结节性硬化症相关癫（痫）的外科治疗

Surgical treatment of epilepsy in tuberous sclerosis complex

结节性硬化症（TSC）是一种少见的常染色体显性遗传神经皮肤疾病,癫（痫）为其最常见的临床表现.研究表明,即使使用多种抗癫（痫）药物,仍有超过50％的TSC相关癫（痫）成为药物难治性癫（痫）.皮质结节是导致癫（痫）的主要原因,针对致（痫）结节及相关区域的外科手术是治疗TSC相关难治性癫（痫）的重要手段.近年来,随着电生理和影像学技术的发展,TSC相关癫（痫）的手术治疗效果明显提高,不仅能够控制大部分患者的癫（痫）发作,而且能够改善患者的智力和发育迟滞.尽管如此,目前国内外尚无一个较为规范的TSC相关癫（痫）的外科治疗程序.现对TSC相关癫（痫）的外科治疗概况、评估手段、手术处理及预后进行综述,希望对TSC相关癫（痫）的外科治疗提供帮助.

Tuberous sclerosis complex （TSC） is a rare autosomal dominant neurocutaneous disease.Epilepsy is the most common manifestation.Studies show that even with a variety of antiepileptic drugs, more than 50% of TSC related epilepsy are drug-resistant.Since cortical tubers are the main epileptogenic foci, resection surgery become an important treatment of intractable epilepsy in TSC.In recent years, with the development of electrophysiology and imaging technology, surgical treatment of epilepsy in TSC shows a great efficacy of controlling seizures and improving intelligence.Up to date, there are no standard procedures of surgical treatment for refractory epilepsy in TSC.This paper aimed to provide some instructions.