摘要：

目的 总结接受多次癫痫外科手术的难治性癫痫临床病理学特征和分型。 方法 共19 例年龄≤ 20 岁、接受2 次或以上癫痫外科手术的难治性癫痫患者，16 例（16/19）接受2 次手术、3 例（3/19）接受3 次手术，两次手术后均行组织病理学检查，参照2011 年国际抗癫痫联盟中局灶性皮质发育不良（FCD）分型标准和2007年世界卫生组织中枢神经系统肿瘤分类进行病理学诊断和分型。随访结束时采用Engel 分级评价预后并计算预后良好率。结果 手术间隔1 ~ 10 年，平均4.24 年。首次术后病理诊断为FCDⅠb 型2 例（2/19）、FCDⅡa型2 例（2/19）、FCDⅢa型1 例（1/19）、FCDⅢd 型1 例（1/19）、星形细胞瘤2 例（2/19）、少突星形细胞瘤1 例（1/19）、混合性生殖细胞肿瘤1 例（1/19）、胚胎发育不良性神经上皮肿瘤1 例（1/19）、错构瘤1 例（1/19）、血管瘤1 例（1/19）、灰质异位1 例（1/19）、瘢痕脑回4 例（4/19），仅行胼胝体切开术1 例（1/19）；再次术后病理诊断为FCDⅢa 型4 例（4/19）、FCDⅢb 型4 例（4/19）、FCDⅢc 型1 例（1/19）、FCDⅢd 型8 例（8/19）、双重病理［FCDⅢa 型伴少突星形细胞瘤以及伴胶质瘢痕和（或）瘢痕脑回］2 例（2/19）。再次术后随访0.50 ~ 5.00 年、平均2.34 年，EngelⅠ 级10 例（10/19）、EngelⅡ 级6 例（6/19）、EngelⅢ级3 例（3/19），预后良好率84.21%。结论 接受多次癫痫外科手术的难治性癫痫病理学分型主要是FCDⅢ型和双重病理。难治性癫痫患者手术效果不理想，部分经2或3次手术，发作控制较好。

关键词: 癫痫, 神经外科手术, 病理学

Abstract:

Objective  To observe and investigate the clinicopathological features and types of refractory epilepsy treated by several epilepsy surgeries.  Methods  There were 19 patients with age less than 20 years who underwent 2 (16/19) or 3 (3/19) epilepsy surgeries. After pathological examination, pathological diagnosis and subtype was made according to focal cortical dysplasia (FCD) classification proposed by International League Against Epilepsy (ILAE) Diagnostic Methods Commission in 2011 and World Health Organization (WHO) Classification of Tumors of Central Nervous System in 2007.  Results  The operation intervals were 1-10 years (average 4.24 years). The pathological diagnoses after first operation were FCDⅠb in 2 cases (2/19), FCDⅡa in 2 cases (2/19), FCDⅢa in one case (1/19), FCDⅢd in one case (1/19), 5 cases of tumor lesions [2 (2/19) of astrocytoma, one (1/19) of oligoastrocytoma, one (1/ 19) of mixed germ cell tumor, one (1/19) of hysembryoplastic neuroepithelial tumor (DNT)], one case (1/19) of hamartoma, one case (1/19) of angioma, one case (1/19) of heterotopic gray matter, and 4 cases (4/19) of ulegyria. The last one (1/19) underwent corpus callosal incision. Pathological diagnoses after reoperation were FCDⅢa in 4 cases (4/19), FCDⅢb in 4 cases (4/19), FCDⅢc in one case (1/19), FCDⅢd in 8 cases (8/19), dual pathology (FCDⅢa with oligoastrocytoma and with glial scar and/or ulegyria) in 2 cases (2/19). Patients were followed up for 0.50-5.00 years after second or third operation (average 2.34 years), and the results showed Engel Ⅰ in 10 patients (10/19), Engel Ⅱ in 6 patients (6/19) and Engel Ⅲ in 3 patients (3/19). The rate of good prognosis was 84.21%.  Conclusions  The pathological diagnoses of brain tissue resected from patients accepting several epilepsy surgeries are mainly FCD Ⅲ and dual pathology. It is suggested that the second or third operation would be effective for refractory epilepsy patients who underwent surgery already.

Key words: Epilepsy, Neurosurgical procedures, Pathology