结节性硬化症合并癫痫的手术治疗

目的：探讨结节性硬化症（TSC）合并难治性癫痫的手术适应征和手术方式，并介绍手术效果与经验。方法：回顾性分析行手术治疗的19例TSC合并难治性癫痫患者的临床资料。术前评估包括同步录像脑电图（V—EEG）监测、PET或SPECT检查、MRI和CT检查以及智商测试。本组病例中8例为局灶性癫痫、11例为双侧或多灶性癫痫，其中10例患者进行了皮层电图（ECoG）检查，4例不能而6例可以确定局限性癫痫起源灶。9例患者的IQ低于70分，3例IQ正常，7例有轻度的IQ缺陷。手术行胼胝体切开1例，病灶切除术4例，脑叶切除术5例，病灶切除联合脑叶切除术6例，胼胝体切开联合病灶切除术2例，胼胝体切开、病灶切除联合脑叶切除术1例。结果：预后11例患者达到EngleI级，5例Ⅱ级，3例为Ⅲ～Ⅳ级。不能进行致痫灶确切定位的4例术后仍有癫痫发作，与术前定位明确、术后无发作的患者比较差异有显著意义。性别、癫痫起始年龄和IQ与预后无明显相关性。结论：TSC合并局灶性癫痫患者手术治疗效果良好，是进行外科手术的适应征。手术对于TSC合并多灶性癫痫患者的效果比局灶性癫痫患者的效果差，但仍可能通过颅内电极EEG定位进行手术治疗。病灶切除和脑叶切除是治疗TSC的基本手术方式。

Epilepsy surgery in patients with tuberous sclerosis complex

Objectives： To explore effective approach for patients with fuberous sclerosis complex （TSC）. Methods： Clinical datum of 19 cases with TSC and secondary intractable epilepsy were retrospec- tively analyzed after surgical therapy. Preoperative assessment included physical examination, initiative sign of seizure, video-EEG monitor, PET/SPECT, MRI ~ CT, IQ test,in which 8 patients with focal epilepsy and 11 patients with bilateral or multi-zone epilepsy, and contex EEG were recoded in 10 pa- tients with multi-zone epilepsy, and focal epileptogenic zone was found in 6 cases. IQ of 9 patients were lower than 70, 3 patients＇IQ were normal,and 7 cases had light deficiency of intelligence. Surgical proce- dures included 1 corpus callosotomie, 4 lesionectomies, 5 lobar restections and 6 lesionectomy ＆lobar resections, 2 corpus callosotomies and lesionectomies, 1 corpus callosotomies and lesionectomy ~ lobar resections. Results：Of the total samples of 11 patients had outcome Engle Class I, 5 of Class II, 3 of Class III or IV. For the 4 patients with uncertain epileptogenic zone, nobody reached seizure free. There was significant difference in surgical outcome among the cases with localizable epileptogenic zone and un- localizble one. The sex, age at seizure onset and IQ were not associated with the outcome. Conclusion： There is excellent surgical outcome for TSC with focal epilepsy, as patients are reasonable candidates for epileptic surgery, such surgery for patients with TSC of multiple epiletogenic zone is less effective than for patients with focal epilepsy, but it is a proper treatment was introcranial electrode was used and epi- leptogenic zone was localized. Lesionectomy and lobar resection are essential procedures for TSC.