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儿童期起病的癫痫性脑病的癫痫手术疗效。

Outcomes of epilepsy surgery in childhood-onset epileptic encephalopathy.

作者信息

Lee Yun-Jin, Lee Joon Soo, Kang Hoon-Chul, Kim Dong-Seok, Shim Kyu-Won, Eom Soyong, Kim Heung Dong

机构信息

Department of Pediatrics, Pusan National University Children's Hospital, Pusan National University School of Medicine, Republic of Korea.

Department of Pediatrics, Pediatric Epilepsy Clinics, Severance Children's Hospital, Brain Research Institute, Yonsei University College of Medicine, Republic of Korea.

出版信息

Brain Dev. 2014 Jun;36(6):496-504. doi: 10.1016/j.braindev.2013.06.010. Epub 2013 Jul 11.

Abstract

PURPOSE

to evaluate the outcomes and role of epilepsy surgery in children with intractable epileptic encephalopathy (EE).

METHODS

ninety-five children (64 boys, 31 girls) with intractable EE were treated by epilepsy surgery at Severance Children's Hospital from 2003 to 2008. Surgical treatments included lobar resection, hemispherotomy and corpus callosotomy (CC). Seventy-six children were Lennox-Gastaut syndrome (LGS), and 19 had West syndrome.

RESULTS

of the 76 patients with LGS, CC was performed in 37 patients (48.7%), lobar resection in 29 (38.2%) and hemispherotomy in 10 (13.2%). Of the 19 patients with West syndrome, respective surgery was performed in 15 patients (78.9%) and CC in 4 (21.1%). Of the patients receiving respective surgery, Engel's class I outcomes were achieved for 24 of 39 (61.5%) of LGS patients, and for 9 of 15 (60.0%) of West syndrome. Malformations of cortical development were commonly observed, appearing in 73.5% (36/49). In neuropsychiatric tests, 19 of 27 with LGS demonstrated improvement in postoperative cognitive function. More significant intellectual improvement correlated well with shorter epilepsy duration, good seizure outcomes, and decreased number of antiepileptic drugs.

CONCLUSIONS

epilepsy surgery should be considered in treating childhood intractable EE with expectation of improvement of both seizure and cognitive outcomes, even in cases of LGS.

摘要

目的

评估癫痫手术治疗儿童难治性癫痫性脑病(EE)的疗效及作用。

方法

2003年至2008年,95例难治性EE患儿(64例男孩,31例女孩)在Severance儿童医院接受癫痫手术治疗。手术方式包括脑叶切除术、大脑半球切除术和胼胝体切开术(CC)。76例为Lennox-Gastaut综合征(LGS),19例为West综合征。

结果

76例LGS患者中,37例(48.7%)接受了CC,29例(38.2%)接受了脑叶切除术,10例(13.2%)接受了大脑半球切除术。19例West综合征患者中,15例(78.9%)接受了相应手术,4例(21.1%)接受了CC。接受相应手术的患者中,LGS患者39例中有24例(61.5%)达到Engel I级疗效,West综合征患者15例中有9例(60.0%)达到该疗效。常见皮质发育畸形,发生率为73.5%(36/49)。在神经精神测试中,27例LGS患者中有19例术后认知功能改善。更显著的智力改善与癫痫病程较短、癫痫发作控制良好及抗癫痫药物数量减少密切相关。

结论

即使是LGS患儿,治疗儿童难治性EE时也应考虑癫痫手术,以期改善癫痫发作及认知结局。

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