Dept. of Neurosurgery, Xinqiao Hospital, The Third Military Medical University, Chongqing, PR China.
Epilepsy Res. 2012 Aug;101(1-2):36-45. doi: 10.1016/j.eplepsyres.2012.02.017. Epub 2012 Mar 28.
The efficacy of surgery for the treatment of epilepsy in patients with West syndrome secondary to tuberous sclerosis is unclear. The charts of 17 patients with tuberous sclerosis and secondary West syndrome who underwent a one-stage surgical resection with a combined palliative operative procedure were reviewed. Engel classification was used to classify the patients with regard to seizure status following surgery. After surgery, 11 patients were in Engel class I, 4 in class II, and 2 in class III. The EEG after surgery was normal in 8 patients, significantly improved in 8, and without significant improvement in 1 patient. Six patients had a recurrence of seizures after surgery, which included 3 patients with continuing spasms and 3 patients where the spasms had resolved but had developed either partial seizures or generalized tonic-clonic seizures. There were significant improvements in the Gesell Developmental Schedules for motor field (P=0.003), adaptive field (P=0.003), language field (P=0.033), and personal-social field (P=0.007). Thus, a one-stage surgical approach can be used to produce satisfactory outcomes in young children with tuberous sclerosis who have secondary West syndrome and seizures that do not respond to conventional antiepileptic therapy, even in when there are multiple epileptogenic foci.
手术治疗结节性硬化症继发 West 综合征癫痫的疗效尚不清楚。本研究回顾性分析了 17 例行一期手术切除联合姑息性手术的结节性硬化症继发 West 综合征患者的临床资料。术后采用 Engel 分级评估患者的癫痫发作控制情况。术后 11 例患者为 Engel Ⅰ级，4 例为 Engel Ⅱ级，2 例为 Engel Ⅲ级。8 例患者术后脑电图正常，8 例明显改善，1 例无明显改善。术后 6 例患者癫痫复发，其中 3 例持续痉挛，3 例痉挛缓解但出现部分性发作或全面性强直阵挛发作。患儿的运动领域（P=0.003）、适应领域（P=0.003）、语言领域（P=0.033）和个人社会领域（P=0.007）的 Gesell 发育量表评分均显著提高。因此，对于结节性硬化症继发 West 综合征且常规抗癫痫治疗无效的婴幼儿，即使存在多个致痫灶，一期手术治疗也可获得满意的疗效。
