结节性硬化病人功能区致痫皮质结节的手术处理(附9例临床分析)

Surgery of cortical tubers of tuberous sclerosis in functional areas

目的探讨结节性硬化（tuberous sclerosis，TS）病人功能区致痫皮质结节的手术适应证、手术方式和预后。方法回顾性分析了我院手术治疗9例功能区致痫皮质结节的经验。4例术前已出现功能障碍的功能区皮质结节，术中予以直接切除结节。其余5例病人在唤醒麻醉状态下电刺激病人语言区、运动区和感觉区，其中4例病人显示皮质结节已无功能，予以切除结节；1例病人其结节部分区域存在反应，切除无功能区域后在残余皮质结节边缘行孤立手术。结果本组随访1～5年，平均3年。9例病人中有6例获得Ⅰ级（Engel分级），2例获得Ⅱ级，1例为Ⅲ级。平均智商（IQ）从术前的67．4分提高到75．1分。本组3例病人出现暂时性的并发症，未出现新的永久性神经功能损害。结论TS功能区致痫皮质结节可以采取切除性手术为主的处理策略。

Objective To investigate the surgical procedures and outcomes for epileptogenic cortical tubers of tuberous sclerosis （TS） in functional areas. Methods Surgery of 9 TS patients with epileptogenic cortical tubers in functional areas were reviewed. Epileptogenicc cortical tubers in functional areas were resected directly when the patient had neurodysfunction. Resection procedure was also conducted when cortical tubers in functional areas showed no response monitored by cortical electrical stimulation. Otherwise, the cortical tubers were isolated when patients showed functional response. Results At follow-up, on average 3 years after surgery, six of 9 patients had an Engel ClassⅠ outcome after surgery and an additional two patients had rare seizure （Engel Class Ⅱ）, and one patient had a decrease in seizure frequency （Engel Class Ⅲ）. Mean intelligence quotient （IQ） improved from 67.4 to 75.1. Temporary complications were observed in three patients and there was no new permenant neurodysfunction. Conclusion Resection procedure can be used for most of TS patients with epileptogenic cortical tubers in functional areas.