额叶癫(癎)的临床及脑电图特点

目的 分析和总结额叶癫(癎)的临床及脑电图特点.方法对80例确诊为额叶癫(癎)患者的临床和脑电图资料进行分析.结果①额叶癫(癎)发作形式:单纯部分性发作、复杂部分性发作及继发全身泛化.发作特点:发作时间短、运动突出,为强直性或姿势性,发作后意识障碍轻或无,发作频、夜间增多;②发作间期脑电图:额部导联的棘波或尖波,一侧或双侧多时性尖波或慢波;发作期脑电图:额部爆发性节律,很快同步化.结论额叶癫(癎)是一组较为特征性的癫(癎)综合征.     

Epilepsy surgery of extra-temporal epilepsy

Epilepsy is one of the most common neurologic diseases. Although in the vast majority of patients the epilepsy can be controlled by antiepileptic drugs, seizures remain medically refractory in about 30 to 40% of patients with focal epilepsy. In the latter patients, epilepsy surgery is an important therapeutic option. Epilepsy surgery in temporal lobe epilepsy has proven to achieve good seizure outcome. In extratemporal lobe epilepsy, however, both presurgical evaluation and surgical techniques are more challenging and, hereby, the results have been less favourable. In the last years, improved presurgical evaluations and sophisticated surgical techniques yielded to better seizure outcome. In this review, we discuss the diagnostic procedures and seizure outcome in extratemporal lobe surgery.     

散发性夜发额叶癫痫遗传学病因分析

目的研究中国夜发性额叶癫痫（nocturnal frontal lobe epilepsy,NFLE）散发患者遗传学病因,探讨烟碱乙酰胆碱受体（nicotinic acetylcholine receptor,nAChR）相关基因在NFLE发病机制中的作用。方法经临床资料、家族史及辅助检查综合诊断,收集77例中国非家族性NFLE患者,提取基因组DNA,聚合酶链反应扩增nAChR相关基因CHRNA4、CHRNB2及CHRNA2部分区域,确认是否存在突变。如存在突变,则进一步分析突变对蛋白质疏水性和二级结构造成的影响。结果发现1例6岁患儿存在1种CHRNA4的新型杂合错义突变,导致nAChR-α4亚单位第3、4跨膜区间胞内环405位的丝氨酸突变为苯丙氨酸（S405F）。在200例中国人群健康对照筛查中未见同样突变。突变对蛋白质疏水性及二级结构均有一定影响。结论中国人群散发NFLE病例中存在CHRNA4基因的S405F突变,可能是NFLE新型遗传学病因。     

颞叶癫痫合并额叶运动症状对癫痫严重程度及预后的影响

目的：颞叶癫痫（TLE）是临床最常见药物难治性癫痫（DRE）。额叶运动症状的出现，提示颞外网络受累，可能是TLE患者预后不佳的重要因素。本研究针对确诊为TLE患者额叶运动症状的出现情况，并通过前瞻性研究分析其对预后预测作用，为临床诊疗决策提供依据。 方法：本研究共前瞻性入组符合国际抗癫痫联盟（ILAE）2017癫痫诊断及分类标准的颞叶癫痫患者151例。所有患者均采集临床发作症状学病史，记录合并额叶运动症状：头眼向对侧偏转、不对称姿势、过度运动的症状学特征，同时进行16小时连续长程视频脑电监测。采用VA-2量表对癫痫严重程度进行评分。对患者进行1年的前瞻性随访，依据ILAE 2010定义对DRE进行诊断。 结果：颞叶癫痫患者合并额叶运动症状（TLE/F+）42例，发生率27.81%。与不合并额叶运动症状的颞叶癫痫患者（TLE/F-）相比，TLE/F+组患者的发作频率（P = 0.002）、VA-2评分更高（P = 0.000），抗癫痫药物使用（TLE/F+ vs. TLE/F-：83.33% vs. 64.22%；P = 0.029）及多药联合率更高（TLE/F+ vs. TLE/F-：38.10% vs. 22.02%；P = 0.036）。多因素分析结果显示：头眼向对侧偏转是发作频率（OR = 8.246，95%CI = 1.977~34.405，P = 0.004）、VA-2评分的共同危险因素，其中头眼向对侧偏转（β = 0.277，P = 0.000）、过度运动（β = 0.343，P = 0.003）与VA评分独立相关。随访1年后TLE/F+组患者（21/42，50.0%）较TLE/F-组患者（16/107，15.0%）诊断难治性癫痫的比例更高（P = 0.000）。 结论：颞叶癫痫患者中约27.81%的合并额叶运动症状，与发作严重程度密切相关。颞叶癫痫患者出现额叶运动症状可能提示了不良的发作预后，应在临床诊疗中早期识别并予以积极干预。     

In refractory temporal lobe epilepsy,consider surgery sooner

Despite evidence that surgery for temporal lobe epilepsy is safe and effective, physicians continue to view it as a last resort in people who do not respond to medical therapy. A randomized study in Canada has demonstrated significantly higher rates of freedom from seizures in patients who underwent surgery rather than medical therapy. If we wait too long to rule a patient's epilepsy medically refractory, we increase the patient's risk of morbidity and death, and we miss a window of opportunity to eliminate seizures and permit the patient a full and productive life.     

Therapeutics in pediatric epilepsy,Part 2: Epilepsy surgery and vagus nerve stimulation

When antiepileptic drugs fail to relieve seizures adequately in children and adolescents, more invasive therapies such as epilepsy surgery and an implanted device to stimulate the vagus nerve should be considered. Temporal lobectomy is an effective treatment of complex partial and secondarily generalized tonic-clonic seizures arising in the mesial structures or lateral temporal neocortex. Excellent outcomes (seizure free or rare, nondisabling seizures) are achieved in at least 70% of children. The most common adverse effect is a superior quadrant field cut that is usually asymptomatic. Transient and more long-lasting language difficulties have been reported when the surgery involves the dominant temporal lobe. The excellent outcome rate for extratemporal surgery ranges from approximately 20% to 80%, with better results seen in patients with an identifiable lesion. Potential morbidity is related to the region of resected neocortex. Corpus callosotomy is an excellent procedure for palliation but is not a cure for seizures that cause falls, with substantial improvement seen in more than 80% of patients. Potential adverse effects include more intense focal seizures and dysphasia, depending on the developmental level of the individual. Hemispherectomy provides seizure relief in 60% to 80% of patients with hemispherical pathologies such as Sturge-Weber or Rasmussen syndromes. Operative mortality has been reported in the range of 0% to 6%; other morbidities include infection and hydrocephalus. Stimulation of the vagus nerve has reduced partial seizures by 50% or more in approximately one third of patients. No adverse cognitive or systemic effects are associated with use of the implanted vagus nerve stimulator.     

脑电图对额叶癫痫患儿诊断及治疗的指导价值

目的探讨脑电图对额叶癫痫患儿诊断及治疗的指导价值。方法选择2017年1月至2019年5月于我院确诊的80例癫痫患儿进行回顾性分析,对患儿实施磁共振成像(MRI)、常规脑电图、长程视频脑电图检查。比较三种检查方法的诊断结果;采用长程视频脑电图监测患儿治疗前、后的癫痫发作情况,评价其癫痫控制效果;并比较不同预后患儿的脑电图表现。结果长程视频脑电图检查对小儿额叶癫痫的诊断灵敏度、特异度、准确率均高于常规脑电图和MRI检查(P<0.05)。长程视频脑电图监测结果显示,治疗后,额叶癫痫患儿的癫痫发作次数明显少于治疗前,癫痫发作单次持续时间明显短于治疗前(P<0.05)。临床治疗总有效率为97.96%;预后良好组的局灶性痫性放电比例低于预后欠佳组,清醒期枕区节律性δ活动比例高于预后欠佳组(P<0.05)。结论长程视频脑电图对小儿额叶癫痫具有良好的诊断价值,还可对小儿额叶癫痫的治疗进行指导,对其治疗效果及预后进行评价。     

Composite voxel-based analysis of volume and T2 relaxometry in temporal lobe epilepsy

Voxel-based analyses of tissue characteristics such as volume and T2 are usually carried out in isolation. However, as the images are analysed in a common voxel-based framework, it is possible to directly assess the spatial relationships of abnormalities detected by each technique. We utilize this approach in well-characterized patients with unilateral temporal lobe epilepsy (TLE) with hippocampal sclerosis (HS). TLE is associated with potentially widespread volume and T2 signal abnormalities in MRI images but the relationship between these two aspects of tissue abnormality is not well understood. Here we use a novel approach of combined univariate and multivariate voxel-wise analysis to investigate the spatial relationship of these abnormalities. We studied 19 TLE patients and compared them to 115 control subjects. Grey matter (GM) and white matter (WM) volume changes were assessed with voxel-based morphometry (VBM), and changes in T2 relaxation times were evaluated with voxel-based relaxometry (VBR). The volume and T2 changes obtained using the combined univariate approach were found in an extensive area, prominently in the ipsilateral hippocampus and amygdala (overlap of GM-VBM and VBR), and in the remaining temporal lobe (overlap of WM-VBR and VBR). Other cortical and subcortical areas showed isolated volume or T2 changes. The multivariate analysis based on the Hotelling T(2) statistic, indicated a similar pattern of distributed changes across the brain but with a greater degree of statistical significance in certain areas. The composite analyses appear to identify a network of affected areas not as easily appreciated by the individual analysis of volume or T2 changes.     

额叶p-mTOR异常表达与外伤性癫痫发生的相关研究

目的:探索外伤性癫痫(Posttraumatic epilepsy,PTE)发生与额叶p-mTOR异常表达的相关性。方法:将78只成年雄性SD大鼠分为正常对照组(A组)6只,生理盐水对照组(B组)和癫痫模型组(C组)各36只,C组右侧额叶注射FeCl_2造模。应用免疫组化技术检测造模后1h、24h、1周、2周、4周各组大鼠额叶皮质中p-mTOR(Ser2448)的动态表达。结果:A组额叶p-mTOR(Ser2448)表达的免疫组织化学染色,神经元胞质呈浅棕色,提示弱阳性表达;B组细胞染色程度较A组加深,p-mTOR(Ser2448)表达阳性细胞数增加;C组可见细胞结构紊乱,胞质呈明显的棕黄色,提示该蛋白在细胞质中呈强阳性表达。C组和B组比较,1h表达开始升高(t=-1.435,P=0.182),但差异无统计学意义,1周表达高峰(t=-4.073,P=0.002),2周降低(t=-2.614,P=0.026),4周时再次升高(t=-2.506,P=0.031),差异具有统计学意义(均P0.05)。结论:PTE的发病机制可能与mTOR信号通路上pmTOR(Ser2448)的表达异常增加有关。     

难治性颞叶内侧癫痫手术的术中配合及护理

癫痫是最常见的神经系统疾病之一。它是一种致残率高、临床反复发作、病程漫长的疾病。其中,颞叶癫痫分为颞叶内侧癫痫、病灶性颞叶癫痫和颞叶外侧新皮质癫痫。颞叶内侧癫痫主要起源于内侧结构杏仁核、海马、钩回、海马旁回,占颞叶癫痫90%以上的病例。普遍的临床实践已经证明,难治性颞叶内     

功能磁共振观察额叶癫痫患者静息态局域一致性

目的 探讨静息状态下额叶癫痫(FLE)患者局域一致性(ReHo)的变化特点.方法 对46例常规结构MRI阴性FLE患者及性别年龄无差异的正常对照组行静息态fMRI,比较 ReHo改变脑区,观察ReHo改变脑区与FLE病程长短的相关性.结果 相比正常对照组,FLE患者ReHo值升高的脑区包括前、中扣带回,双侧岛叶、丘脑及右侧基底核区,ReHo降低脑区包括左侧额上回,左侧颞中、下回及小脑.相关性分析结果显示,FLE患者前、中扣带回,额上回ReHo值与病程长短呈正相关,双侧辅助运动区、右侧枕叶ReHo值与病程呈负相关.结论 FLE患者静息态下脑功能异常,扣带回、岛叶、丘脑及基底核区等区域存在ReHo改变.     

采用选择性海马杏仁核切除治疗颞叶癫痫病人的护理

1998年3月～2004年12月，我院对41例颞叶癫痫病人行经颧弓翼点入路选择性海马杏仁核切除术，手术顺利，术后并发症少，疗效满意。现将护理体会报告如下。     

采用选择性海马杏仁核切除治疗颞叶癫痫病人的护理

1998年3月—2004年12月,我院对41例颞叶癫痫病人行经颧弓翼点入路选择性海马杏仁核切除术,手术顺利,术后并发症少,疗效满意。现将护理体会报告如下。1临床资料41例病人中男19例,女22例,年龄4岁～51岁,平均21.9岁。病程1年～20年,平均8.4年。发作形式:强直-阵挛性发作29例,失神发作6例,复杂部分性发作6例。其中4例病人发作时有自动症,表现为双手摸索、咂嘴和行为异常。10例病人发作前有先兆,表现为头痛、头晕,胃内有气体上升感,或者出现恐惧感。病因:脑炎脑膜炎3例,高热惊厥史4例,颅脑外伤5例,其余19例未发现明确病因。所有病人均经过1年以上…     

Magnetic resonance imaging-based computer-assisted stereotactic resection of the hippocampus and amygdala in patients with temporal lobe epilepsy

In patients with medically intractable complex partial seizures of temporal lobe origin, stereotactic amygdalohippocampectomy can now provide excellent results. Target structures can be accurately identified and completely resected with use of a carbon dioxide laser. In a series of 18 patients who underwent this computer-interactive procedure, all experienced a cessation or dramatic reduction in frequency of seizure activity. Because the inferior optic radiations are disrupted with use of the posterolateral approach, nondisabling postoperative visual field deficits always ensue. In addition, two of our patients who underwent left-sided procedures had transient minor speech problems, perhaps attributable to postoperative swelling of the lateral temporal lobe. Patients in whom a surface electroencephalogram discloses a posterior temporal focus of seizure activity are candidates for stereotactic amygdalohippocampectomy.     

Seizure recurrence in temporal lobe epilepsy

We report on a patient with clinical, electroencephalographic and radiological features of temporal lobe epilepsy, whose seizures were initially controlled with antiepileptic treatment. Five years after the onset of seizures, he developed more frequent and prolonged episodes of confusion, which were misinterpreted as secondary to temporal lobe epilepsy. Further investigation revealed the presence of hypoglycaemia and the eventual diagnosis of metastatic insulinoma.     

Epilepsy surgery in children

Epilepsy surgery is becoming an increasingly used therapy for children with severe, medically intractable seizures. Temporal lobe ablation, corpus callosotomy, and hemispherectomy are currently the most commonly performed procedures. In this review the presurgical evaluation of patients with medically intractable seizures is described and risks and benefits of the surgery discussed. In selected patients temporal lobectomies and hemispherectomies may totally eliminate seizures while corpus callosotomies frequently reduce number and severity of generalized seizures.