个月时阳性病理的发现与无癫痫发作结果显著相关(P=0.035),但 12 个月时无相关性。亚组分析显示,与阴性病理组相比,皮质发育不良组在 6 个月或 12 个月时无癫痫发作结果无显著相关性。值得注意的是,与阴性病理组相比,海马硬化的发现与无癫痫发作结果有显著相关性(6 个月和 12 个月时的结果分别为 P=0.009 和 0.004)。切除的手术标本中未发现明显的组织病理学并不排除癫痫发作的自由。总之,本研究强调了 MRI 阴性癫痫中癫痫病理的异质性,以局灶性皮质发育不良最为常见。手术标本中阳性病理的存在可能是短期良好癫痫发作结果的良好指标。有一小部分病例没有发现病理异常。