难治性癫痫相关脑血管畸形15例临床病理分析

目的分析并比较因脑血管畸形导致难治性癫痫从而接受癫痫外科手术的15例患者的临床及病理学特点。方法回顾性分析接受脑病灶及致痫灶切除手术的15例脑血管畸形患者的临床资料及病理资料。结果 15例患者均表现为难治性癫痫,其中包括8例海绵状血管瘤,7例Sturge-Weber综合征(SWS)。男性8例,女性7例,发病年龄1月龄~29岁,病程2~24年。手术年龄2~31岁。2例行单脑叶切除,12例行多脑叶切除,1例行功能性大脑半球切除。脑标本镜下可见海绵状血管瘤并局灶性皮质发育不良(FCD)Ⅲc型;软脑膜血管瘤病、灰质中弥漫钙化,脑实质内小血管壁钙化,并伴发局灶性皮质发育不良(FCD)Ⅲc型。术后随访0.5~8年,EngelⅠ级11例,EngelⅡ级2例,EngelⅢ级1例,EngelⅣ级1例。结论脑海绵状血管瘤患者接受癫痫外科手术后随访效果良好;SWS患者颅内病变程度可能与遗传学相关,SWSⅠ型患儿应尽早手术,术后效果良好;SWSⅢ型患者接受血管瘤病病灶及致痫灶切除术后,随访效果良好。     

颅面血管瘤病

目的探讨颅面血管瘤病的病理改变及治疗原则。方法回顾分析1980年1月～1995年12月治疗的7例(男1例,女6例)颅面血管瘤病患者的病理改变及手术治疗结果。其中Ⅰ型4例,Ⅱ型2例,Ⅲ型1例,主要临床表现为面部血管痣、癫痫发作、智力障碍以及偏瘫;脑电图检查显示病变部位异常脑电波。结果7例患者中除1例死亡外,其余6例手术后临床症状及脑电图均有不同程度改善。病理改变包括颅骨不对称,颅内钙化,脑萎缩,脑室扩大,硬脑膜和蛛网膜血管扩张、增生及脑血管扩张,静脉畸形等。结论对于药物控制不满意的颅面血管瘤病患者,可考虑手术治疗。     

颅内蛛网膜囊肿治疗中的有关问题

目的 探讨颅内蛛网膜囊肿的手术指征和手术方法.方法 回顾性分析209例颅内蛛网膜囊肿患者的临床表现、影像学资料、治疗方法等临床资料.结果 其中198例行CT蛛网膜下腔-脑池造影,非交通性47例均行于术治疗.43例行囊肿大部切除+脑池开放术,3例行脑室-腹腔分流术,1例行囊肿-腹腔分流术.所有手术病人手术顺利,各种术式各有利弊.结论 颅内蛛网膜囊肿应根据囊肿大小、临床症状体征、与蛛网膜下腔是否交通及年龄来决定治疗方式.手术首选囊肿切除+脑池开放术.     

Sturge-Weber综合征1例报道并文献复习

正脑颜面血管瘤综合征(Sturge-Weber syndrome,SWS)也称作脑三叉神经血管瘤综合征。它是一种罕见的神经皮肤综合征,以三叉神经眼支支配区葡萄酒色痣、同侧软脑膜血管瘤和眼脉络膜血管瘤为主要特征。本研究就近期收治1例SWS患者报道如下,并结合国内外相关文献进行分析,旨在提高大家对该病认识。1临床资料患者,女,44岁。因"右侧头面部萦红色凸起伴息肉40     

脑脊液持续外引流对蛛网膜下腔出血病人的影响——108例回顾性分析

本文回顾1983年至1988年间收治的108例蛛网膜下腔出血病人的情况,分析脑脊液持续外引流对蛛网膜下腔出血患者脑血管痉挛和脑积水的影响。病情按Hunt和Hess法分级为Ⅰ—Ⅲ级,动脉瘤破裂24小时内入院,48小时内手术者为本组研究对象。年龄70岁以上,有严重的手术或其他并发症者,或动脉瘤破裂后病情明显恶化者不在本研究之内。经CT扫描显示有与迟发性脑缺血损害有关的低密度灶改变者38例,需行分流手术的脑积水32例。这些病人蛛网膜下腔内的血块在动脉瘤手术时未予以广泛清除。所有病人均静脉注射皮质类固醇、抗生素及甘油。92例子入院当天至第三天内行脑脊液持续外引流,引流方法有枕大池、脑室或腰穿法,     

年逾70岁病人241个大脑的神经病理学研究

人脑皮质神经元密度的测定提示人生过程中神经元在不断丧失。本文着重研究老年人脑重量的下降与年龄的关系,重点分析脑重量的变化与年龄、性别及常见脑病的相互关系。本组241例均系长期住院老年病人,死后常规尸检,进行脑重量的测定,     

Sturge-Weber综合征的外科治疗：附1例报告

Sturge-Weber综合征 （ Sturge-Weber syndrome, SWS ）又称脑面血管瘤病（encephalofacial angiomatosis）或脑三叉神经血管瘤病（encephalotrigeminal angiomatosis），是一种罕见的以颜面和颅内血管瘤病为主要特征的先天性神经皮肤综合征。Sturge和Weber于1879年、1922年相继报道此病例。1936年，Bergstrand首次使用Sturge-Weber综合征这一名称，并由此命名。由于SWS发病率很低，因此对其诊断及治疗一直没有统一标准。本文报道了我科诊治的1例SWS，以探讨SWS外科治疗的术前评估及手术方法。     

颅内海绵状血管瘤的诊断与显微外科治疗

目的探讨颅内海绵状血管瘤的诊断与显微手术治疗效果。方法分析32例（34个病灶）颅内海绵状血管瘤病人的临床表现、影像学特征、手术治疗及预后。病人术前均行头颅M刚和（或）CT检查,单发病灶30例,多发病灶2例。主要临床表现为头痛、癫痫及神经功能障碍。均行最微外科手术。结果全切除33个病灶,部分切除1个病灶,术前诊断均与病理相符。15例伴癫痫者术中均予皮质脑电监测,根据术中发现行扩大切除5例,皮质热灼3例;术后达EngelI级14例,EngelⅡ级1例,术后无新发癫痫病例。24例随访3—26个月,均未发现复发病例。结论影像技术的进步使颅内海绵状血管瘤的术前诊断率大大提高。颅内海绵状血管瘤可通过显微手术切除获得良好疗效。伴有癫痫者需行术中皮质脑电监测并根据术中发现决定手术策略。     

CT脑立体定向显微直视手术切除脑功能区及深部病变

采用CT脑立体定向技术引导开颅显微外科手术切除脑重要功能区及脑深部病变18例,其中位于运动区皮质下28例、语言中枢皮质下12例、脑深部8例 临床表现主要为癫痫、偏瘫、失语 病变性质主要为脑囊虫病、慢性炎性组织、结核瘤、肺吸虫病、海绵状血管瘤、脂肪瘤及血吸虫病.术后所有病人症状均逐渐消失,完全恢复健康,无手术并发症及死亡.     

顽固性癫癎的神经病理学改变与临床研究

目的探讨顽固性癫癎(IEP)的神经病理学改变与脑电图及术后疗效的关系.方法对45例顽固性癫癎病人用皮质脑电图描记下致癎灶切除,切除的组织送病理检查,探测病理组织与脑电图及术后疗效的关系.结果星形细胞瘤5例,表皮样囊肿4例,神经节细胞瘤2例,动静脉畸形7例,粘连性蛛网膜炎7例,炎性肉芽肿3例,外伤性脑软化灶4例,其余13例为神经元变性,胶质细胞增生、变性、含铁血黄素沉积、淋巴细胞浸润或血管增生,管壁增厚或有继发性脑萎缩等.结论顽固性癫癎的致癎灶组织均存在着病理改变,病理改变与术后疗效密切相关.     

皮层脑电监测下颞叶病变合并癫的手术治疗

目的探讨皮层脑电监测下合并颞叶病变的癫手术治疗效果。方法 21例伴有癫症状的颞叶病变患者,术中通过皮层脑电图确定癫灶,切除病变后,切除或热灼可疑癫疒间灶。术后随访患者的癫发作情况。结果 21例患者切除颞叶病变前均可通过皮层脑电图探及疒间波,病变及疒间灶完全切除后,癫波消失者19例,2例功能区患者虽多次皮层热灼,仍可见偶发棘波。术后20例未再有癫疒间发作,1例有部分性发作,用抗癫疒间药可控制。结论术中皮层脑电监测切除或热灼癫灶是一种有效控制颞叶病变切除术后癫发作的方法。     

Pathologic features of dysplasia and accompanying alterations observed in surgical specimens from patients with intractable epilepsy

Malformations caused by abnormalities of cortical development, or cortical dysplasias, were examined in surgical specimens from 108 patients with medically intractable epilepsy to determine the scope of histopathologic changes. The relevance of the clinical findings was also evaluated. Various types and degrees of dysplastic features were observed in various combinations, including architectural abnormalities, an increased number of neurons in the molecular layer and/or cortical layer II, neuronal clustering, an increased number of satellite oligodendrocytes, abnormal gyration, single and/or aggregates of heterotopic neurons in the white matter, and the appearance of cytologically abnormal cells, such as giant or dysmorphic neurons and balloon cells. In the temporal lobe specimens, microdysgenesis (corresponding to mild malformations caused by abnormalities of cortical development and type IA/B focal cortical dysplasias) was more frequently observed than Taylor-type focal cortical dysplasia (type IIA/B), whereas in the frontal lobe specimens, the frequency of occurrence of both types was even. The ages at seizure onset and surgery of patients with the latter type were significantly lower than those of patients with the former. On the other hand, prominent astrocytosis in the cortex and white matter was evident in all cases, and many corpora amylacea and neurofibrillary tangle-like inclusions were observed in a subset of cases. An ultrastructural investigation revealed dilatation of the postsynaptic dendritic spines and shafts in the cortex and features indicating the occurrence in the white matter of demyelination followed by remyelination. Thus, with regard to the epileptogenic lesions, although dysplastic changes constitute the pathogenetic basis, the overlapping subsequent degenerative processes involving synapses, dendrites, and axons might contribute to the development of epileptogenic processes. Astrocytes might also actively participate in the development of the pathogenesis of epilepsy.     

Focal CT abnormality and epileptogenic focus

In 31 patients with temporal lobe epilepsy, the precise site of epileptogenic focus was determined by means of a depth EEG recording as one of the presurgical evaluations. In 13 patients, a CT scan revealed focal lesions; 7 in the left temporal lobe and 6 in the right temporal lobe. In 5 of the 7 patients and in 5 of the 6 patients the epileptogenic foci were determined in the temporal lobe on the side of a CT lesion. However, in 2 of the patients with a CT lesion in the left temporal lobe, independent epileptogenic foci were found in both the temporal lobes, and in the other patient with a CT lesion in the right temporal lobe, they were found in the right frontal and left temporal lobes. Thus, the CT lesions agreed in lateralization and focality with the epileptogenic foci in 10 of the 13 patients (77%), but they disagreed in 3 (23%). A CT lesion disclosed in the temporal lobe does not necessarily indicate the side and/or site where the epileptogenic focus may be localized. Although exceptions may be made, spatial disagreement was exemplified between the CT lesion and epileptogenic focus. Therefore, extreme caution has to be taken on the side and/or site of the epileptogenic focus when functional surgical indication is to be made.     

Focal CT Abnormality and Epileptogenic Focus

Abstract: In 31 patients with temporal lobe epilepsy, the precise site of epileptogenic focus was determined by means of a depth EEG recording as one of the presurgical evaluations. In 13 patients, a CT scan revealed focal lesions; 7 in the left temporal lobe and 6 in the right temporal lobe. In 5 of the 7 patients and in 5 of the 6 patients the epileptogenic foci were determined in the temporal lobe on the side of a CT lesion. However, in 2 of the patients with a CT lesion in the left temporal lobe, independent epileptogenic foci were found in both the temporal lobes, and in the other patient with a CT lesion in the right temporal lobe, they were found in the right frontal and left temporal lobes. Thus, the CT lesions agreed in lateralikeation and focality with the epileptogenic foci in 10 of the 13 patients (77%), but they disagreed in 3 (23%). A CT lesion disclosed in the temporal lobe does not necessarily indicate the side and/or site where the epileptogenic focus may be localized. Although exceptions may be made, spatial disagreement was exemplified between the CT lesion and epileptogenic focus. Therefore, extreme caution has to be taken on the side and/or site of the epileptogenic focus when functional surgical indication is to be made.     

Submicroscopic changes of cortex nerve cells in chronic mirror epileptic focus in rats.

Submicroscopic changes of neurons, synapses, glial cells and some elements of capillaries were established by studying chronic mirror foci which appeared in the rat cerebral cortex after implantation of cobalt-gelatinous rods into the contralateral hemisphere. The results were compared with normal control cortex and with changes after thermocoagulation of the symmetrical contralateral cortex. It is shown that epileptogenic lesions produce a significant reduction of RNA-components, edema and vacuolization of neuronal cytoplasm, hypertrophy and proliferation of glial cells. The revealed pathological changes of the synapses were manifested by dilatation of some vesicles and the appearance of heterogenic inclusions. The characteristic features of Wallerian degeneration of callosal synapses and the reversible retrograde changes of the neurons which send their axons into the traumatized zone of the contralateral hemisphere were observed to be similar after both experimental lesions.     

Cerebral cavernous malformations in the setting of focal epilepsies: pathological findings,clinical characteristics,and surgical treatment principles

Cavernous cerebral malformations (CCMs) are a well-defined epilepsy-associated pathology. They represent lesions/conglomerates of abnormally configured vessels leading to seizures either as a result of physiological changes affecting the cerebral cortex immediately surrounding the CCM (an epileptogenic mechanism that is relevant for both temporal and extratemporal lesions), or as a result of promoting epileptogenicity in remote but anatomo-functionally connected brain regions (a mechanism that is particularly relevant for temporal lobe lesions). This review details the pathological findings in CCMs and discusses the mechanisms of epileptogenicity in this context. The bulk of the review will focus on therapeutic strategies. Medical therapy using antiepileptic drugs is recommended as a first-line therapy, but surgical removal of the CCM with the surrounding cortex should be pursued if seizures prove to be drug resistant. Early timing of the resection and complete removal of any associated epileptic pathology are critical for best outcomes. In addition to reviewing the available data from prior series, we present original research from two specialized epilepsy centers targeted at answering particularly pressing clinical questions mainly related to the ideal timing and extent of surgery. Further research is needed to define the best surgical strategies in patients with temporal lobe CCMs and structurally normal hippocampi.     

Identification of the epileptogenic lobe in neocortical epilepsy with proton MR spectroscopic imaging

PURPOSE: The aim of this study was to evaluate the usefulness of multislice magnetic resonance spectroscopic imaging (MRSI) in combination with tissue segmentation for the identification of the epileptogenic focus in neocortical epilepsy (NE). METHODS: Twenty patients with NE (10 with MRI-visible malformations, 10 with normal MRI) and 19 controls were studied. In controls, N-acetylaspartate NAA/Cr and NAA/Cho of all voxels of a given lobe were expressed as a function of white matter, and thresholds were determined by calculating the 95% prediction intervals (PIs) for NAA/Cr and NAA/Cho. Voxels with NAA/Cr or NAA/Cho values less than the 95% PI were defined as "pathological." Z-scores were calculated. Depending on the magnitude of those z-scores, we used two different methods (score-localization or forced-localization) to identify in a given subject the lobe with the highest percentage of pathological voxels, which was supposed to represent the epileptogenic lobe. RESULTS: MRSI correctly identified the lobe containing the epileptogenic focus as defined by EEG in 65% of the NE patients. MRSI localization of the focus was correct in 70% of the patients with an MRI-visible malformation and in 60% of the patients with normal MRI. Of the patients, 15% had metabolically abnormal brain regions outside the epileptogenic lobe, and 35% of the patients had evidence for secondary hippocampal damage. CONCLUSIONS: MRSI may be helpful for the identification of the epileptogenic focus in NE patients, even in NE with normal MRI.     

颅内电极在功能区癫痫治疗中的应用

目的 探讨颅内电极在功能区癫痫治疗中致痫灶定位及功能区定位中的作用.方法 回顾性分析经我科治疗的涉及功能区的癫痫患者34例,经颅内电极植入明确致痫灶后,均行皮层电刺激定位功能区,根据致痫灶与功能区关系图决定治疗方案.结果 致痫灶与中央前后回相邻者10例,术中行单纯致痫灶切除术;与中央前后回部分重叠者14例,术中行非功能区致痫灶切除,功能区致痫灶皮层电凝热灼术;完全位于中央前后回皮层区域内者5例,术中行单纯皮层电凝热灼术.术后癫痫发作较术前明显减少,无明显术后功能缺失.结论 颅内电极植入是定位功能区癫痫致痫灶及功能区的有效方法.     

Surgical technique for the insertion of grids and strips for invasive monitoring in children with intractable epilepsy

Despite improved imaging, and electrical and magnetic external mapping, there are a large number of children with intractable epilepsy in whom a focus cannot be defined by non-invasive techniques. Invasive monitoring with depth electrodes, electrode grids and/or strips is required in up to 50% of children with a suspected focal seizure disorder. In children with suspected temporal lobe epilepsy the invasive techniques are required to identify which temporal lobe is the primary focus, to separate temporal from frontal foci, and to define the extent of involvement of the lateral temporal cortex. In children and infants with non-temporal epilepsy, invasive monitoring is required to define the epileptogenic zone and to map areas of cortical specialization. The current techniques used for surgical implantation are described here. In a correctly selected population invasive monitoring will define the epileptogenic focus or foci in 90% of children; 80% will have surgically treatable epilepsy. Infection rates are less than 1% for subdural strips and 6% for grids. In 88 cases no incidence of meningitis occurred.     

Electrophysiologic pathologic correlations in patients with complex partial seizures

We studied interictal activity and site of ictal onset in 26 patients with complex partial seizures of temporal lobe origin. All patients had prolonged electrocorticographic recordings from subdural electrode arrays placed both over the convexity and beneath the temporal lobe. We found a significant correlation between the epileptogenic focus and the type of pathologic lesion found at time of surgery. Macroscopic lesions strongly tended to have an epileptogenic focus on the lateral surface of the temporal lobe; patients with only microscopic abnormalities tended to have an epileptogenic focus in the mesial/basal region of the temporal lobe.