脑裂畸形继发难治性癫的微创外科治疗

目的探讨脑裂畸形继发难治性癫的致灶定位以及微创外科治疗方法。方法回顾性分析11例脑裂畸形继发难治性癫病人的临床资料,术前通过多模态神经影像和长程视频脑电图进行解剖与功能定位。在神经导航引导下使用皮质电极描记了解脑裂畸形病灶与癫波的关系,显微镜下将脑裂畸形的致灶切除。其中位于功能区的脑裂畸形,可使用功能MRI(fMRI)导航并辅以小功率皮质热灼。结果随访11例,时间12个月。术后癫发作完全消失9例,好转2例。结论多模态神经影像和长程视频脑电图可以对脑裂畸形继发的难治性癫进行致灶的解剖与功能定位,在保护脑功能的基础上将脑裂畸形的致灶切除是手术关键。     

脑局部孤立离断术治疗难治性癫痫

目的 探讨脑局部孤立离断术的患者适应证、术前评估手段及手术疗效。方法 分析6名难治性癫痫患者的术前术后资料，对患者的评估手段、手术结果进行分析。结果 6名患者中，随访9-15个月，5名发作消失，达到Engel Ⅰ级治疗，1名患者发作得到有效控制，发作频率减少90％以上。1例患者出现头皮下积液，术后2个月复查时，头皮下积液完全消失。结论 在手术适应证的要求下，综合多种术前评估方法，尤其是脑功能的准确测定可以使脑局部孤立离断术成为一种安全有效的手术。     

Peri-ictal SPECT and surgical treatment for intractable epilepsy related to schizencephaly

The authors evaluated four patients with schizencephaly who underwent subtraction ictal SPECT coregistered to MRI (SISCOM) prior to epilepsy surgery. Three patients had a SISCOM alteration that was concordant with the epileptic brain tissue. Two of these patients were rendered seizure-free and one individual experienced a significant reduction in seizures. The patient with an indeterminate SISCOM had an unfavorable outcome. SISCOM is useful in evaluating patients with schizencephaly for epilepsy surgery.     

额叶离断术治疗无影像学异常的难治性额叶癫痫

目的 探讨额叶离断术治疗无影像学异常的难治性额叶癫痫的可行性、有效性、并发症及适应证.方法 2006年6月至2012年1月解放军第153医院全军神经外科中心对12例无影像学异常的难治性额叶癫痫患者,采用额叶离断术治疗,对手术适应证的选择、手术方法、离断范围、额叶功能区的保护方法、手术效果以及并发症进行总结分析.12例患者中,10例行单纯额叶离断,2例一侧额叶离断后皮质脑电图(ECoG)监测发现离断部位后缘仍有癫痫波,加行胼胝体前部切开术.结果 5例术后出现短暂精神、记忆及性格改变,2例出现一侧肢体轻度偏瘫,均于1个月内恢复正常.无颅内出血、感染及严重并发症.随访1.0～5.5年,Engel Ⅰ级9例,Ⅱ级3例.结论 在术中直接皮层电刺激技术及ECoG监测下,采用额叶离断术治疗无影像学异常的难治性额叶癫痫,是一种安全有效的手术方法.     

顶枕及颞顶枕离断手术治疗难治性癫癎

目的探讨顶枕及颞顶枕离断治疗难治性癫癎的疗效。方法回顾性分析16例难治性癫癎病人的临床资料,其中行颞顶枕离断9例,顶枕离断5例,颞叶切除及顶枕离断2例。结果随访16例,时间0.5~3.9年,平均1.6年。根据Engel分级,术后疗效:Ⅰ级13例,Ⅱ级1例,Ⅲ级2例。术后发生脑水肿2例,无严重神经功能损伤及死亡病例。结论顶枕及颞顶枕离断技术是致癎灶位于一侧顶枕及颞顶枕部位的安全、有效的治疗方法。     

大脑半球离断术治疗儿童难治性癫痫

目的 探讨大脑半球离断术治疗儿童难治性癫痫的手术方法及疗效.方法 2007年8月至2011年10月北京三博脑科医院进行12例大脑半球离断手术.经侧裂半球离断术1例,经纵裂半球离断并颞叶切除4例,中央区造瘘半球离断并颞叶切除5例,颞叶、岛盖切除岛周半球离断术2例.手术年龄平均7.6岁(2.1 ～11.9岁).结果 术后随访0.5 -4.5年,Engel Ⅰ级10例,EngelⅡ级1例,Engel Ⅲ级1例.术后因离断不完全再次行离断手术1例.术后患者的认知及生活能力较术前提高,无脑积水等严重神经功能损伤及死亡病例.结论 大脑半球离断术治疗儿童半球性难治性癫痫完全缓解率83％ (10/12),手术疗效确定,是治疗儿童半球病变性癫痫的安全、有效的方法.     

Hemispherotomy for intractable epilepsy

Context: Hemispherotomy is a surgical procedure for hemispheric disconnection. It is a technically demanding surgery. Our experience is presented here. Aims: To validate and compare the two techniques for hemispherotomy performed in patients with intractable epilepsies. Settings and Design: A retrospective study 2001-March 2007: Nineteen cases of hemispherotomies from a total of 462 cases operated for intractable epilepsy. Materials and Methods: All the cases operated for intractable epilepsy underwent a complete epilepsy surgery workup. Age range 4-23 years (mean 5.2 years), 14 males. The seizure frequency ranged from 2-200 episodes per day; four were in status; three in epilepsia partialis continua. The pathologies included Rasmussen's, hemimegelencephaly (unilateral hemispheric enlargement with severe cortical and subcortical changes), hemispheric cortical dysplasia, post-stroke, post-traumatic encephalomalacia and encephalopathy of unknown etiology. The techniques of surgery included vertical parasaggital approach and peri-insular hemispherotomy. Neuronavigation was used in seven cases. Results: Class I outcome [Engel's] was seen in 18 cases and Class II in one assessed at 32-198 weeks of follow-up. The four patients in status epilepticus had Class I outcome. Four patients had an initial worsening of weakness which improved to preoperative level in five to eight weeks. Power actually improved in three other patients at 32-36 weeks of follow-up, but hand grip weakness persisted. In all the other patients, power continued to be as in preoperative state. Cognitive profile improved in all patients and 11 cases returned back to school. Conclusions: Both techniques were equally effective, the procedure itself is very effective when indicated. Four of our cases were quite sick and were undertaken for this procedure on a semi-emergency basis.     

癫痫的放射外科

立体定向放射外科治疗难治性癫痫早有报道，具有定位精确，对周围组织损伤小，疗效好，安全、无创等优点。但对致痫灶的定位，照射剂量的选择及机理的研究目前尚无定论。近年来，采用PET、MEG等综合定位，低剂量照射靶区的治疗方法得到肯定。     

改良大脑半球切除术治疗儿童半侧巨脑畸形伴顽固性癫癎的研究

目的探讨儿童半侧巨脑畸形并顽固性癫癎的临床、脑电图、组织学及影像学特点,以及大脑半球切除术对其治疗的效果评估。方法 对6例半侧巨脑畸形的患儿临床资料进行回顾性分析,此6例患儿均实施了大脑半球切除术的治疗。结果 6名实施了手术的患儿中5名癫癎发作完全控制,1名部分缓解,而且患儿的精神运动发育障碍得到明显改善。结论 半侧巨脑畸形的患儿有相似的临床、组织学、脑电图及影像学表现。用大脑半球切除术进行治疗可以获得成功。另外,我们认为有必要在术前和术后进行长程脑电图监测,以验证正常大脑半球中没有癫癎样的并常放电。     

Cloxazolam treatment for patients with intractable epilepsy

We examined the antiepileptic effect of cloxazolam on seizures in 23 patients with intractable epilepsy. Most of the patients had central nervous system complications, as well as frequent seizures, and were being treated with polypharmacy. Cloxazolam was administered 1 to 5 mg/day (0.05 to 0.14 mg/kg) at initiation, in two divided doses daily, and gradually increased (1 to 4 mg/day; 0.05 to 0.15 mg/kg) every month at the outpatient clinic. Plasma levels of the main active metabolite, chloro-N-desmethyldiazepam, were measured in 13 patients. Four of 23 patients (17%) became seizure-free, and nine patients (39%) manifested a good response. Both patients with generalized and partial epilepsy manifested a good response. The spectrum of cloxazolam as an antiepileptic was wide. Effective doses were 0.07 to 0.56 mg/kg, and plasma effective levels of chloro-N-desmethyldiazepam were 12.3-115.1 ng/mL. Cloxazolam may be an effective and safe antiepileptic for intractable epilepsy, and should be used as an adjunct to other antiepileptic drugs or administered after other agents.     

Clinical outcome after complete or partial cortical resection for intractable epilepsy

This is the first epilepsy surgery series to analyze the definition of "completeness" of resection, based solely on results of chronic scalp and subdural EEG recording. When patients had complete removal of all cortical areas with ictal and interictal epileptiform discharges, the clinical outcome was usually good. When areas with epileptiform discharges were left behind, good outcome was significantly less frequent. This correlation between complete resection and good outcome was independent of the presence or absence of CT-detected structural lesions or sharp waves on post-resection electrocorticography. These results support completeness of resection, defined by prolonged extraoperative EEG, as an important factor in seizure surgery.     

Metabolic treatments for intractable epilepsy

When a child on anticonvulsant medications continues to have seizures, what other options should be considered? Over the past 100 years, dietary therapies for the treatment of intractable epilepsy have become more widely recognized, and their use has continued to expand throughout the world. An increasing number of studies has shown efficacy of these metabolic treatments in improving seizure control. Currently, 4 types of dietary therapy are available in the clinic: the classic long chain fatty acid "ketogenic" diet, the medium chain triglyceride diet, the modified Atkins diet, and the low glycemic index treatment. These therapies should be considered earlier in the treatment of intractable epilepsy because they offer a different approach to treatment that has proven efficacious, tolerable, and cost-effective.     

Clorazepate therapy for intractable epilepsy

Thirty-one epileptic patients with seizures refractory to conventional anticonvulsants were treated by adding clorazepate dipotassium to their regimen. Twelve cases showed improvement in seizure frequency, three of whom attained a seizure free state. Response to clorazepate was not related to the type of epilepsy, but patients with secondary generalized epilepsy tended to be less responsive than those with partial epilepsy. Among the various seizure types, generalized tonic-clonic seizures and simple partial seizures showed, although not significant, a tendency to be more responsive to clorazepate therapy than other seizure types, including complex partial seizures, atypical absence, atonic seizures, and tonic seizures. Drowsiness was the main adverse effect, of which 14 patients complained. Six patients were withdrawn from clorazepate because of drowsiness, but in the remaining 8 patients, this side effect disappeared within a week. The appearance of adverse effect was not related to the dose of clorazepate given. Clorazepate may be an effective secondary anticonvulsant in the treatment of intractable epilepsy.     

Effect of topiramate for patients with intractable epilepsy

The effects of topiramate (TPM) were evaluated in 51 patients with intractable epilepsy. Callosotomy and hemispherotomy were performed in 16 patients and one patient before the administration of TPM, respectively. The 50% responder rate (50%RR) was recorded in 39% of the total patient population and in 58% of patients with symptomatic location-related epilepsy (SLE). TPM was most effective for frontal lobe epilepsy (FLE), and the 50%RR was recorded in 88% of those patients. TPM (50%RR) was more effective in secondary generalized seizures (in 75%) and complex partial seizures (in 67%) in comparison to that of tonic-clonic seizures (in 44%) and drop attacks (in 29%). Seventy-one percent of the patients with atypical absence seizures increased seizure frequency. The 50%RR was recorded in 22% of the patients who underwent epilepsy surgery, and 29% of those patients also showed seizure aggravation due to TPM. These results suggest the efficacy of TPM for intractable epilepsy.     

Interhemispheric commissurotomy for congenital hemiplegics with intractable epilepsy

Five patients with congenital hemiplegia and intractable epilepsy had interhemispheric commissurotomy (the split-brain operation). After intervals of 2 to 12 years, the response has been excellent in four cases. One patient had moderate benefit. There were no late complications. Commissurotomy seems to be substantially better than hemispherectomy for this syndrome.     

Executive functioning in children with intractable frontal lobe or temporal lobe epilepsy

The aim of the present study was to shed light on the executive functioning deficits that might differentiate children with frontal lobe epilepsy (FLE) from children with temporal lobe epilepsy (TLE). Participants included 19 youth with intractable FLE and 47 youth with intractable TLE. Participants completed the Wisconsin Card Sorting Test (WCST), verbal fluency, Trail Making Test (Trails A and B), Digit Span Forward (DSF), and Digit Span Backward (DSB). When compared to the normative sample, the FLE group performed significantly worse on DSF, DSB, Trails B, and the WCST. Similarly, the TLE group performed significantly worse on DSF and DSB compared to the normative sample. Youth with FLE had significantly greater difficulty on the WCST compared to the TLE group. Overall, the results indicated that youth with FLE had significantly greater difficulty with concept formation compared to children with TLE. No differences between groups emerged on tasks assessing attention, working memory, mental flexibility, or rapid word retrieval. Both groups performed significantly below the normative sample levels on attention and working memory tasks. As a whole, it appears that some, although not all, executive dysfunction is specific to FLE.     

Oligoastrocytoma presenting with intractable epilepsy.

OBJECTIVE: Oligoastrocytomas (OA) are mixed gliomas with distinct oligodendroglial and astrocytic neoplastic components. Very little about OA has been reported in the intractable epilepsy population. METHODS: We undertook a retrospective review of 923 patients who underwent resective surgery for intractable epilepsy between 1996 and 2004. RESULTS: 6/923 (0.7%) patients were diagnosed with OA. Five patients were female, one was male. Median age at diagnosis was 25 years (range 19-44 years). Tumors arose from the left side in all patients and from the temporal lobe in five patients. Three patients had complex partial seizures. Median length of refractory epilepsy prior to surgery was 10.5 years (range 1-28 years), and the median number of antiepileptic drugs used was 2 (range 1-10). Preoperative WADA testing for language and memory localization was done for three patients; preoperative stereotactical localization was done for three patients. Surgical pathology revealed low-grade OA (WHO II) in five patients, and anaplastic OA in one. There were no surgical complications, clinical or radiographic tumor recurrence at a mean follow up period of 3.2 years (range 2-8). Excellent seizure freedom was achieved in 5/6 patients. CONCLUSION: As a result of our small sample size, general conclusions may be imprecise, but this review suggests that OA behave similar to other tumors related to intractable epilepsy: they usually have a preoperative seizure course of many years, an excellent rate of seizure-freedom following surgery, and are in general, low-grade tumors with an indolent course for which serial imaging is sufficient follow-up.     

大脑半球多脑叶离断术治疗难治性癫(痫)

目的 介绍一种新的治疗半球性病变引起的难治性癫(痫)的手术方法,并就该于术方法和适应证进行讨论.方法 应用新的大脑半球多脑叶离断术.分步骤离断额叶,颞叶和顶枕叶与丘脑、基底节的联系,通过侧脑室额角和枕角离断胼胝体前后部,只保留中央前后回皮层及其与丘脑、基底节和内囊的联系,治疗一例半球性病变引发的成年难治性癫(痫)患者....     

Flunitrazepam for sleep disturbance in children with intractable epilepsy

Add-on therapy with flunitrazepam (FNZ) was performed in 5 children with marked sleep disturbance and intractable seizures. Correction of the sleep disturbance was attained immediately after the start of FNZ administration in all patients. Furthermore, a significant decrease in the seizure frequency (3 patients) and improved quality of life (4 patients) were concomitantly observed. There was no adverse effect or interaction with conventional AEDs on long-term use.