Iwatani Yoshiko, Kagitani-Shimono Kuriko, Tominaga Koji, Okinaga Takeshi, Mohri Ikuko, Kishima Haruhiko, Kato Amami, Sanefuji Wakako, Yamamoto Tomoka, Tatsumi Aika, Murata Emi, Taniike Masako, Nagai Toshisaburo, Ozono Keiichi
Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan.
Brain Dev. 2012 Oct;34(9):731-8. doi: 10.1016/j.braindev.2012.01.008. Epub 2012 Feb 14.
It has been hypothesized that early seizure control may prevent children with intractable epileptic spasms (ES) from developmental regression and may contribute to better developmental outcome. The effectiveness of surgery for ES has been reported. We investigated long-term post-operative outcomes of seizure control and development in patients with symptomatic West syndrome (S-WS) who underwent epilepsy surgery. Six children who underwent surgical intervention for intractable ES were retrospectively investigated. Cortical malformations were observed on pre-operative MRI in all patients, with hemispheric or multilobar involvement in four children and focal lesions in two. Following surgery, we measured motor function, developmental age (DA), language skills, and sociopsychological function for up to 7years (mean, 4.9years). Post-operative seizure outcome was Engel Class I (n=4) or III (n=2). Motor function and DA was increased following surgery in six and five patients, respectively. Two patients started to speak in sentences following focal resection. Autistic features were noted in four of the five examined patients post-operatively. None of the patients showed developmental regression following surgery. Epilepsy surgery for S-WS with ES may result in good seizure control and improvement in motor development. Improvement in cognitive function was modest in this small cohort of children and autistic features were noted post-operatively in a substantial proportion of the children. While seizure control can be obtained by epilepsy surgery, early intervention for sociopsychological comorbidities may be warranted in children with S-WS.
据推测，早期控制癫痫发作可能会防止患有难治性癫痫痉挛（ES）的儿童出现发育倒退，并可能有助于获得更好的发育结果。已有报道称手术治疗ES有效。我们调查了接受癫痫手术的症状性韦斯特综合征（S-WS）患者术后癫痫控制和发育的长期结果。对6例因难治性ES接受手术干预的儿童进行了回顾性研究。所有患者术前MRI均观察到皮质畸形，其中4例为半球或多叶受累，2例为局灶性病变。术后，我们对患者进行了长达7年（平均4.9年）的运动功能、发育年龄（DA）、语言技能和社会心理功能测量。术后癫痫结果为恩格尔I级（n=4）或III级（n=2）。术后6例患者的运动功能和5例患者的DA有所提高。2例患者在局灶性切除术后开始说出完整的句子。术后在5例接受检查的患者中有4例出现自闭症特征。所有患者术后均未出现发育倒退。对伴有ES的S-WS进行癫痫手术可能会有效控制癫痫发作并改善运动发育。在这一小群儿童中，认知功能的改善较为有限，且相当一部分儿童术后出现自闭症特征。虽然癫痫手术可以控制癫痫发作，但对于S-WS儿童，可能有必要对其社会心理合并症进行早期干预。
