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顽固性癫痫患者下丘脑错构瘤的胼胝体间切除术

Transcallosal resection of hypothalamic hamartomas in patients with intractable epilepsy.

作者信息

Harvey A Simon, Freeman Jeremy L, Berkovic Samuel F, Rosenfeld Jeffrey V

机构信息

Children's Epilepsy Program, Royal Children's Hospital, Parkville, Victoria, Australia.

出版信息

Epileptic Disord. 2003 Dec;5(4):257-65.

Abstract

A variety of surgical treatments for intractable epilepsy with hypothalamic hamartoma (HH) are described, although most are derived from limited patient experience gathered from several centres. We describe the results of transcallosal resection of HH in 29 consecutive patients undergoing surgery at one centre. Twenty-nine patients aged 4-23 years (mean 10 years) underwent HH surgery with a minimum of 12 months follow-up. A comprehensive, presurgical epilepsy evaluation, supplemented with endocrine and ophthalmological assessments was performed in all cases. HH were resected via a transcallosal, interforniceal approach to the third ventricle, with the assistance of frameless stereotaxy, limiting the resection to the margins of the third ventricular walls and floor and minimising traction and diathermy. Complete or near-complete (>95%) resection of the HH was achieved in 18/29 patients, 75-95% resection was achieved in seven patients (four of whom had complete or near-complete disconnection of residual HH) and less than 50% resection was achieved in four. Postoperatively (follow-up 12-70 months, mean 30 months), 15 became seizure-free (nine off antiepileptic medication), seven had >90% reduction in seizure frequency, three had 55-80% reduction in seizure frequency, and four had less than 40% reduction in seizure frequency. Of 16 patients who had seizures in the early postoperative period, six became seizure. No patient or lesion characteristics were associated with postoperative seizure freedom, including features of symptomatic generalised epilepsy. Neurobehavioural improvement and resolution of EEG abnormalities were seen in the majority. Complications were transient hemiparesis in two, transient hypernatraemia in 17, short-term memory impairment in 14 (persistent in four), weight gain in ten (persistent in five), need for supplemental thyroxine in five, and lowered growth hormone (uncertain clinical significance) in six. Transcallosal resection of HH is an effective treatment for intractable epilepsy, with 76% patients in our seizures being seizure-free or having >90% seizure reduction. The operative risks include stroke, short-term memory disturbance, weight gain and minor endocrine disturbances. Based on published data, the transcallosal approach appears to be safer and more effective than other operative strategies.

摘要

本文描述了多种用于治疗下丘脑错构瘤(HH)所致难治性癫痫的手术方法,不过大多数方法都源于多个中心积累的有限患者经验。我们阐述了在同一中心连续接受手术的29例患者经胼胝体切除HH的结果。29例年龄在4至23岁(平均10岁)的患者接受了HH手术,且至少随访12个月。所有病例均进行了全面的术前癫痫评估,并辅以内分泌和眼科评估。在无框架立体定向技术的辅助下,通过经胼胝体、经穹窿间入路至第三脑室切除HH,将切除范围限制在第三脑室壁和底部边缘,尽量减少牵拉和透热疗法。18/29例患者实现了HH的完全或近乎完全(>95%)切除,7例患者实现了75 - 95%的切除(其中4例残留HH完全或近乎完全离断),4例患者切除率低于50%。术后(随访12 - 70个月,平均30个月),15例患者癫痫发作停止(9例停用抗癫痫药物),7例癫痫发作频率降低>90%,3例降低55 - 80%,4例降低不到40%。术后早期有癫痫发作的16例患者中,6例癫痫发作停止。包括症状性全面性癫痫特征在内,没有患者或病变特征与术后癫痫发作停止相关。大多数患者出现神经行为改善和脑电图异常消失。并发症包括2例短暂性偏瘫、17例短暂性高钠血症、14例短期记忆障碍(4例持续存在)、10例体重增加(5例持续存在)、5例需要补充甲状腺素以及6例生长激素降低(临床意义不确定)。经胼胝体切除HH是治疗难治性癫痫的有效方法,我们的患者中有76%癫痫发作停止或发作频率降低>90%。手术风险包括中风、短期记忆障碍、体重增加和轻微内分泌紊乱。根据已发表的数据,经胼胝体入路似乎比其他手术策略更安全、更有效。

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