[1]华刚,谭红平,姜楠,等.结节性硬化的诊断及相关药物难治性癫痫的手术治疗[J].临床神经外科杂志,2019,16(4):303-313.[doi:10.3969/j.issn.1672-7770.2019.04.006]
 HUA Gang,TAN Hong-ping,JIANG Nan,et al.Diagnosis of tuberous sclerosis and surgical treatment for related drug-refractory epilepsy[J].Journal of Clinical Neurosurgery,2019,16(4):303-313.[doi:10.3969/j.issn.1672-7770.2019.04.006]
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结节性硬化的诊断及相关药物难治性癫痫的手术治疗()
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《临床神经外科杂志》[ISSN:1672-7770/CN:32-1727/R]

卷:
16
期数:
2019年第4期
页码:
303-313
栏目:
癫痫专题
出版日期:
2019-08-15

文章信息/Info

Title:
Diagnosis of tuberous sclerosis and surgical treatment for related drug-refractory epilepsy
作者:
华刚谭红平姜楠李少春张立民陈俊喜郭强朱丹
510510 广州,广东三九脑科医院癫痫中心癫痫外科
Author(s):
HUA Gang TAN Hong-ping JIANG Nan et al.
The Epilepsy Surgery Department of Epilepsy Center, Guangdong Sanjiu Brain Hospital, Guangzhou 510510, China
关键词:
结节性硬化术中皮层电极监测SEEG监测技术显微外科手术治疗
Keywords:
tuberous sclerosis intraoperative cortical electrode monitoring SEEG monitoring microsurgery treatment
分类号:
R742.1
DOI:
10.3969/j.issn.1672-7770.2019.04.006
文献标志码:
A
摘要:
【摘要】目的 研究结节性硬化的诊断及相关药物难治性癫痫的手术治疗方法。方法回顾性分析广东三九脑科医院2015年4月—2018年7月手术治疗的10例结节性硬化合并药物难治性癫痫患者的临床资料,并复习相关文献。本组患者的手术,采取直接术中皮层电极脑电监测下(7例),以及植入电极立体脑电图监测后(3例)行致痫灶切除两种方式。结果结节性硬化患者的病变累及多系统,可引起复杂临床表现。而累及神经系统主要表现为药物难治性癫痫及认知功能损害;癫痫发作类型多样。脑电图表现为一侧大脑半球局灶性或多灶性棘-慢波,也可表现为双侧大脑半球多灶性棘-慢波,甚至全面性棘-慢波。MRI表现为皮层及皮层下、室管膜下多发异常信号影,偶见室管膜下巨细胞星型细胞瘤。CT检查可见钙化灶。术后经3个月~3年半的随访,除1例患者的预后为Engle Ⅲ级外,其余患者均达到Engle Ⅰ级。结论根据患者的临床表现,结合影像学和基因检查,结节性硬化的诊断不困难。对于合并有药物难治性癫痫的结节性硬化患者,选择合适的病例积极进行外科手术治疗能获得良好的疗效。
Abstract:
Abstract: ObjectiveTo summarize the diagnosis of tuberous sclerosis and the surgical treatment of its related drug-refractory epilepsy. MethodsThe clinical data of 10 patients of tuberous sclerosis complicated with drug-refractory epilepsy underwent surgical treatment from April 2015 to July 2018 in Guangdong Sanjiu Brain Hospital were analyzed retrospectively and related literatures were reviewed. In this group of patients, two strategies were proposed for the treatment of epileptogenic foci: direct epithelial cortical electrode monitoring and SEEG monitoring. ResultsThe lesions in patients with tuberous sclerosis involved multiple systems and could cause complex clinical manifestations. The involvement of the nervous system was mainly characterized by drug-refractory epilepsy and cognitive impairment. The types of seizures could be diverse. The EEG showd focal or multifocal spike-slow waves in one hemisphere, or multifocal spike-slow waves in multiple hemisphere or even comprehensive spike-slow waves. MRI showed multiple abnormal signals in the cortex, subcortex and subependymal membrane. Subependymal giant cell astrocytoma could be seen occasionally, while CT examination showed calcification. After 3 months to 3 years and a half of follow-up, all patients were all Engle I grade, except one patient Engle III grade. ConclusionsIt is not difficult to diagnosis with tuberous sclerosis, according to the clinical manifestations of patients and combining with genetic and imaging examinations. Choosing the right case for active surgical treatment can achieve good results for the tuberous sclerosis patients with drug-refractory epilepsy.

相似文献/References:

[1]梁乐,马康平,付静,等.室管膜下巨细胞星形细胞瘤的临床及病理特点分析(附2例报告)[J].临床神经外科杂志,2017,14(03):186.
 LIANG Le,MA Kang-ping,FU Jing.et al.Corresponding author:LI Yun-lin.Clinicopathological study on 2 cases of subependymal giant cell astrocytomas[J].Journal of Clinical Neurosurgery,2017,14(4):186.

更新日期/Last Update: 2019-08-01