致痫性局灶性脑皮层发育不良患者的PET-CT与MRI研究

目的 研究致痫性局灶性脑皮层发育不良(FCD)的PET-CT与MRI检查特点,评价其在癫痫外科术前致痫灶定位诊断中的应用价值.方法 回顾性分析2009年1月至2011年10月珠江医院神经外科癫痫病灶切除术后病理证实为FCD患者临床资料,总结其中39例资料完整患者的病灶分布、病理亚型、MRI及PET-CT影像特点、预后等指标,分析各指标间的相互关系.结果 本组MRI阳性31例,主要表现为灰白质交界不清、局部皮层增厚和形态异常、T2WI与Flair 像上白质信号增高等特点.其中T2、Flair高信号在FCDⅡb型非常常见,而FCD Ⅰ型则鲜有表现.PET-CT病灶阳性表现36例,略高于MRI阳性率.按照Engel预后分级法,本组Ⅰ级74％(29/39),Ⅱ级18％ (7/39),Ⅲ级5％ (2/39),Ⅳ级3％(1/39).结论 在致痫性FCD病例中,MRI主要表现为灰白质交界不清、局部皮层增厚和形态异常、T2WI与Flair像上白质信号增高等特点.PET-CT检查可提高单纯MRI检查的准确性.     

儿童局灶性皮质发育不良的磁共振高分辨成像特征

目的探讨儿童局灶性皮质发育不良(Focal cortical dysplasia,FCD)的3D高分辨核磁共振(MRI特征。方法回顾性分析2015年4月-2018年6月山东大学齐鲁儿童医院收治的42例经病理证实为FCD的患儿MRI资料,观察下述征象:局灶性灰白质分界模糊、皮质结构异常(增厚或变薄)、T2WI/FLAIR白质信号增高,伴或不伴transmantle征(皮层下白质内向脑室方向延伸的异常信号),T2WI/FLAIR灰质信号增高,异常脑沟或脑回形态及节段性和/或脑叶发育不全/萎缩。结果 42例患儿中,37例(88.1%)可见MRI阳性征象,FCDⅠ型13例(35.1%),主要MRI特征为局灶性灰白质分界模糊、相应部位皮质结构异常及T2WI/FLAIR白质信号增高;FCDⅡ型17例(45.9%),表现为局灶性灰白质分界模糊及皮质结构异常、T2WI/FLAIR白质信号增高及transmantle征;FCDⅢ型共7例(18.9%),其中海马萎缩2例(28.6%)、胚胎发育不良性神经上皮瘤(Dysembryoplastic neuroepithelial tumor,DNET) 2例(28.6%)、节细胞瘤1例(14.3%)、软化灶并胶质增生2例(28.6%)。结论 FCD患儿的3D高分辨MRI特征具有特异性,可提高FCD病灶检出率。     

脑皮质发育不良继发顽固性癫痫的病理分型及手术治疗

目的 探讨局灶性脑发育不良(FCD)的临床特征、病理学、影像学的特点及手术疗效.方法 42例外科手术切除致痫灶并经病理证实为FCD的患者中,根据Palmini病理学分型进行分类,并对其临床特征、影像学特点及手术疗效进行回顾性分析.结果 42例患者中,按致痫灶部位分类颢叶24例、额叶14例、顶叶6例及枕叶3例,其中多脑叶5例.术前影像学检查阳性率62%.组织学分型FCDⅠA型9例,FCDⅠB型21例,FCDⅡA型5例,FCDⅡB型7例,其中以FCD Ⅰ B型最为常见,多位于颞叶且常伴有海马硬化.所有患者术后至少随访1年以上,癫痫术后治愈率FCD位于颞叶67%,颞叶以外43%(EngleⅠa).结论 FCD是难治性癫痫常见的病理学改变,其病理分型与临床特征和致痫灶部位存在相关性,为制定手术方案和判定手术效果提供了依据.     

难治性癫痫172例临床病理分析

目的 探讨难治性癫痫病灶切除手术患者的临床病理学分型及特点.方法 收集清华大学玉泉医院2008年1月至2009年6月,172例难治性癫痫手术治疗患者的病理标本及临床资料.采用HE和免疫组化染色,探讨各种类型癫痫病灶的临床病理学特点.结果 经病理诊断,局灶皮质发育不良138例(其中FCD Ⅰ B 115例、FCDⅡA 15例、FCDⅡB 8例)、微发育不良2例、胚胎发育不良性神经上皮瘤4例、Rasmussen脑炎7例、瘢痕脑回16例,节细胞瘤、多小脑回畸形、血管畸形、下丘脑错构瘤及结节性硬化各1例.结论 局灶皮质发育不良为引发难治性癫痫最常见病因,其中以FCDⅠ B亚型最为多见.     

18氟-氟脱氧葡萄糖-正电子发射断层显像在评估局灶性皮质发育不良所致难治性癫痫致痫区中的应用

我国目前有癫痫患者逾千万,其中30％的患者经过规范的药物治疗后不能满意地控制发作,发展为难治性癫痫.针对其病因学的研究发现,造成难治性癫痫的脑组织病变主要包括皮质发育不良和肿瘤两大类[1].局灶性皮质发育不良(focal cortical dysplasia,FCD)是局限的大脑皮质发育畸形,越来越多的新皮质病灶被高分辨MRI发现,其术后病理均提示FCD的存在[2].然而不少FCD患者高分辨MRI仅表现为局灶性皮质增厚、灰质-白质交界不清,或轻微的白质高信号,这些微小改变很容易漏诊,而且约30％ FCD(特别是Ⅰ型FCD)患者的MRI表现是阴性的[3].     

局灶皮层发育不良致难治性癫痫160例临床病理分析

目的探讨局灶皮层发育不良的临床病理学分型及特点。方法收集160例病理诊断为局灶皮层发育不良的难治性癫痫手术治疗患者的病理标本及临床资料。采用HE和免疫组织化学染色,探讨各种类型癫痫病理的临床病理学特点。结果局灶皮层发育不良160例,其中FCDⅠB134例、FCDⅡA16例、FCDⅡB10例。结论局灶皮层发育不良为引发难治性癫痫最常见病因,而亚型中以FCDⅠB型最为多见。免疫组化有助于FCD的诊断与分型。     

局灶性脑皮质发育不良致难治性癫痫病理分型和手术疗效分析

目的 探讨难治性癫痫术后局灶性脑皮质发育不良(FCD)不同病理分型与术后疗效的相巨关系.方法 回顾性分析54例经手术治疗后,病理证实为FCD的药物难治性癫痫患者的临床资料,分析病理分型与手术预后的关系.结果 轻型组(FCD Ⅰ A)24例,重型组(FCD Ⅰ B+ⅡA+ⅡB)30例.术后有效率:轻型组96%,重型组70%,总有效率82%.结论 随着FCD病理改变程度的逐渐加重,手术后疗效越来越差,FCD可能是影响药物难治性癫痫术后疗效的一个重要因素.     

3例局灶性皮质发育不良Ⅰ型诊治思考并文献复习

目的探讨局灶性皮质发育不良(FCD)Ⅰ型的临床临床特点及诊治过程,以期提高对该病的认识和重视,早期获得正确合理的治疗。方法回顾性分析3例FCDⅠ型致难治性癫痫的临床资料,并结合文献总结分析其临床特点。结果经临床、影像学、视频脑电及术后病理常规检查明确诊断为FCDⅠ型。该3例患者均表现为儿童期发病,临床表现为癫痫发作。1例患者术前MRI发现病灶,呈阳性表现;另外2例术前MRI未见明显异常,呈阴性表现。3例患者均行PET-CT检查,例1示右侧顶叶较对侧摄取FDG明显减低;例2表现为右侧额中回异常信号;例3表现为双侧额叶、左侧颞叶放射性摄取减低。术后常规病理检查均确诊为FCDⅠ型,术后恢复良好无功能损害。结论 FCDⅠ型临床常表现为难治性癫痫,多于儿童时期发病,可以发生于大脑的任意脑叶,头部MRI可呈阴性表现,手术治疗是主要的治疗方法。     

脑局灶性皮层发育不良相关性难治性癫痫的临床病理分析

目的探讨脑局灶性皮层发育不良(FCD)相关性难治性癫痫的临床病理特点。方法对29例2000年1月至2009年8月在本院接受难治性癫痫外科手术并病理诊断为FCD患者的临床资料、神经影像学以及病理学资料进行回顾性分析。并对所有患者进行随访,包括术后服用抗癫痫药物、影像学检查以及癫痫改善或复发的情况。结果 29例患者平均年龄23.5岁,平均病程11.3年,发作形式以复杂部分性发作为主。影像学检查有4例可见海马硬化。病理组织学以FCDⅠ型多见,具体分型为轻微皮层发育不良(mildMCD)3例,FCDⅠa型6例,FCDⅠb型10例,FCDⅡa型5例,FCDⅡb型5例。从发病部位来看,额叶最多见(15例),其次为颞叶(8例),顶叶(6例)。具有双重病理改变的有4例(FCDⅠa型伴海马硬化2例,FCDⅠb型伴海马硬化1例,FCDⅡb伴海马硬化1例)。5例合并胚胎发育不良性神经上皮瘤(DNT)。免疫组化染色示巨大神经元、未成熟神经元、形态异常神经元及白质内异位神经元NeuN均阳性,少数气球细胞呈nestin阳性表达。术后所有病例影像学复查无FCD改变,癫痫控制结果Ⅰa级6例,Ⅰ级5例,Ⅱ级3例,Ⅲ级2例,Ⅳ级13例。手术切除治疗后随访5个月以上,总治愈率为16/29,其中轻型组8/9,重型组8/20。结论 FCD相关性难治性癫痫中FCDⅠb型为最多见类型,分型与预后有关。     

57例皮质发育异常相关性癫痫临床病理分析

目的 探讨皮质发育异常(MCD)相关癫痫的临床病理特征.方法 回顾性分析57例MCD相关癫痫的临床病理资料,分析其病理学特征.结果 MCD相关癫痫占同期手术治疗癫痫的43%.57例MCD相关癫痫中,脑沟回结构紊乱8例;皮质微发育不良(MD)8例;局灶性皮质发育不良(FCD)41例,其中FCD Ⅰ A 13例,FCD Ⅰ B 15例,FCD Ⅱ A7例,FCD Ⅱ B 6例.57例MCD中22例伴海马硬化;1例FCDⅡB伴胚胎发育不良性神经上皮瘤(DNT);1例FCDⅡB局部向神经节神经胶质瘤(GGs)过渡.结论 MCD与难治性癫痫关系密切,以FCD Ⅰ型最为常见,多数病例伴有海马硬化.     

高分辨MRI在Ⅰ型局灶性皮质发育不良患儿术前评估中的应用

目的 探讨高分辨MRI成像在儿童Ⅰ型局灶性皮质发育不良(FCD)术前评估中的价值.方法 回顾性分析52例经病理学证实的FCD Ⅰ型患儿的MRI及相关临床资料,比较高分辨成像与MRI常规序列对Ⅰ型FCD各主要MRI征象(局灶性灰白质分界模糊、局灶性皮质结构异常、白质异常信号灶及局限性脑叶萎缩/发育不全)的检出率,以及对病...     

Diffusion tensor imaging abnormalities in focal cortical dysplasia

PURPOSE: Focal cortical dysplasia (FCD) is one of the most common underlying pathologic substrates in patients with medically intractable epilepsy. While magnetic resonance imaging (MRI) evidence of FCD is an important predictor of good surgical outcome, conventional MRI is not sensitive enough to detect all lesions. Previous reports of diffusion tensor imaging (DTI) abnormalities in FCD suggest the potential of DTI in the detection of FCD. The purpose of this study was to study subcortical white matter underlying small lesions of FCD using DTI. METHODS: Five patients with medically intractable epilepsy and FCD were investigated. Diffusion tensor imaging images were acquired (20 contiguous 3 mm thick axial slices) with maps of fractional anisotropy (FA), trace apparent diffusion coefficient (trace/3 ADC), and principal eigenvalues (ADC parallel and ADC perpendicular to white matter tracts) being calculated for each slice. Region of interest analysis was used to compare subcortical white matter ipsilateral and contralateral to the lesion. RESULTS: Three subjects with FCD associated with underlying white matter hyperintensities on T2 weighted MRI were observed to have increased trace/3 ADC, reduced fractional anisotropy and increased perpendicular water diffusivity which was greater than the relative increase in the parallel diffusivity. No DTI abnormalities were identified in two patients with FCD without white matter hyperintensities on conventional T2-weighted MRI. CONCLUSIONS: While DTI abnormalities in FCD with obvious white matter involvement are consistent with micro-structural degradation of the underlying subcortical white matter, DTI changes were not identified in FCD lesions with normal appearing white matter.     

Different presurgical characteristics and seizure outcomes in children with focal cortical dysplasia type I or II

Purpose:   Cortical dysplasia (FCD) is a frequent cause of epilepsy in childhood. Two major pathological variants are distinguished, FCD type I and II. The aim of the study was to characterize differences between FCD type I and II with respect to imaging and EEG findings, clinical and neuropsychological presentations, and surgical outcome.
Methods:   Forty children with refractory epilepsy and histopathologically confirmed FCD were retrospectively analyzed. FCD type I was identified in 24 and FCD type II in 16 patients.
Results:   Characteristic MRI abnormalities in FCD type I included subtle white matter signal changes and regional reduction of the white matter volume. Typical MRI findings in FCD type II were increased cortical thickness, transmantle sign, gray-white matter junction blurring, fluid-attenuated inversion recovery (FLAIR) and proton density (PD) gray matter signal changes as well as T1w, and PD white matter signal changes. Continuous EEG slowing was significantly more common in patients with FCD type I. Children with FCD type I presented with lower levels of intelligence and were suffering more often from maladaptive behavior and behavioral disorders. Surgical outcome was significantly worse in the FCD type I group (seizure freedom was achieved in 21% FCD type I patients and in 75% FCD type II subjects, p < 0.001).
Conclusions:   Clinically important differences were found in children with distinct histopathological subtypes of FCD. Due to prominent neuropsychological deficits and worse seizure outcome, treatment strategies in FCD type I are more challenging than previously reported and these children should be recognized and specifically addressed within the incoherent group of patients with malformative brain disorders.     

Comparison of MRI features and surgical outcome among the subtypes of focal cortical dysplasia

PurposeFocal cortical dysplasia (FCD) is the most common pathological diagnosis in patients who have undergone surgical treatment for intractable neocortical epilepsy. However, presurgical identification of MRI abnormalities in FCD patients remains difficult, and there are no highly sensitive imaging parameters available that can reliably differentiate among FCD subtypes. The purpose of our study was to investigate the surgical outcome in FCD patients with identifiable MRI abnormalities and to evaluate the prognostic role of the various MRI features and the characteristics of FCD pathology.MethodsWe retrospectively recruited epilepsy patients who had undergone surgical treatment for refractory epilepsy with focal MRI abnormalities and the pathological diagnosis of FCD. We evaluated the surgical outcome according to the pathological subtypes, and studied the prognostic roles of various MRI features. We used recently proposed three-tiered FCD classification system which included FCD type III when FCD occurs in association with other potentially epileptogenic pathologies.ResultsA total of 69 patients were included, and 68.1% of patients became seizure free. Patients with FCD type III had a lower chance for achieving seizure freedom (7/15) than in patients with isolated FCD (FCD types I and II) (40/54, p = 0.044). Cortical thickness and blurring of gray–white matter junction were more common in isolated FCD than in FCD type III, but most MRI features failed to differentiate between FCD types I and II, and only the transmantle sign was specific for FCD type II. We failed to find a prognostic value of specific MRI abnormalities of prognostic value in terms of post-epilepsy surgery outcome in FCD patients.ConclusionsOur study showed that patients with FCD III have poor surgical outcome. Typical MRI features of isolated FCD such as cortical thickness and blurring of gray–white matter junction were less common in FCD type III and only transmantle sign was helpful in differentiating between FCD types I and II.     

磁共振阴性的局灶性脑皮质发育不良的手术治疗

目的探讨磁共振难以确定病灶的致痫性局灶性脑皮质发育不良的诊断和定位方法,提高手术治疗效果。方法回顾性分析联合应用视频脑电图(VEEG)、脑磁图(MEG)及术中皮层电极脑电图监测(ECo G)检查,诊断、定位并经手术后病理证实为局灶性皮质发育不良(FCD)的24例磁共振检查阴性的难治性癫痫患者的临床资料。结果 24例癫痫患者行手术治疗,病理FCDⅠa型5例,FCDⅠb型3例,FCDⅠc型5例,FCDⅡa型6例,FCDⅡb型5例。术后随访1~5年,EngelⅠ级9例,EngelⅡ级5例,EngelⅢ级8例,EngelⅣ级2例。结论联合应用VEEG、MEG和(或)ECo G技术有助于准确诊断和定位磁共振阴性的FCD,提高FCD致难治性癫痫的手术疗效。     

In vivo profiling of focal cortical dysplasia on high-resolution MRI with computational models

PURPOSE: On MRI, focal cortical dysplasia (FCD) is characterized by a combination of increased cortical thickness, hyperintense signal within the dysplastic lesion, and blurred transition between gray and white matter (GM-WM). The visual identification of these abnormal characteristics may be difficult, and it is unclear to what degree these features occur among different FCD lesions. Our purpose was to investigate the pattern of occurrence of abnormal MRI characteristics in FCD by using a set of computational models and to generate quantitative lesion profiling. METHODS: A set of voxel-wise operators was applied to high-resolution 3D T1-weighted MRI in 23 patients with histologically proven FCD and 39 healthy controls, creating maps of GM thickness, maps of relative intensity highlighting areas with hyperintense signal, and maps of gradient magnitude modeling the GM-WM transition. All FCD lesions were segmented manually on the T1-weighted MRI. RESULTS: FCD volumes ranged from 734 mm3 to 80,726 mm3 (mean, 8,629 mm3 +/- 16,238). The manually segmented FCD lesions were used to estimate features in the lesional area and to determine possible local variations of each feature by means of a histogram. In 78% of the patients, FCD lesions were characterized by simultaneous GM thickening, hyperintense signal, and blurring of the GM-WM transition. Moreover, in all patients, the FCD lesion had at least two of these three characteristics. CONCLUSIONS: The three features occurred regardless of the lesion volume, and they characterized not only large FCD lesions, but also subtle ones that had been overlooked by conventional radiologic inspection before surgery.     

Role of MRI in patient selection for surgical treatment of intractable epilepsy in infancy

Epilepsy surgery is an effective treatment in selected patients with localization-related intractable epilepsy. The success of epilepsy surgery is in part dependent upon identification of a lesion on MRI. In infants, the surgical epileptogenic substrates include focal cortical dysplasia (FCD), hemimegalencephaly, tuberous sclerosis complex, Sturge Weber syndrome, hypoxic-ischemic or cerebrovascular injury and low-grade tumor. The sensitivity of MRI in identifying the epileptogenic substrate is influenced by the nature of the epileptogenic substrate, MRI technique and expertise of the interpreting physician. The MRI features of some lesions such as FCD may differ in infants compared to children and adults; the white matter adjacent to FCD may demonstrate lower T2 and higher T1 signal in some infants due to premature myelination, while in others, the white matter demonstrates higher T2 or lower T1 signal due to demyelination, dysmyelination or gliosis, similar to children and adults. The appearances of some lesions, such as FCD, may change with time, due to brain maturation or seizure related changes. MRI for patients with localization-related intractable epilepsy should have high-resolution, multiplanar and multisequence. In infants, volumetric T1 and high-resolution T2 imaging are recommended. FLAIR and proton density sequences are less helpful in infants due to lack of myelin in the white matter. The physician interpreting the scan should be familiar with the imaging appearances of epileptogenic substrates and may need to review the scan more than once if a lesion is not seen on initial inspection.     

Focal cortical dysplasia. Clinical-radiological-pathological associations

INTRODUCTION: The term focal cortical dysplasia (FCD) describes a particular migration disorder with a symptomatology mainly characterised by drug-resistant epileptic seizures, typical neuroradiological images, and histological characteristics, as well as a very positive response to surgical treatment in the majority of cases. MATERIAL AND METHODS: A total of 7 patients were studied, comprising 6 children with a mean age of 34.3 months and one 25-year-old male with very persistent focal seizures and MRI images that showed FCD. RESULTS: Three of the patients (all girls) were operated on while very young, with extirpation of the FCD and the surrounding area; with the histopathology study showed agreement between the MRI images and the macroscopic study of the slices. The histology study showed findings typical of a Taylor-type FCD (poor differentiation between the cortical grey matter and the subcortical white matter, and balloon cells). Three years after the FCD extirpation, the same 3 patients remained seizure-free with no anti-epilepsy medication. Two others have seizure control with medication, another (the adult) is on the surgical waiting list, and the remaining patient refused the operation. CONCLUSION: Taylor-type FCD is associated with a high percentage of all drug-resistant focal seizures, and it needs to be identified and extirpated as soon as possible. Well planned and well-performed surgery that leaves no remains of dysplasia can cure the disease it in many cases.