Bourgeois Marie, Crimmins Darach William, de Oliveira Ricardo Santos, Arzimanoglou Alexis, Garnett Matthew, Roujeau Thomas, Di Rocco Federico, Sainte-Rose Christian
Service de Neurologie Pédiatrique, Hôpital Necker-Enfants Malades, Paris, France.
J Neurosurg. 2007 Jan;106(1 Suppl):20-8. doi: 10.3171/ped.2007.106.1.20.
The authors sought to analyze the success rate of surgery in the management of medically intractable epilepsy in children with Sturge-Weber syndrome and to determine whether the extent and timing of surgery affected seizure and developmental outcomes.
The authors performed a retrospective review of 27 children who underwent surgery at their institution for medically resistant epilepsy, and they examined the outcomes with regard to epilepsy control and neuropsychological development. Seventeen children (63%) experienced onset of their epilepsy when they were younger than 1 year of age. These patients were significantly more likely to have hemiparesis (p < or =0.001) and status epilepticus (p < or = 0.001) and be developmentally delayed (p < or = 0.025) than children whose epilepsy started later in life. Eight patients underwent a hemispherectomy (either anatomical or functional), and complete resolution of epilepsy was noted in all. Of the 19 patients in whom a focal resection was performed, 11 (58%) became seizure free. The 10 children in whom there was residual disease were more likely to have continuing epilepsy than the nine whose lesions were completely excised (p< or = 0.05). Seventeen children exhibited improvement in their developmental status following surgery. This improvement was significantly affected by completeness of resection (p< or = 0.05) and age at surgery (p< or = 0.009). Seizure freedom per se was not affected by the timing of surgery.
Medically intractable epilepsy in children can be treated effectively by surgery. The degree of resection or disconnection of diseased tissue, but not patient age at the time of surgery, is an important factor in achieving epilepsy control. Early surgery is more likely to improve developmental outcome.
作者试图分析手术治疗小儿斯-韦综合征（Sturge-Weber syndrome）药物难治性癫痫的成功率，并确定手术范围和时机是否会影响癫痫发作及发育结果。
作者对在其机构接受手术治疗药物难治性癫痫的27例儿童进行了回顾性研究，并检查了癫痫控制和神经心理发育方面的结果。17例儿童（63%）在1岁前出现癫痫发作。与癫痫发作较晚的儿童相比，这些患者出现偏瘫（p≤0.001）、癫痫持续状态（p≤0.001）和发育迟缓（p≤0.025）的可能性明显更高。8例患者接受了大脑半球切除术（解剖性或功能性），所有患者癫痫均完全缓解。在接受局灶性切除的19例患者中，11例（58%）癫痫发作停止。残留病变的10例儿童比病变完全切除的9例儿童更有可能持续癫痫发作（p≤0.05）。17例儿童术后发育状况有所改善。这种改善受到切除完整性（p≤0.05）和手术年龄（p≤0.009）的显著影响。癫痫发作停止本身不受手术时机的影响。
小儿药物难治性癫痫可通过手术有效治疗。病变组织的切除或离断程度而非手术时的患者年龄是实现癫痫控制的重要因素。早期手术更有可能改善发育结果。
