胚胎发育不良性神经上皮瘤11例临床病理学观察

目的 研究胚胎发育不良性神经上皮瘤（DNT）的临床、影像及病理学特征.方法 回顾分析11例DNT患者的临床和影像学资料,对术中切除标本进行病理形态学观察,并对患者进行长期随访.结果 临床以难治性癫（癎）发作为主要表现,11例中有9例在20岁之前发病.磁共振成像（MRI）示皮质或皮质下低T1、高T2信号影,边界清晰,呈囊状或脑回样.7例显示强化.6例行术中皮质脑电图（ECOG）检查,发现5例瘤灶切除后仍有部分（癎）样放电存在.组织学由神经元和神经胶质异源性成分混合构成,1例为简单型,7例为复杂型,3例为非特异型.8例标本充足的病例有7例伴皮质发育不良改变.癫（癎）控制结果Ⅰ级9例,Ⅱ级2例,无肿瘤复发.结论 DNT为WHO Ⅰ级的良性肿瘤,应结合临床、影像和病理学特征确诊DNT,准确的诊断有重要的治疗和预后判断价值.     

胚胎发育不良性神经上皮瘤1例

l病历摘要女性,33岁;因间断双眼点状闪光、短暂记忆缺失20年于2012年2月入院。未服用抗癫痫药物,症状无进行性加重,神经系统查体未见异常。头颅CT示：右侧枕叶类圆形低密度影,边缘模糊,最大层面直径约3cm。头部MRI示：右侧颞,     

脑功能区病变继发癫癎的外科治疗

目的 探讨脑功能区病变继发癫痫的外科治疗方法。方法 对30例重要功能区病变继发癫痫病人在术中全麻唤醒下应用皮质体感诱发电位及电刺激定位脑重要功能区．通过皮质脑电图及深部电极脑电图定位癫痫灶，采用神经导航或术中B超监测，行显微手术病变切除加扩大切除或热灼．术后常规应用抗癫痫药物。结果 本组切除病变前均可记录到癫痫波：扩大切除后22例未再发现癫痫波，5例胶质瘤病人虽经多次皮质热灼仍可见偶发棘波，3例颞叶海绵状血管瘤病变切除（未加海马及杏仁核切除）后反复热灼仍可见少量棘波。术后癫痫疗效评价：Ⅰ级23例，Ⅱ级3例，Ⅲ级2例，Ⅳ级2例；总有效率86．7％。结论 联合应用神经导航、术中超声技术、诱发电位及电刺激技术、皮质脑电图监测及微创技术等．切除引起癫痫的病变并同期切除和（或）热灼癫痢灶。对继发性癫痫极为有效。颞叶继发性癫痫应考虑同时进行前颞叶切除。     

磁源性影像对颞叶癫(癎)定位的临床价值

目的探讨磁源性影像（MSI）对颞叶癫癎患者癫癎灶的定位价值。方法23例颞叶癫癎的患者进行了MSI检查,将结果与普通EEG、视频EEG和皮质EEG结果进行比较。其中8例行手术治疗,5例行伽玛刀治疗。结果23例患者中MEG显示单致癎灶15例,多致癎灶8例,MEG于V-EEG的符合率为84.6%,17例MRI检查异常,MEG与MRI结果符合率76.4%。8例术中ECoG定位检查,与MEG定侧定位均完全符合。13例患者MEG定位后行手术或伽玛刀治疗,疗效满意。结论MSI对颞叶癫癎定位准确,具有指导临床进一步治疗的价值。     

难治性癫(癎)手术前脑磁图定位的临床价值

目的 评价脑磁图对难治性癫(癎)手术前定位的临床价值.方法 52例难治性癫(癎)患者均于手术前进行MRI、脑磁图、头皮脑电图和视频脑电图等神经电生理学检查,并与手术中侵入性硬膜下皮质脑电图定位准确性进行比较.结果 与手术中侵人性硬膜下皮质脑电图比较,MRI定位区域与手术区域相距≤2cm×2cm者34例,73.53%(25/34)获得良好手术疗效(Engel方案Ⅰ～Ⅱ级),与手术区域相距>2cm×2cm者3例,66.67%(2/3)手术疗效良好(Engel方案Ⅰ～Ⅱ级);定位敏感度为89.47%,特异度为78.57%,准确度为86.54%.脑磁图定位区域与手术区域相距≤2cm×2cm者45例,91.11%(41/45)手术疗效良好(Engel方案Ⅰ～Ⅱ级),与手术区域相距>2cm×2cm者7例,42.86%(3/7)获得良好手术疗效(Engel方案Ⅰ～Ⅱ级);定位敏感度为97.37%,特异度为71.43%,准确度为90.38%.视频脑电图定位区域与手术区域相距≤2cm×2cm者43例,69.77%(30/43)手术疗效良好(Engel方案Ⅰ～Ⅱ级),与手术区域相距>2cm×2cm者9例,44.44%(4/9)手术疗效良好(Engel方案Ⅰ～Ⅱ级);定位敏感度为92.11%,特异度为42.86%,准确度为78.85%.结论 难治性癫(癎)患者采用手术前脑磁图定位较其他癫(癎)灶定位方法更为精确,对手术切除癫(癎)灶具有临床指导意义.     

胚胎发育不良性神经上皮瘤与颞叶癫

目的探讨胚胎发育不良性神经上皮瘤（dysembryoplastic neuroepithelial tumor,DNT）相关颞叶癫的临床特点及外科治疗方法。方法回顾性分析21例颞叶癫DNT病人的临床资料,均以部分性癫起病,平均起病年龄轻。MRI上有典型的＂倒三角征＂和瘤内分隔表现。PET检查表现为病灶及周边脑皮质葡萄糖摄取减低。均行标准前颞叶切除术。结果术后随访1~6年,癫控制良好,无肿瘤复发病例。术后病理提示：单纯型DNT 6例,复杂型DNT 15例,伴局灶性脑皮质发育不良13例。结论应用标准前颞叶切除治疗癫相关的颞叶DNT,手术预后较好。     

胚胎发育不良性神经上皮瘤

目的探讨胚胎发育不良性神经上皮瘤(DNT)的诊断和治疗。方法分析总结我院治疗的4例DNT患者的临床和病理资料。结果所有患者以癫疒间发作为临床表现;MRI上为T1低信号,T2高信号病灶,无水肿,无占位效应;手术治疗后癫疒间发作控制满意;病理可见特异的多发性瘤结节和胶质神经细胞。结论DNT是一种良性病变,手术治疗效果良好,本病的准确诊断对其治疗有重要意义。     

脑电刺激治疗难治性癫(癎)

脑电刺激治疗为不适合手术的难治性癫(癎)病人提供了一种选择,主要包括脑深部刺激、迷走神经刺激和闭合环路刺激.前两者主要通过调控丘脑-皮质通路来抑制皮质兴奋性,减少癫(癎)发作;后者通过早期预警触发刺激来抑制发作的传播扩散.本文就几种脑电刺激治疗的原理、方法、疗效和并发症等方面内容进行简要回顾总结.     

脑磁图癫(癎)灶和皮层功能区定位在癫(癎)外科的应用

目的 探讨脑磁图在癫(癎)外科术前评估中的应用价值.方法 回顾性分析216例手术治疗顽固性癫(癎)病例,术前应用脑磁图辅助定位皮层癫(癎)灶及重要皮质功能区范围,其中18例将脑磁图与神经导航系统结合.结果 本组216例患者,按照Engel癫(癎)疗效分级,Ⅰ级71.75%,Ⅱ级10.19%,Ⅲ级8.80%, Ⅳ级9.26%.脑磁图癫(癎)灶定位与术中皮质脑电图定位符合率87.9%.无一例出现手术后永久神经功能障碍.结论 脑磁图是无创确定癫(癎)灶和功能区皮质空间位置关系的重要工具,脑磁图结合神经导航术前和术中的皮质功能区定位方法应用可使癫(癎)外科实现微侵袭和精确手术.     

低频重复经颅磁刺激治疗难治性癫(癎)的临床探索

目的 观察低频及阈下强度的重复经颅磁刺激治疗难治性癫瘸的疗效.方法 采用重复经颅磁刺激治疗12例难治性局灶性癫痼患者.分析并评价治疗前后癫瘸发作频率、发作日及发作潜伏期;采集治疗前后脑电信号,分析脑电图各频带相对功率值(%)和尖波发放数目.结果 12例患者经低频重复经颅磁刺激首次治疗后,癫(癎)发作频率及发作日均减少(t=2.450,P=0.032;t=2.797,P=0.017),其中8例发作潜伏期延长.脑电图8频带相对功率值(δ%)降低(t=3.047,P=0.011)、β频带相对功率值(β%)增加(t=-2.703,P=0.021),尤以α频带相对功率值(α%)增加显著(t=-3.680,P=0.004);脑电图尖波发放数目减少(t=2.411,P=0.035).再次治疗后上述指标仍呈改善趋势,δ%降低、α%增幅变大,尤其δ%降低幅度增加至2.08倍,背景波明显改善.结论 采用低频、阈下强度的重复经颅磁刺激治疗难治性癫(癎)患者,可以减少临床发作频率、延长发作潜伏期和改善脑电图异常放电.低频重复经颅磁刺激作为非药物性神经调控领域的新方法,治疗难治性癫(癎)安全、有效,具有较好的临床应用前景.     

18例罕见胚胎发育不良性神经上皮肿瘤的治疗

目的探讨胚胎发育不良性神经上皮肿瘤（dysembryoplastic neuroepithelial tumor，DNT）的临床治疗特点。方法对2001年至2007年治疗18例DNT病人的临床症状，影像学、电生理和病理资料进行回顾性分析。结果18例DNT病人表现癫痫发作，发作形式与部位有关，14例行脑电图描记，其中10例行术中皮层脑电图描记切除病灶，术后随访1月～6年，3例仍有癫痫发作，15例癫痫发作消失，无肿瘤复发。结论DNT属良性肿瘤，手术效果良好，行术中脑电图描记可有效切除癫痫灶。     

继发性癫痫的手术治疗

继发性癫痫病因复杂．治疗方法和手段也不尽相同。自2000年7月至2006年5月我们对37例继发性癫痫患者根据病因不同进行不同手术治疗．取得了较好的疗效，现报告如下。     

迷走神经刺激治疗难治性癫(附12例分析)

目的探讨迷走神经刺激术(VNS)治疗顽固性癫的治疗效果和机制。方法回顾性分析12例顽固性癫病人的手术经验。其中脑炎后继发性癫3例,Lenonx-Castaut综合征(LGS)3例,原因不明6例。均行VNS治疗,手术将迷走神经刺激电极缠绕于迷走神经干上,在左腋前线作一皮肤切口,将刺激器置入皮下并与刺激电极相连接。术后2周开机,刺激电流从0.25mA逐渐调至1.5mA,刺激时间为30s,间歇(180～5)min,脉宽500～1000μs,频率30Hz。结果术后3个月,本组癫发作频率平均减少46%;术后随访1年以上5例,癫发作频率平均减少60%以上,且发作程度减轻,全身强直-阵挛性发作明显减少,精神状态改善。结论VNS手术创伤小,副作用少,术后能减少发作的频率,提高生活质量,对不适合开颅手术的难治性癫是一种安全有效、耐受性良好的治疗方法。     

Efficacy of vagus nerve stimulation as a treatment for medically intractable epilepsy in brain tumor patients. A case-controlled study using the VNS therapy Patient Outcome Registry

PurposeVagus nerve stimulation (VNS) therapy is a procedure to control seizure frequency in patients with medically intractable epilepsy. However, there is no data on efficacy in the subset of these patients with brain tumors. The purpose of this study is to evaluate the efficacy of VNS therapy in patients with brain tumor-associated medically intractable epilepsy.MethodsData from the VNS therapy Patient Outcome Registry, maintained by the manufacturer of the device, Cyberonics Inc. (Houston, TX, USA), was queried to characterize the response of patients in whom a brain tumor was listed as the etiology of epilepsy. A case–control analysis was implemented and patient outcome was measured by Engel classification, median seizure response and responder rate (≥50% seizure reduction) using t-tests and chi-squared tests.ResultsIn 107 patients with an epilepsy etiology related to a brain tumor, seizure reduction was 45% at 3 months and 79% at 24 months with a responder rate of 48% at 3 months and 79% at 24 months. There was no statistical difference in seizure reduction compared with 326 case–control patients from the registry without brain tumors. There was no significant difference in anti-epileptic drug (AED) usage from baseline to 24 months post implant in either group.ConclusionsVNS therapy is equally effective in patients who suffer seizures secondary to brain tumors as in patients without history of a brain tumor. VNS therapy is a viable treatment option for patients with brain tumor associated medically intractable epilepsy, assuming cytoreductive and other adjuvant therapies have been fully explored.     

胚胎发育不良性神经上皮肿瘤继发癫痫的外科治疗

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)继发癫痫的临床特点和手术疗效.方法 回顾性分析2014年12月至2019年1月手术治疗并经病理证实的53例DNT合并癫痫的临床资料.结果 肿瘤次全切除9例,全切除23例,扩大切除21例.术后病理检查结果均为DNT,其中合并局灶性脑皮质发育不良20例、海马硬化4例、神经节细胞...     

幕上原始神经外胚瘤的诊断与治疗(附5例报告)

目的 探讨幕上原始神经外胚瘤（SPNET）的临床特点及治疗效果。方法 总结5例SPNET病人的临床资料，影像学表现、病理特点及治疗结局。行急诊手术2例，常规手术3例，术后3例全脑放疗，2例联合化疗。结果 颅内病灶全切除3例，部分切除2例，随访4-18个月，死亡1例，2例无病情进展，2例进展。结论 SPNET是一种恶性程度极高的肿瘤，对化疗和放疗较敏感。     

Primary intramedullary primitive neuroectodermal tumor (PNET) – case report and review of the literature

Spinal primitive neuroectodermal tumors (PNET) are very rare tumors, and intramedullary localization is even less common. Indeed, amongst the 23 cases of the spinal PNET described in the literature, only eight cases had an intramedullary localization. Following the WHO 2000 classification, PNETs have been considered embryonal tumors composed of undifferentiated neuroepithelial cells with a capacity of differentiation into different cellular lines, such as astrocytic, ependymal, melanotic and muscular. They have been considered to arise from a neoplastic transformation of primitive neuroepithelial cells, thereby making their presence possible in any part of the central nervous system. The optimal treatment for these malignant tumors is not yet clear, although, over the years, radiotherapy has been considered the best treatment for spinal PNETs. The described case is that of a 38-year-old man with a primary intra-extramedullary PNET, treated by laminectomy, open biopsy and chemotherapy. The patient, 18 months after the onset of his symptomatology, died without cerebral tumor involvement.     

中枢神经系统感染后继发难治性癫痫的病灶切除治疗

Previous studies have focused on medial temporal lobe epilepsy secondary to central nervous system infections. Several large-sample analyses of multi-lobe injuries or complications of medial temporal lobe epilepsy have been reported. The present study selected 29 patients (10 males and 19 females with a mean age of 18 years) with refractory epilepsy secondary to central nervous system infections (meningitis in 8, encephalitis in 21) from Beijing Functional Neurosurgical Institute from May 2006 to August 2008. All patients underwent computer tomography or magnetic resonance imaging, as well as electroencephalogram examinations; cortical electrodes were embedded in 11 patients. In addition, 13 (45%) patients underwent anterior temporal lobectomy, and 16 (56%) underwent extratemporal corcticectomy. Results showed that 18 (62%) patients obtained favorable outcomes following surgical treatment, including 80% with temporal lobe epilepsy and 50% with extratemporal epilepsy. Central nervous system infection was not a contraindication for epilepsy treatment, and identification of epileptic foci proved to be crucial. In addition, a young age at infection, as well as prolonged latent period from time of infection to initial afebrile seizure, were 2 predictive factors for all patients. Cortical electrodes significantly increased the detection rate of epileptic foci, but did not improve prognosis of foci excision.     

Focal epilepsy associated with dysembryoplastic neuroepithelial tumor in the area of the caudate nucleus

Dysembryoplastic neuroepithelial tumors (DNTs) are usually located within the supratentorial cortex, often in the temporal lobe and they are frequently associated with intractable complex partial seizures. DNTs in extracortical sites are rare. Thus far, 21 cases of 36 DNT-lesions occurring in these areas have been reported; only 8 out of them had epilepsy. We report a case of a 39-year-old woman who had pharmacoresistant epilepsy associated to a DNT in the caudate nucleus-periventricular area treated by lesionectomy. During a 4-year follow-up period, the patient was seizure free and the tumor did not recur. We discuss the hypothetical epileptogenic mechanism involved and we review the pertinent literature.