立体脑电图引导射频热凝毁损治疗下丘脑错构瘤继发癫痫

目的探讨立体脑电图(SEEG)引导射频热凝毁损技术在下丘脑错构瘤继发癫痫治疗中的临床应用价值。方法回顾性分析2015年6月至2018年1月北京丰台医院神经外科接受SEEG引导射频热凝毁损治疗的7例下丘脑错构瘤继发癫痫患者的临床资料,包括详细病史、症状学、脑电图及影像学资料。术前评估考虑下丘脑错构瘤为致痫灶,随后利用立体定向框架于下丘脑错构瘤处植入SEEG电极,并行视频脑电监测,根据SEEG的放电区域进行射频热凝毁损术。若毁损后癫痫样放电未消失,则进行第二次毁损,直至癫痫样放电消失。分析术后并发症,并进行电话或门诊随访,应用Engel分级进行预后评估。结果7例患者热凝毁损术后的平均随访时间为(19.6±11.1)个月,其中5例患者的癫痫发作消失(Engel分级Ⅰ级),1例患者毁损术后的癫痫发作频率减少约75%(Engel分级Ⅲ级),1例患者毁损术后的癫痫发作频率未明显改善(Engel分级Ⅳ级)。3例患者术后早期出现高热、嗜睡、尿崩症、电解质紊乱及记忆力下降等并发症,经治疗后均恢复。结论本研究初步认为SEEG引导的射频热凝毁损术是治疗下丘脑错构瘤继发癫痫安全、有效的治疗手段。在诊断明确的前提下完全离断错构瘤基底与下丘脑的联系是癫痫控制的关键。     

立体脑电图引导多电极立体交叉射频热凝治疗药物难治性癫痫

目的观察立体脑电图(SEEG)引导多电极立体交叉射频热凝(RFTC)治疗药物难治性癫痫的安全性及有效性。方法回顾性纳入首都医科大学宣武医院神经外科2015年7月至2017年12月采用SEEG引导多电极立体交叉RFTC毁损治疗的药物难治性癫痫患者,共48例。其中颞叶内侧癫痫(MTLE)22例,下丘脑错构瘤9例,额叶癫痫5例,岛叶癫痫4例,脑室旁结节1例,结节性硬化1例,多脑叶癫痫6例。术后临床疗效评估采用Engel分级标准,并观察手术的相关并发症。结果48例患者随访时间为5~35个月,平均(16.3±7.3)个月。截止至末次随访,Engel分级Ⅰ级者23例(47.9%),Ⅱ级者12例(25.0%),Ⅲ级者11例(22.9%),Ⅳ级者2例(4.2%);术后1年无发作率(Engel分级Ⅰ级)为71.9%(23/32)。术后MTLE和下丘脑错构瘤患者疗效较好,Engel Ⅰ级的比率(或比例)分别为59.1%(13/22)和5/9;多脑叶癫痫(Engel Ⅲ级的比例为4/6)、额叶癫痫(Engel分级Ⅲ级的比例为1/5例,Ⅳ级的比例为2/5)疗效相对较差。术后出现一过性并发症7例(14.6%),永久性并发症者1例(2.1%)。结论SEEG引导多电极立体交叉RFYC治疗药物难治性癫痫微创、安全、有效;相较于其他部位,对MTLE及下丘脑错构瘤引发的癫痫疗效较好。该技术可作为经典切除手术的一种补充治疗方法。     

立体定向脑电图引导下射频热凝治疗难治性癫痫的研究进展

随着立体定向脑电图在部分难治性癫痫患者术前评估时的广泛应用,立体定向脑电图引导下射频热凝成为治疗难治性癫痫患者的一种相对安全的替代方案。该技术在我国开展还不久,本文通过相关研究及文献复习以了解立体定向脑电图引导下射频热凝治疗难治性癫痫的临床应用、安全性及预后等。     

SEEG引导下RF-TC治疗脑室旁灰质移位症所致药物难治性癫痫（附2例报道并文献复习）

目的 探讨立体定向脑电图（SEEG）引导下射频热凝毁损术（RF-TC）治疗脑室旁灰质移位症（GMH）所致药物难治性癫痫的疗效。方法 回顾性分析2例脑室旁GMH所致药物难治性癫痫的临床资料,并结合相关文献进行分析。结果 术前根据临床表现、影像学特表现以及脑电图诊断为脑室旁GMH所致药物难治性癫痫,行机器人辅助SEEG记录精确定位致痫区及传导途径,并在SEEG引导下行RF-TC治疗。所有电极偏差在3 mm误差范围以内,术后未发生手术相关并发症,无神经系统相关并发症。术后随访1年,1例癫痫完全控制,1例癫痫发作频率减少80%。结论 脑室旁GMH是导致药物难治性癫痫的原因之一,SEEG引导下RF-TC治疗的效果良好,是一种安全的、有效的治疗方式。     

基于立体定向脑电图引导下射频热凝术治疗难治性癫痫的研究进展

难治性癫痫是指两种或多种适当的抗癫痫药物仍不能控制发作的癫痫,约占所有癫痫患者的1/3,严重影响患者生活质量。随着神经外科微创技术的发展以及术前评估体系的日趋完善,立体定向脑电图引导下射频热凝术已经逐渐应用于难治性癫痫的外科治疗,因其可观的疗效和较少的并发症,已成为难治性癫痫患者一种相对安全有效的替代疗法。该文综合以往研究,对立体定向脑电图引导下射频热凝术治疗难治性癫痫的发展、治疗过程做一系统阐述,并对其疗效与并发症做出分析。     

SEEG引导下射频热凝毁损术在癫痫外科中的应用

目的总结SEEG引导下射频热凝毁损术治疗药物难治性癫痫的手术疗效及治疗体会。方法对我科2016年12月至2018年5月进行SEEG引导下射频热凝毁损术治疗的13例药物难治性癫痫患者的临床资料进行回顾性分析,并复习相关文献。结果本组纳入病种包括下丘脑错构瘤,侧脑室壁灰质异位结节及其他皮质发育异常。行SEEG引导下射频热凝毁损术后有7例出现短期并发症:3例偏瘫,2例发热、1例中枢性面瘫,1例视物模糊,出院时均完全恢复。术后常规服用抗痫药物,经过3个月至21个月的随访,10例患者EngleⅠ级,2例EngleⅡ级,1例EngleⅣ级。发作未完全控制患者均再次行开颅手术切除致痫灶,病理分别为FCDⅡa型、MCD(大脑发育畸形)、灰质异位,疗效进一步随访中。结论 SEEG引导下射频热凝毁损术是癫痫外科治疗方案中的一项有力补充,选择合适的病例进行射频热凝毁损术安全有效,甚至可以达到无发作。     

立体脑电图引导射频热凝毁损治疗药物难治性癫痫的疗效及其影响因素分析

目的探讨立体脑电图(SEEG)引导射频热凝(RFTC)毁损治疗药物难治性癫痫的疗效及其相关影响因素。方法回顾性分析2019年6月至2021年6月兰州大学第二医院神经外科收治的36例药物难治性癫痫患者的临床资料。所有患者均设计SEEG电极植入方案, 根据SEEG监测信息确定发作起始区并行RFTC毁损。术后通过电话或门诊随访, 采用Engel分级评估患者的预后。采用单因素分析和多因素logistic回归分析探讨影响药物难治性癫痫患者预后的因素。结果 36例患者接受RFTC毁损治疗后随访(13.7±6.9)个月(6～32个月)。RFTC毁损后2、12、24个月的无癫痫发作率(比例)分别为58.3%(21/36)、30.0%(6/20)和1/7。末次随访结果显示, 预后良好(Engel分级Ⅰ、Ⅱ级)17例(47.2%), 预后不良(Engel分级Ⅲ、Ⅳ级)19例(52.8%)。单因素分析结果表明, 癫痫发作类型为未继发强直-阵挛发作、MRI显示有明显病灶、正电子发射断层显像术(PET)检查结果提示有明显的低代谢区、癫痫发作模式(SOP)为SOP1和SOP2、采用根治性RFTC毁损、RFTC毁损...     

脑室周围结节型灰质异位所致癫痫的诊疗的研究进展

脑室周围结节型异位(periventricular nodular heterotopia, PNH)是一种由神经元细胞迁移障碍引起的脑发育畸形病,表现为脑室周围存在结节状的异位灰质。PNH可能是由于相关基因的突变所致。在PNH的MRI图像上可以看见脑室周围存在结节状异位灰质,其信号强度与大脑皮质一致。功能磁共振等影像学技术也开始应用于PNH的研究,并取得了一定的成果。PNH最常见的临床症状为癫痫,因此如何确定致癫区和切断其癫痫样电活动传播是治疗的关键,脑电图(EEG)技术成为了诊断PNH相关癫痫的必不可少的方法,特别是立体定向脑电图(SEEG)技术的出现使得癫痫的诊疗方面有了极大地进步。PNH所致的难治性癫痫的的外科治疗包括传统切除手术、射频热凝治疗(radiofrequency thermal coagulation, RF-TC)和激光间质内热凝(laserinterstitial thermal therapy LITT),目前以RF-TC为主要的治疗方式。     

Bilateral periventricular nodular heterotopia associated with coeliac disease and palatoschisis

Periventricular nodular heterotopia (PNH) is considered a distinct entity in relation to the other forms of neuronal migration disorders (NMD), because PNH patients usually have normal neurological and mental examination results. We report the case of a 48-year-old woman with bilateral periventricular nodular heterotopia associated with epilepsy, coeliac disease, palatoschisis and other dysmorphic features. Her intelligence quotient (I.Q.) and the results of a neurological examination were normal, but she suffered from a drug-resistant epileptic syndrome characterised by predominantly generalised and sporadic partial seizures. It has recently been suggested that an X-linked dominant inheritance may play a role in bilateral periventricular nodular heterotopia, and it is thought that a genetic defect is probably responsible for coeliac disease. In our patient, a genetic disorder may have produced both diseases and the dysmorphic syndrome, although the coexistence of PNH, epileptic seizures, coeliac disease and palatoschisis could be coincidental. Further observations are needed to ascertain whether the simultaneous presence of these disorders is simply an unusual association of unrelated pathologies or a new and distinct pathological entity.     

Automated morphometric magnetic resonance imaging analysis for the detection of periventricular nodular heterotopia

Purpose: To describe a novel magnetic resonance imaging (MRI) postprocessing technique for the detection of periventricular nodular heterotopia (PNH) and to evaluate its diagnostic value. The method is a further development of voxel‐based morphometric analysis with focus on a region of interest around the lateral ventricles to increase the sensitivity and specificity for automated detection of abnormally located gray matter in this area. Methods: T₁‐weighted MRI volume data sets were normalized and segmented in statistical parametric mapping (SPM 5 software), and the distribution of gray matter was compared to a normal database. As a new approach, individual masks derived from segmentation of the lateral ventricles were used to restrict the search for ectopic gray matter to the periventricular area. PNH were automatically detected by localizing the maximum deviation from the normal database in this area, provided that the z‐score exceeded a certain threshold. The optimal z‐score threshold for maximum sensitivity and specificity was determined by a receiver operating characteristic (ROC) curve analysis. The method was applied in 40 patients with PNH and 400 controls. Key Findings: PNH were detected in 37 of 40 patients, and false positives were found in 34 of 400 controls, amounting to 92.5% sensitivity and 91.5% specificity. In 17 of the patients in whom PNH could be identified, these lesions had been overlooked in the past, and in 8 patients even in the high‐resolution MRI subsequently used for postprocessing. Significance: The results suggest that automated morphometric MRI analysis with focus on ectopic gray matter in the periventricular areas facilitates the evaluation of MRI data and increases the sensitivity for the detection of PNH.     

Early cognitive and behavioral problems in children with nodular heterotopia

Adults with periventricular nodular heterotopia (PNH) have epilepsy and dyslexia, but most have normal intelligence. It is not known whether PNH-related reading difficulty can be detected earlier in childhood or whether associated behavioral problems are present. We studied 10 children with PNH, 3 of whom did not have seizures, and 10 matched controls with neuropsychological testing and parental rating instruments at two time points separated by about 1 year. Children with PNH performed significantly worse than controls on a task related to reading fluency. In addition, those with PNH showed significantly worse adaptive skills, and a measure of conduct problems significantly worsened over time. Mood and behavioral problems were reported more commonly, though not significantly so, in children with PNH. These findings demonstrate that reading dysfluency can be evident in children with nodular heterotopia, even in the absence of epilepsy, but also highlight difficulties with behavior in this population.     

Gray matter volumes and cognitive ability in the epileptogenic brain malformation of periventricular nodular heterotopia

Periventricular nodular heterotopia (PNH) is a brain malformation clinically characterized by the triad of epilepsy, normal intelligence, and dyslexia. We investigated the structure–function relationship between cerebral volumes and cognitive ability in this disorder by studying 12 subjects with PNH and 6 controls using volumetric analysis of high-resolution anatomical MRI and neuropsychological testing. Total cerebral volumes and specific brain compartment volumes (gray matter, white matter, and cerebrospinal fluid) in subjects with PNH were comparable to those in controls. There was a negative correlation between heterotopic gray matter volume and cortical gray matter volume. Cerebral and cortical volumes in PNH did not correlate with Full Scale IQ, unlike in normal individuals. Our findings support the idea that heterotopic nodules contain misplaced neurons that would normally have migrated to the cortex, and suggest that structural correlates of normal cognitive ability may be different in the setting of neuronal migration failure.