痫灶切除辅助性脑皮层横行纤维热灼治疗功能区癫痫的临床研究

目的：采用低功率热灼的方法，毁损脑皮层的横行纤维，治疗功能区顽固性癫痫，观察该方法在临床的实用性和有效性。方法：124例难治性癫痫患者，手术切除非功能区致痫灶后，辅助热灼功能区的致痫灶或癫痫样放电。非功能区致痫灶切除后，功能区致痫灶热灼前后均行皮层脑电图监测。病理观察热损伤的深度和范围。结果：热灼后肉眼下见：皮层呈红白相间的条纹状改变，与多软膜下横纤维切断术（MST）类似。显微结构见：热损伤仅在脑浅皮层（Ⅰ-Ⅱ）。随访108例术后病例1-5年，癫痫发作消失的占75.9%，总有效率91.7%。术后9例病人出现一过性的功能障碍，但均在一周左右恢复。结论：非功能区致痫灶切除辅助功能区致痫灶热灼，其疗效肯定，方法安全可靠，易在临床推广。     

痫灶切除辅助脑皮层横行热灼治疗功能区顽固性癫痫

目的为了验证脑表浅层热灼治疗功能区癫痫的可行性及临床效果,我们首次应用功能区痫灶切除后,并辅助功能区皮层痫灶双极电凝热灼治疗.方法难治性癫痫124例,颞叶癫痫75例(包括原发52例;继发23例),非颞叶癫痫48例及1例功能性大脑半球切除者,以上病例均行病灶和痫灶切除.不能切除的功能区皮层痫灶行双极电凝脑表多处横行热灼.双极电凝输出功率为4μ,持续时间为1-2秒,脑表热灼间距为5mm.痫灶切除前后和功能区痫灶热灼前后均行皮层脑电监测.对术后早期和远期的表现进行了观察.结果功能区皮层热灼无SAH出现,脑软膜上血运不受热灼影响.病理证实浅表层(Ⅰ-Ⅱ)有不同程度的热凝损伤.其下Ⅲ层部分神经元变性,固缩,胶质增生不明显.而深部(Ⅳ-Ⅵ)神经元未受损.对64例术后病人随访6个月-3年,癫痫发作消失病人为51例;癫痫发作减少大于75%的病人为10例,癫痫发作减少大于50%的病人为4例,总有效率为97.5%.结论位于功能区不能切除的痫灶,辅以脑表热灼的治疗方式,可大大提高手术疗效.热灼治疗,它的优点在于安全、易行及治疗效果好,故可在临床推广应用.     

皮层电极监测下切除致痫性脑胶质瘤的临床研究

目的探讨致痫性脑胶质瘤的手术治疗方法。方法术中使用皮层电极和深部电极对相应部位皮层脑电进行监测，确定痫性放电的部位及范围，行肿瘤切除＋致痫灶切除、皮层热灼或软膜下横切。肿瘤切除后再行脑电监测，若仍有癫痫波，再行切除。结果本组51例中，38例肿瘤全切，8例次全切，5例部分切除。术后一过性偏瘫或失语8例。随访6～57个月，按谭氏分级：Ⅰ级32例，Ⅱ级7例，Ⅲ级5例，Ⅳ级4例，V级3例；胶质瘤复发8例，且均伴有癫痫发作。结论皮层电极监测下切除致痫性胶质瘤是治疗伴有癫痫症状的脑胶质瘤的有效方法，能提高患者的生存质量。     

难治性癫痫的外科治疗(附36例临床分析)

目的探讨难治性癫痫的致病灶定位和术式选择。方法回顾分析36例难治性癫痫患者的临床资料,术前均行视频脑电监测(VEEG)和CT/MR检查,结合临床症状定位致痫灶。采用颞前叶及海马、杏仁核切除+广泛致痫皮层低功率电凝热灼25例;额叶癫痫+周围皮层低功率皮层热灼术5例,病灶及周围皮层切除+低功率皮层热灼术4例,行单侧病灶切除+胼胝体前部切开+致痫皮层低功率电凝热灼2例。结果经6个月至3年的随访,满意29例,显著改善5例,良好2例。结论部分性发作的癫痫患者,最可靠的治疗方法是致痫灶的切除,但对于全面性发作的病人,其致痫区往往比较广泛而无法切除,联合皮层热灼术可以减少发作频率、减轻发作程度和改变发作形式,提高治疗效果。准确定位致病灶,选择恰当的手术方式是外科治疗难治性癫痫取得良好效果的关键。     

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.     

胼胝体切开联合皮层热灼术治疗难治性癫痫(附82例回顾分析)

目的 探讨胼胝体切开联合皮层热灼术治疗难治性癫痫的手术方式与治疗效果.方法 82例难治性癫痫行胼胝体切开术治疗,其中18例行单纯胼胝体切开、33例行胼胝体切开 致痫皮层低功率电凝热灼、31例行单侧致痫灶切除 胼胝体切开 致痫皮层低功率电凝热灼.术后随访6个月～2年.结果 11例(13.4%)癫痫发作完全消失;41例(50%)癫痫发作次数显著减少;16例(19.5%)癫痫发作程度减轻;8例(9.8%)无明显改善;6例(7.3%)发作略有加重;无死亡病例.手术后疗效以Wilsion标准评判,优良率为83%.结论 部分性发作的癫痫患者,最可靠的治疗方法是致痫灶的切除,但对于全面性发作的病人,其致痫区往往比较广泛而无法切除,胼胝体切开可以阻断两侧半球间痫样放电的传播,联合皮层热灼术可以减少发作频率、减轻发作程度和改变发作形式,提高治疗效果.     

外伤后晚期难治性癫痫的手术治疗

目的探讨外伤后晚期难治性癫痫的致痫灶精确定位、手术方法及其治疗效果。方法68例外伤后晚期难治性癫痫患者,采用神经影像学、视频脑电图、症状学以及术中皮层脑电图（ECoG）监测等方法综合定位致痫灶。采取单纯脑软化灶切除术治疗11例,脑软化灶＋周边致痫皮层切除术治疗31例,脑软化灶＋周边致痫皮层切除十功能区致痫皮层低功率电凝热灼术或多处软膜下横切术治疗20例,脑软化灶＋周边致痫皮层切除术＋胼胝体前部切开术治疗6例;17例同期行颅骨缺损修补术。术后继续正规服用抗癫痫药物。结果60例随访1—7年,EngelⅠ级21例,Ⅱ级32例,Ⅲ级4例,Ⅳ级3例,总有效率88．3％。具有精神症状的患者术后精神症状均明显缓解。出现暂时性轻偏瘫18例,永久性轻偏瘫2例,暂时性失语6例,颅内感染3例。结论手术是治疗外伤后晚期难治性癫痫的有效方法。术前准确定位致痫灶、术中ECoG监测及采取合适的术式是手术成功的关键。     

结节性硬化症癫痫的术前评估及手术方式的选择

目的 总结结节性硬化症所致癫痫患者的术前评估及手术方式,观察术后疗效.方法 回顾性分析北京三博脑科医院2004年6月至2011年6月手术治疗的20例结节性硬化症患者临床资料并系统随访1～5年.结果 经过综合评估,单纯致痫结节切除术8例;电极植入后致痫结节切除术1例;脑叶切除术4例;致痫结节切除+致痫皮层热灼术2例;脑叶切除+离断术1例;迷走神经刺激术2例;胼胝体全段切开术2例.术后随访Engel Ⅰ级11例,Ⅱ级2例,Ⅲ级5例,Ⅳ级2例,有效率达90％ (18/20).结论 经过精确术前评估,选择合适的手术方式,可以有效地控制或减轻结节性硬化引起的癫痫发作.     

皮层电极监测下切除致痫性脑胶质瘤的临床研究

目的使用皮层电极监测切除致痫性脑胶质瘤,探讨致痫性脑胶质瘤的治疗方法.方法本组病人35例,男19例,女16例.术前行EEG、CT或MRI检查.EEG示轻度异常脑电图7例,中度异常脑电图22例,重度异常脑电图6例.CT或MRI检查皆可见占位改变.其中额叶11例,颞叶8例,额顶叶7例,顶叶5例,颞枕叶3例,岛叶深部1例.常规手术开颅显露相应部位使用VEEG1161型伟思脑电图仪对皮层脑电进行监测.监测范围包括全部肿瘤,重点于肿瘤周边脑组织.确定大体致痫范围,行肿瘤切除.肿瘤切除后再行脑电监测若仍有癫痫波,根据皮层电极之定位切除致痫灶.直至致痫灶全部切除.对疑有深部癫痫灶者,使用深部电极经皮层穿刺对深部脑组织进行监测描记.对重要脑功能区予以保护.结果全部病人皆行显微镜下肿瘤全切除.病理证实星形细胞瘤8例,间变性星形细胞瘤13例,少突胶质细胞瘤7例,间变性少突胶质细胞瘤5例,胶质母细胞瘤2例.术后随访6个月～5年.未再发生癫痫者29例(82.9%);总有效率94.3%.结论只有在切除胶质瘤时一并切除致痫灶,才是治疗肿瘤并根治癫痫的最佳方法.使用皮层电极监测胶质瘤的切除,具体很多优点.     

全麻唤醒和术中电刺激在脑功能区病灶性癫痫手术中的应用

目的 探讨全麻唤醒和术中电刺激在脑功能区病灶性癫痫手术中的应用及意义.方法 对11例涉及脑功能区病灶性癫痫患者术前行MRI和头皮视频脑电图检查,其中6例行fMRI检查.全麻下手术,其中7例行食道咽腔导管插管全麻唤醒.术中皮层脑电图定位痫样放电皮层,皮层电刺激定位皮质功能区,根据病灶、致痫皮层和皮层功能区关系选择相应手术方式,在切除病灶时作皮层电刺激以保护皮质下功能传导束.结果 10例病灶全切除,1例次全切除.术后2例暂时性偏瘫.随访6～24月,患者无神经功能障碍,Kamofsky评分平均100分,使用1种抗癫痫药物,癫痫控制满意.结论 食道咽腔导管插管全麻唤醒屉一种安全、简便的麻醉唤醒方法;术中电刺激监测能够最大限度切除病灶,妥善处理致痫皮层,有效保护脑功能区,改善患者的生活质量.     

Sturge-Weber disease: operative indications and surgical results

Patients with Sturge-Weber disease with epilepsy refractory to medical therapy have been reported to develop slowly progressive neurological deficits and ultimately become moderately or severely disabled. We studied six patients with Sturge-Weber syndrome including its incomplete form. Three out of six patients with Sturge-Weber syndrome revealed evolution of calcified angioma on computed tomography. All of the three cases developed medically intractable seizures. Total and/or subtotal hemispherectomy was performed for these three cases. The surgery was effective for controlling seizures in all three cases except one with infantile spasm with hypsarrythmia on electroencephalogram who is still on anticonvulsant. Although the unremitting deterioration in mental retardation and hemiparesis was not effectively prevented by the surgery possibly because the timing of surgery was delayed in one case, the surgery not only stopped the frequent medically-intractable seizures, but also dramatically prevented the psychomotor deterioration in the other case. Although the role of surgical treatment for the patients with Sturge-Weber syndrome remains poorly defined, one can expect excellent results if the indications for surgery are carefully analyzed and hemispherectomy is performed on an individual basis.     

Microgyria associated with Sturge-Weber angiomatosis

A case is reported of an infant affected with Sturge-Weber disease who underwent left hemispherectomy due to untreatable seizures when 97 days old. Pathological analysis of the surgical specimens revealed the presence of four-layered microgyric cortex below the angiomatosis, intense gliosis, and the presence of calcifications of both the abnormal cortex and the underlying white matter. These findings suggest that the early infantile form of Sturge-Weber disease is associated with a developmental disorder of the cortical organization. Such abnormalities are consistent with the presence of an epileptogenic condition that is unresponsive to pharmacological treatment.     

Corpus callosum section in the treatment of intractable seizures in the Sturge-Weber syndrome

The Sturge-Weber syndrome includes unilateral cerebral cortical angiomatosis, which often leads to progressive cerebral dysfunction and epileptic seizures that are medically difficult to control. Cerebral resections and hemispherectomy have been successfully performed in the past in intractable epileptic cases. Two children with medically unresponsive generalized seizure activity secondary to the Sturge-Weber syndrome have been surgically treated by dividing their corpus callosum. Cessation of generalized epilepsy was achieved in both cases. Corpus callosotomy is presented as a less destructive and safer procedure in dealing with intractable seizures in the Sturge-Weber syndrome.     

Outcomes of 32 hemispherectomies for Sturge-Weber syndrome worldwide

BACKGROUND: Epilepsy affects 80% of patients with Sturge-Weber syndrome; the majority of seizures begin before the age of 1. When seizures are intractable to medications and unihemispheric, hemispherectomy is often advised. OBJECTIVE: To examine the natural history of patients who underwent hemispherectomy and identify the outcomes in terms of seizure reduction, cognition, and motor deficits. METHODS: A questionnaire was mailed to the parents of patients identified by the Sturge-Weber Foundation as having had a hemispherectomy between 1979 and 2001. Forty-six percent (32/70) of the parents responded. RESULTS: The mean age at onset of seizures was 4 months, and the median age at surgery was 1.2 years. Children had failed to respond to 3.7 anticonvulsants prior to surgery and averaged 387 seizures/month. Forty-seven percent had complications (e.g., hemorrhage and hypertension) in the perioperative period; however, 81% are currently seizure-free, with 53% off anticonvulsants. Hemispherectomy type (anatomic versus functional versus hemidecortication) did not influence outcome. Age at onset of seizures did not predict seizure freedom; however, an older age at hemispherectomy was positively correlated. Postoperative hemiparesis was not more severe than before surgery. Cognitive outcome was not related to the age at operation, side of operation, or seizure freedom. CONCLUSIONS: Children undergoing hemispherectomy presented at a young age and had frequent seizures for approximately 1 year but are now mostly seizure-free. Age at surgery did not have an adverse effect on either seizure or cognitive outcomes.     

Late complications of hemispherectomy: report of a case relieved by surgery

A case of Sturge-Weber disease treated with left hemispherectomy presented, 11 years later, with complications related to delayed intracranial haemorrhage. A loculation syndrome of the right lateral ventricle was detected and it was corrected by a ventriculoatrial shunt operation. The side of the hemispherectomy was evacuated of all the chronic products of haemorrhage, including the subdural membrane. The patient was relieved of her symptoms. It is considered that complications related to delayed haemorrhage after hemispherectomy are remediable.     

Epilepsy surgery in bilateral Sturge-Weber syndrome

Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere. Successful surgical treatment with functional hemispherectomy was followed by good long-term seizure control in both patients. The dramatic seizure control was accompanied by markedly improved quality of life for the family and children. These cases indicate that the spectrum of children that may benefit from epilepsy surgery should not be viewed too restrictively, and subsets of children with localization related epilepsy caused by extensive lesions may be resective surgical candidates with a good seizure outcome prognosis.     

脑面血管瘤病19例临床病理分析

目的分析脑面血管瘤病（Sturge-Weber syndrome,SWS）的临床、病理学特点,探讨其诊治方案及临床疗效。方法回顾性分析采用致灶手术切除加热灼治疗的19例SWS病人的临床资料,对临床表现、病理改变进行总结分析。结果大体改变：单脑叶型、单侧多脑叶型、半球型和双侧型。镜下改变可见病变区蛛网膜增厚,蛛网膜下腔呈海绵状血管瘤结构,血管管壁增厚、玻璃样变性及不规则钙化;皮质神经元减少,胶质细胞、微血管增生,以皮质的外颗粒层、外椎体层钙化最为显著,相邻皮质神经元排列紊乱,发育不良。随访5个月,恢复良好15例病人,有4例年龄〉10岁病人症状未见改善。结论 SWS是少见的先天性疾病,药物作用有限,建议在运动功能受损出现之前手术。手术年龄越小,其大脑功能的可塑性越好,恢复越理想。     

Visual functional magnetic resonance imaging in patients with Sturge-Weber syndrome

The purpose of this study is to report different patterns of visual cortex activation in patients with Sturge-Weber syndrome as compared with healthy control subjects. Utilizing a visual paradigm of flashing lights, three children with Sturge-Weber syndrome were studied with functional magnetic resonance imaging. The results are compared with those documented in eight normal sedated children, and six young adult awake volunteers, using the same paradigms. All adult volunteers manifested bilateral activation in primary visual cortex (Brodmann's 17 and 18 areas). Two of them also had activation in secondary visual cortex (Brodmann's 19 area). In the eight sedated normal children, seven manifested activation in primary visual areas. The last exhibited no activation. The patients with Sturge-Weber syndrome demonstrated in the affected occipital lobe increased activation in one patient (11 months old), no activation in the second (12 years of age), and abnormal distribution of the activation in the third (11 months old). This report demonstrates that the vascular malformation of Sturge-Weber syndrome does not necessarily prevent cortical activation in the expected occipital cortex and may be associated with different patterns of abnormal activation. Assessing cortical function with functional magnetic resonance imaging in patients with Sturge-Weber syndrome may be helpful in decisions of surgical management and counseling.     

Rasmussen综合征治疗策略的研究

目的探讨大脑半球切除术对Rasmussen综合征的治疗效果并对Rasmussen综合征的病因、发病机制、临床特征、诊断和治疗进行分析。方法对2例Rasmussen综合征病人行大脑半球切除术,观察治疗效果。结果Rasmussen综合征确切的病因及发病机制尚不清楚,但比较容易诊断,及早进行手术切除病侧大脑半球,近期可获良好效果。结论Rasmussen综合征应尽早诊断并及早进行外科治疗,同时对其确切的病因、发病机制和远期疗效应进行深入的研究。     

手术治疗不伴面部血管瘤的Sturge-Weber综合征1例

目的 总结不伴面部血管瘤的致(癎)性Sturge-Weber综合征的诊治经验.方法 报告1例女性病人,9岁,表现为药物难治性癫(癎)1年.发作形式为微笑-意识丧失-倒地抽搐.不伴面部和全身血管瘤.MRI显示:左侧顶枕交界区皮质病变,T1W呈等信号,增强后病变沿脑回强化;PET显示:病变及周围葡萄糖代谢降低,病变呈"电车轨道样"钙化.在神经导航和术中皮质脑电图(ECoG)监测下行左侧顶枕叶致(癎)灶切除术.结果 病理报告为软脑膜血管瘤.随访11个月,病人无癫(癎)发作,无严重手术并发症发生.结论 应加强对不伴面部血管痣性Sturge-Weber综合征的认识.手术切除致(癎)灶是治疗致(癎)性Sturge-Weber综合征的有效方法.