57例皮质发育异常相关性癫痫临床病理分析

Clinical pathological analysis on 57 patients of epilepsy with malformations of cortical development

目的探讨皮质发育异常（MCD）相关癫痫的临床病理特征。方法回顾性分析57例MCD相关癫痫的临床病理资料，分析其病理学特征。结果MCD相关癫痫占同期手术治疗癫痫的43％。57例MCD相关癫痫中，脑沟回结构紊乱8例；皮质微发育不良（MD）8例；局灶性皮质发育不良（FCD）41例，其中FCDⅠA13例，FCDIB15例，FCDⅡA7例，FCDⅡB6例。57例MCD中22例伴海马硬化；1例FCDⅡB伴胚胎发育不良性神经上皮瘤（DNT）；1例FCDⅡB局部向神经节神经胶质瘤（GGs）过渡。结论MCD与难治性癫痫关系密切，以FCDⅠ型最为常见，多数病例伴有海马硬化。

Objective To approach the clinical pathological characteristics of epilepsy with malformations of conical development （MCD）. Method The clinical information and pathologic characteristic were studied retrospectively in 57 cases of epilepsy with MCD. Results Among the total operated epilepsy cases, the percentage of epilepsy with MCD was 43% （57/132）. The ratio between man and woman was 3.8 to 1.0. The average age was 25.4 years old. The average history of epileptic attack was 10.4 years. Among the 57 cases of epilepsy with MCD, there were 8 cases in major disruption;8 cases in microdygenesis（MD） ; 41 cases in focal cortical dysplasia（FCD）. In FCD, there were 13 cases in FCD Ⅰ A; 15 cases in FCD Ⅰ B;7 cases in FCD Ⅱ A;6 cases in FCD Ⅱ B. Further more,22 cases were accompanied with hippocampal sclerosis;1 case of FCD Ⅱ B with dysembryoplastic neuroepithelial tumors（DNT） ;1 case of FCD Ⅱ B with gangliogliomas （GGs）. Conclusions There is a close relationship between MCD and epilepsy; FCD Ⅰ type is the most common subtype of MCD; many cases have hippocampal sclerosis.