Resective surgery for focal cortical dysplasia in children: a comparative analysis of the utility of intraoperative magnetic resonance imaging (iMRI)

Purpose

Seizure freedom following resection of focal cortical dysplasia (FCD) correlates with complete resection of the dysplastic cortical tissue. However, difficulty with intraoperative identification of the lesion may limit the ability to achieve the surgical objective of complete extirpation of these lesions. Intraoperative magnetic resonance imaging (iMRI) may aid in FCD resections. The objective of this study is to compare rates of postoperative seizure freedom, completeness of resection, and need for reoperation in patients undergoing iMRI-assisted FCD resection versus conventional surgical techniques.

Methods

We retrospectively reviewed the medical records of pediatric subjects who underwent surgical resection of FCD at Children’s National Medical Center between March 2005 and April 2015.

Results

At the time of the last postoperative follow-up, 11 of the 12 patients (92 %) in the iMRI resection group were seizure free (Engel Class I), compared to 14 of the 42 patients (33 %) in the control resection group (p = 0.0005). All 12 of the iMRI patients (100 %) achieved complete resection, compared to 24 of 42 patients (57 %) in the control group (p = 0.01). One (8 %) patient from the iMRI-assisted resection group has required reoperation, compared to 17 (40 %) patients in the control resection group.

Conclusion

Our results suggest that the utilization of iMRI during surgery for resection of FCD results in improved postoperative seizure freedom, completeness of lesion resection, and reduction in the need for reoperation.

     

Epilepsy with dual pathology: Surgical treatment of cortical dysplasia accompanied by hippocampal sclerosis

Purpose: The presence of two or more epileptogenic pathologies in patients with epilepsy is often observed, and the coexistence of focal cortical dysplasia (FCD) with hippocampal sclerosis (HS) is one of the most frequent clinical presentations. Although surgical resection has been an important treatment for patients with refractory epilepsy associated with FCD, there are few studies on the surgical treatment of FCD accompanied by HS, and treatment by resection of both neocortical dysplastic tissue and hippocampus is still controversial. Methods: We retrospectively recruited epilepsy patients who had undergone surgical treatment for refractory epilepsy with the pathologic diagnosis of FCD and the radiologic evidence of HS. We evaluated the prognostic roles of clinical factors, various diagnostic modalities, surgical procedures, and the severity of pathology. Results: A total of 40 patients were included, and only 35.0% of patients became seizure free. Complete resection of the epileptogenic area (p = 0.02), and the presence of dysmorphic neurons or balloon cells on histopathology (p = 0.01) were associated with favorable surgical outcomes. Patients who underwent hippocampal resection were more likely to have a favorable surgical outcome (p = 0.02). Conclusions: We show that patients with complete resection of epileptogenic area, the presence of dysmorphic neurons or balloon cells on histopathology, or resection of hippocampus have a higher chance of a favorable surgical outcome. We believe that this observation is useful in planning of surgical procedures and predicting the prognoses of individual patients with FCD patients accompanied by HS.     

Taylor-type focal cortical dysplasia in infants: some MRI lesions almost disappear with maturation of myelination

Identification of focal cortical dysplasia (FCD) on magnetic resonance (MR) images of young children with refractory focal epilepsy is important, as surgical resection may offer improvement of seizure control and subsequent developmental progress. However, the MR appearances of malformations of cortical development may change during brain maturation. We report 4 children with refractory focal epilepsy, whose MR images in infancy showed localized cortical and subcortical signal abnormalities (hypointense on T(2)-weighted and hyperintense on T(1)-weighted images), suggestive of abnormal cortical development. The visibility of these lesions was significantly reduced on later MR images. Subtle blurring of the gray-white matter junction in these areas was the only indicator of cortical abnormality in 3 patients, which was recognized only after comparison with earlier images. Taylor-type FCD was subsequently confirmed in all patients, following surgical cortical resection of the lesions. MR images performed early within the first year of life in children with epilepsy are important to identify areas of FCD. The appearances of FCD on later scans can be very subtle escaping recognition, and conclusions may be misleading with respect to diagnosis and appropriateness of surgical treatment.     

Focal cortical dysplasia type IIb: completeness of cortical, not subcortical, resection is necessary for seizure freedom

Purpose: Focal cortical dysplasia type IIb (FCD IIb) lesions are highly epileptogenic and frequently cause pharmacoresistant epilepsy. Complete surgical resection leads to seizure freedom in most cases. However, the term “complete” resection is controversial with regard to the necessity of performing resections of the subcortical zone, which is frequently seen in these lesions on magnetic resonance imaging (MRI). Methods: We retrospectively analyzed 50 epilepsy patients with histologically proven FCD IIb. The extent of surgical resection was determined by SPM5‐based coregistration of the preoperative and postoperative MRI scans. Postoperative outcome was analyzed with regard to (1) the completeness of the resection of the cortical abnormality and (2) the completeness of the resection of the subcortical abnormality. Key Findings: Complete resection of the cortical abnormality led to postoperative seizure freedom (Engel class Ia) in 34 of 37 patients (92%), whereas incomplete cortical resection achieved this in only one of 13 patients (8%, p < 0.001). Among the patients with complete cortical resection, 36 had FCDs with a subcortical hyperintensity according to MRI. In this group, complete resection of the subcortical abnormality did not result in a better postoperative outcome than incomplete resection (90% vs. 93% for Engel class Ia, n.s.). Significance: Complete resection of the MRI‐documented cortical abnormality in FCD IIb is crucial for a favorable postoperative outcome. However, resection of the subcortical hyperintense zone is not essential for seizure freedom. Therefore, sparing of the subcortical white matter may reduce the surgical risk of encroaching on relevant fiber tracts. In addition, these findings give an interesting insight into the epileptogenic propensity of different parts of these lesions.     

Focal cortical dysplasia and intractable epilepsy in adults: clinical,EEG, imaging,and surgical features

PURPOSE: The clinical features of focal cortical dysplasia (FCD) in adults are poorly understood. The purpose of this report is to describe the clinical, electrographic, and neuroimaging characteristics of adults with FCD undergoing surgical resection for intractable epilepsy. METHODS: Case series of 55 patients, aged 17-57 years, with a histopathological diagnosis of FCD. Medical history, neurological examination, non-invasive video-EEG, neuroimaging, and surgical outcome data were analyzed retrospectively. RESULTS: There were 36 patients with temporal, 19 with extra-temporal lobe resections. Mean age at surgery was 29 years. Mean age at epilepsy onset was 10 years. Dual pathology was seen in 56% of patients, with 68% of these having hippocampal sclerosis (HS). Epilepsy risk factors included febrile seizures (16%), head trauma (16%), CNS infections (11%), and perinatal stroke (4%). Interictal EEG showed regional epileptiform activity in 89% of patients. Only 24% were diagnosed with FCD pre-operatively. Of those with dual pathology, only 6% were suspected of having FCD pre-operatively. Of those patients with >12 months follow-up, surgical outcomes were as follows: 65% seizure-free, 19% significant improvement, 16% without significant improvement. CONCLUSIONS: In this series of adult patients with intractable epilepsy and FCD, a significant number have other seizure risk factors, normal neurological examinations and neuroimaging, and regional EEG findings. Dual pathology was common in patients with FCD. FCD should be considered as an etiology of epilepsy even in patients whose evaluation suggests other mechanisms.     

颞叶外节细胞胶质瘤继发癫痫的手术治疗研究

目的研究颞叶外节细胞胶质瘤(GG)继发癫痫的临床特点和手术治疗方式及疗效。方法回顾性分析手术治疗并经病理证实的20例颞叶外节细胞胶质瘤继发癫痫患者的临床资料。术后癫痫控制效果按改良Engel分级标准评定;分析患者的发作表现、病变部位、影像学特点、病理改变、手术方式及病变切除程度与癫痫控制的关系。结果节细胞胶质瘤位于额叶者2例、顶叶7例、枕叶5例、颞枕交界处6例。MRI检查显示肿瘤为囊性者5例、囊实性6例、实性9例;增强扫描病变有强化12例,病变周围水肿5例。CT检查示病变有钙化4例。癫痫发作表现为简单部分性发作(伴意识保留的局灶性发作)者4例,复杂部分性发作(伴意识障碍的局灶性发作)5例,继发性全面性强直阵挛(局灶进展到双侧的强直阵挛发作)11例。肿瘤次全切除者2例,全切除15例,扩大切除3例。术后病理检查示合并脑皮质发育不良者2例。术后随访1~3年,癫痫控制效果为EngelⅠ级17例(85.0%),Ⅱ级2例(10.0%),Ⅲ级1例(5.0%)。预后相关临床因素分析显示,肿瘤切除的方式和程度与预后有关。结论颞叶外节细胞胶质瘤继发癫痫多为药物难治性,手术切除可取得良好效果。术前须综合评估病变部位和临床、影像学特点等资料制定手术策略,术中尽可能行肿瘤加致痫皮层全切除,以提高疗效。必要时可考虑先行SEEG电极植入后,再行致痫灶精准切除术。     

A comprehensive clinico‐pathological and genetic evaluation of bottom‐of‐sulcus focal cortical dysplasia in patients with difficult‐to‐localize focal epilepsy

Aims. We comprehensively studied the clinical presentation, stereo‐EEG and MRI findings, histopathological diagnosis, and brain somatic mutations in a retrospective series of drug‐resistant patients with difficult‐to‐localize epilepsy due to focal cortical dysplasia at the bottom of a sulcus (BOS‐FCD). Methods. We identified 10 patients with BOS‐FCD from the Cleveland Clinic epilepsy surgery database submitted for intracranial video‐EEG monitoring. Brain MRI, including voxel‐based morphometric analysis and surgical tissue submitted for histopathology, was reviewed. Paraffin tissue samples from five patients were made available for targeted next‐generation sequencing. Postsurgical follow‐up was available in nine patients. Results. BOS‐FCD was identified in the superior frontal sulcus in six patients, inferior frontal sulcus in one patient, central sulcus in one patient, and intraparietal sulcus in two patients. All patients had stereotyped seizures. Intracranial EEG recordings identified ictal onset at the BOS‐FCD in all 10 patients, whereas ictal scalp EEG had a localizing value in only six patients. Complete resection was achieved by lesionectomy or focal corticectomy in nine patients. Histopathologically, six patients had FCD type IIb and three had FCD type IIa. Next‐generation sequencing analysis of DNA extracted from lesion‐enriched (micro‐dissected) tissue from five patients with FCD type II led to the identification of a germline frameshift insertion in DEPDC5, introducing a premature stop in one patient. Eight out of nine patients with available follow‐up were completely seizure‐free (Engel Class IA) after a mean follow‐up period of six years. Conclusion. Our results confirm previous studies classifying difficult‐to‐localize BOS‐FCD into the emerging spectrum of FCD ILAE type II mTORopathies. Further studies with large patient numbers and ultra‐deep genetic testing may help to bridge the current knowledge gap in genetic aetiologies of FCD.     

Prospective magnetic resonance imaging identification of focal cortical dysplasia,including the non—balloon cell subtype

The purpose of this study was to determine the role of high-resolution T2-weighted fast multiplanar inversion-recovery (FMPIR) magnetic resonance (MR) imaging in detecting and delineating microscopic focal cortical dysplasia (FCD). We performed MR scans with FMPIR on 42 patients with suspected neocortical epilepsy. Ten MR studies were read prospectively as showing FCD; these case histories, electroencephalographic studies, and neuroimaging data were reviewed. Eight of these patients subsequently underwent focal cortical resection guided by intraoperative electrocorticography. The MR findings were correlated with pathological findings in these 8 patients. For purposes of radiological-pathological correlation, the FCD lesions were divided into two classes. Radiological classification was based on the absence (type A) or presence (type B) of T2 prolongation of the subcortical white matter. Pathological grading as type I or type II was based on a previously described pathological grading system. Specific MR findings associated with FCD included focal blurring of the gray-white matter interface (n = 9), thickening of the cortical ribbon (n = 7), and T2 prolongation of the subcortical white matter (n = 4). In 3 patients, the only MR finding that suggested FCD was localized blurring of the gray-white matter junction. In 2 of these 3 patients, the MR diagnosis of FCD could be made only by FMPIR. FCD was confirmed histologically in 7 of 8 patients, with insufficient tissue for complete histopathological evaluation in 1 case. Radiological classification of FCD agreed with pathological classification in 5 of 7 cases. Correlation of MR findings with intraoperative electrocorticography results indicated that the MR study localized the epileptogenic lesion correctly in 8 of 8 cases. Scalp ictal electroencephalographic studies localized the epileptogenic lesion in 5 of 8 cases; positron emission tomographic scans were focally abnormal in 3 of 3 cases. FMPIR MR imaging permitted accurate diagnosis and localization of FCD in all patients with pathologically proved FCD. MR identification of FCD aided presurgical planning and intraoperative management of these patients.     

Neuroimaging of Focal Cortical Dysplasia

Focal cortical dysplasia (FCD) is a common cause of pharmacoresistant epilepsy that is amenable to surgical resective treatment. The identification of structural FCD by magnetic resonance imaging (MRI) can contribute to the detection of the epileptogenic zone and improve the outcome of epilepsy surgery. MR epilepsy protocols that include specific T1 and T2 weighted, and fluid-attenuated inversion recovery (FLAIR) sequences give complementary information about the characteristic imaging features of FCD; focal cortical thickening, blurring of the gray-white junction, high FLAIR signal, and gyral anatomical abnormalities. Novel imaging techniques such as magnetic resonance spectroscopy (MRS), magnetization transfer imaging (MTI), and diffusion tensor imaging (DTI) can improve the sensitivity of MR to localize the anatomical lesion. Functional/metabolic techniques such as positron emission tomography (PET), ictal subtraction single photon emission computed tomography (SPECT), functional MRI (fMRI), and magnetic source imaging (MSI) have the potential to visualize the metabolic, vascular, and epileptogenic properties of the FCD lesion, respectively. Identification of eloquent areas of cortex, to assist in the surgical resection plan, can be obtained non-invasively through the use of fMRI and MSI. Although a significant number of FCD lesions remain unidentified using current neuroimaging techniques, future advances should result in the identification of an increasing number of these cortical malformations.     

Neuroimaging in identifying focal cortical dysplasia and prognostic factors in pediatric and adolescent epilepsy surgery

Purpose: The purpose of this study is to determine the sensibility of each imaging tool in identifying focal cortical dysplasia (FCD) in children and adolescents with epilepsy and to define the prognostic factors of pediatric and adolescent epilepsy surgery. Methods: We identified 48 children with FCD who underwent resective surgery and analyzed their preoperative data. The results of various anatomic and functional neuroimaging studies were compared for accuracy in locating the lesion. We also investigated clinical factors that affected the outcome of surgical treatment. Key Findings: Brain magnetic resonance imaging (MRI) was able to localize FCD in 30 patients and fluorodeoxyglucose positron emission tomography (FDG‐PET) and/or subtraction ictal single photon emission computed tomography (SPECT) coregistered with MRI provided additional information that helped to define the lesion in 13 patients. When comparing the pathologic results between a mild malformation of cortical development (MCD) and FCD type I and II, we noted a strong tendency for patients with FCD to have MRI abnormalities (p = 0.005). In addition, severe pathologic features (Palmini’s classification, FCD type II) (p = 0.025) showed significant correlation with a better surgical outcome. To define the primary epileptogenic area, various interictal epileptiform discharges and the results of multimodal neuroimaging studies were helpful, and younger age at the time of operation could aid in more favorable surgical outcomes (p = 0.048). Significance: Our study showed a significant relationship between pathologic grade and the detectability of FCD by brain MRI. In addition, early surgery can be justified by showing that advanced neuroimaging studies in children with FCD and even with extensive epileptiform discharges have a higher rate of success.     

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.     

Intraoperative ultrasound to define focal cortical dysplasia in epilepsy surgery

Focal cortical dyplasia (FCD) is a frequent cause of medication-resistant focal epilepsy. Patients with FCD may benefit from epilepsy surgery. However, it is difficult to intraoperatively define lesion boundaries. In this case report we present a novel tool to identify FCD intraoperatively. A patient with frontal lobe epilepsy underwent resection of a left frontomesial FCD. Image guidance was achieved by intraoperative ultrasound, which depicted the lesion with a higher resolution than preoperative MRI. Postoperatively the patient remained seizure free. Intraoperative ultrasound may be helpful in identifying and targeting subtle epileptogenic lesions, which are difficult to visualize.     

Low-grade focal cortical dysplasia is associated with prenatal and perinatal brain injury

Purpose: Prenatal and perinatal adverse events are reported to have a pathogenetic role in focal cortical dysplasia (FCD). However, no data are available regarding the prevalence and significance of this association. A cohort of children with significant prenatal and perinatal brain injury and histologically proven mild malformations of cortical development (mMCD) or FCD was analyzed. Methods: We retrospectively evaluated a surgical series of 200 patients with histologically confirmed mMCD/FCD. Combined historical and radiologic inclusion criteria were used to identify patients with prenatal and perinatal risk factors. Electroclinical, imaging, neuropsychological, surgical, histopathologic, and seizure outcome data were reviewed. Results: Prenatal and perinatal insults including severe prematurity, asphyxia, bleeding, hydrocephalus, and stroke occurred in 12.5% of children with mMCD/FCD (n = 25). Their epilepsy was characterized by early seizure onset, high seizure frequency, and absence of seizure control. Patients with significant prenatal and perinatal risk factors had more abnormal neurologic findings, lower intelligence quotient (IQ) scores, and slower background EEG activity than mMCD/FCD subjects without prenatal or perinatal brain injury. MRI evidence of cortical malformations was identified in 74% of patients. Most patients underwent large multilobar resections or hemispherectomies; 54% were seizure‐free 2 years after surgery. Histologically “milder” forms of cortical malformations (mMCD and FCD type I) were observed most commonly in our series. Conclusions: Surgically remediable low‐grade cortical malformations may occur in children with significant prenatally and perinatally acquired encephalopathies and play an important role in the pathogenesis of their epilepsy. Presurgical detection of dysplastic cortex has important practical consequences for surgical planning.     

Focal Cortical Dysplasias: clinical implication of neuropathological classification systems

Focal Cortical Dysplasias (FCDs) are highly epileptogenic brain lesions and are a frequent cause for drug-resistant focal epilepsies in humans. FCDs present with variable histopathological patterns, including architectural, cytoarchitectural or white matter abnormalities. Pathomechanisms compromising neuroblast proliferation, migration, or differentiation are likely to play a role in the etiology of FCD variants. FCDs were subsumed, therefore, into the broad spectrum of malformations of cortical development. The most frequent subtype comprises FCD Type II, which in general occurs as isolated lesion in extratemporal location and is histopathologically characterized by dysmorphic neurons (Type IIA) and balloon cells (Type IIB). Neuroimaging hallmarks include hyperintense T2-signaling and a “transmantle sign”. Electrophysiological recordings show peculiar interictal spike patterns and complete surgical resection results in favorable seizure control. In contrast, FCD Type I can be identified in young children with severe epilepsy and psychomotor retardation. Parietal, temporal, and occipital lobes may be involved in seizure generation, although neuroimaging often reveals normal contrast intensities. Surgical resection strategies ameliorate seizure frequencies in many children, whereas complete seizure relief can be achieved only in rare cases. According to the currently used FCD classification system, the same histopathological FCD Type I variant can be diagnosed as associated lesion in the large cohort of epilepsy patients with hippocampal sclerosis, low-grade glio-neuronal tumors, vascular malformations, or glial scarring. MRI is often not helpful to detect the dysplastic cortical areas. In addition, there is no specific electrophysiological pattern for an associated dysplastic lesion. Surgical resection of the epileptogenic area results, however, in favorable seizure control. These findings argue for a revised neuropathological classification system that distinguishes isolated versus associated FCD variants to obtain a better correlation with electro-clinical findings and prediction of postsurgical seizure control.     

Limited resection of focal cortical dysplasia and associated epileptogenic cortex may lead to positive surgical outcome

A drug-resistant epilepsy patient with premotor type IIb focal cortical dysplasia is described with a positive postoperative outcome following partial resection of the lesion and epileptogenic zone. Presurgical fMRI of the sensorimotor areas showed haemodynamic responses over the posterior border of the lesion and ictal EEG-fMRI revealed activation of both the primary sensorimotor strip and premotor lesion area. Almost continuous 1-2 Hz interictal spiking was recorded during a chronic ECoG study over the primary sensorimotor cortex. Following partial resection of the lesion, an acute ECoG revealed marked reduction of epileptic activity over the sensorimotor area. Post-operatively, seizure control was significantly improved (class IV ILAE outcome). Although partial FCD resections predict a worse postoperative outcome, individual patients may still respond favourably. The type of lesion, resected portion, and histopathology may be included among factors related to successful outcome.     

高场强术中磁共振对脑胶质瘤切除程度及手术策略的影响

目的 评价高场强术中磁共振(iMRI)对脑胶质瘤手术切除程度及手术策略的影响.方法 解放军总医院神经外科自2009年10月至2010年6月将高场强iMRI系统应用于胶质瘤切除术患者106例,术前了解术者的切除意图(全切、次全切、大部切除),术前1 d患者常规行MRI扫描,应用影像数据和软件计算术前肿瘤体积,术中常规使用神经导航手术,依据术者的需求采集影像.必要时行iMRI扫描计算术中残余肿瘤体积和肿瘤体积切除百分比,分析使用iMRI对肿瘤切除程度、手术策略的影响.结果 术前计划全切48例,次全切41例,大部切除17例.术中第一次扫描示42例(39.6%)完全切除,64例(60.4%)仍有残留,其中25例由于肿瘤与重要功能区或重要传导束紧邻而未作进一步切除,其余39例(36.8%)改进手术策略,标记出残留肿瘤后进一步手术,25例(23.6%)胶质瘤最终全切除,肿瘤体积切除百分比由(76.5±20.5)%提高到(94.2±8.7)%,差异有统计学意义(U=2.000,P=0.000);最终实际全切67例,次全切25例,大部切除14例,全切率有所提高.106例患者平均肿瘤体积切除百分比由第一次扫描时的(86.3±20.2)%提高到最终扫描时的(93.6±12.4)%,差异有统计学意义(U=4.000,p=0.000).结论 高场强iMRI的应用可显著提高脑胶质瘤的切除程度,改进手术策略.

Abstract：

Objective To evaluate the impact of high-field intraoperative magnetic resonance imaging (iMRI) on extension of resection and surgical strategy modification for glioma surgery. Methods One hundred and six patients, admitted to our hospital from October 2009 to June 2010, were performed glioma resections with the help of high-field iMRI. Questionnaires were filled and collected prospectively to record the surgeons' intention on the extent of resection (EoR) and the intra-operative estimation of EoR before every iMRI scan. The scan imagings were collected based on the request of the surgeon, and the percentage of tumor removal was calculated according to the iMRI data. The impact of iMRI on the tumor EoR and modification of surgical strategy was then evaluated. Results Preoperatively, 48 patients were intended to achieve total tumor removal, 41 sub-total tumor removal, and 17 partial removal. The first intraoperative MRI scan revealed that 42 (39.6%) patients achieved complete resection, while residual tumors were depicted in 64 (60.4%).Further tumor resections were performed in 39 patients (36.8%), but the other 25 patients could not perform further resection for their tumors were closely neighbored to the important functional region or important tracts. Finally, in the whole cohort, the percentage of tumor resection volume was increased from (76.5±20.5)% to (94.2±8.7)%, with significant differences (U=2.000, P=0.000); 67 patients got complete removal, 25 sub-total removal and 14 partial removal; The total removal rate was significantly increased from 45.3% (48/106) to 63.2% (67/106): the average percentage of tumor resection volume in the second time of scan ([93.6±12.41%) was obviously increased as compared with that in the first scan ([86.3±20.21%, U=4.000, P=0.000). Conclusion High-field iMRI may increase the extent of glioma resection, and has significant impact on the intraoperative modification of the surgical strategy.     

Voxel-based morphometry reveals excess gray matter concentration in patients with focal cortical dysplasia

PURPOSE: Many patients with focal cortical dysplasia (FCD) continue to have seizures after surgical treatment. The usual explanation for the poor surgical outcome is the presence of residual dysplastic tissue missed by the preoperative neuroimaging investigation and therefore not resected during surgery. We apply a voxel-based morphometry (VBM) analysis to the magnetic resonance imaging (MRI) scans from patients with epilepsy and visually detected FCD to investigate whether (a) VBM is able to detect gray-matter concentration (GMC) abnormalities in patients with FCD, and (b) whether the extent of GMC abnormalities in the brain of these patients differs from the regions observed by using visual inspection. METHODS: We studied 11 patients with visually detected FCD (eight of them with histologic confirmation of FCD). The GMC from each one of these patients was compared with the mean GMC from a control group of 96 normal healthy subjects by using an optimized VBM protocol. RESULTS: Ten of 11 patients showed statistically significant GMC excess, and among patients with GMC excess, only one showed GMC excess that was not exactly correspondent to the visually detected FCD. Seven patients exhibited excess in GMC extending beyond the area of visually detected FCD. CONCLUSIONS: This preliminary neuroimaging study suggests that (a) VBM can detect GMC excess in patients with FCD, and (b) GMC excess in these patients can extend to brain areas not visually defined as abnormal. Abnormal areas detected by VBM can possibly correspond to mild malformations of cortical development, supporting the notion that the surgical refractoriness observed in patients with FCD can be due to the incomplete resection of the dysplastic tissue.     

Predictors of outcome in pediatric epilepsy surgery

OBJECTIVE: To determine the correlation between pre- and perioperative variables on the outcome of children undergoing focal resections for medically intractable partial epilepsy. METHODS: Retrospective analysis of pre- and perioperative variables in a cohort of 75 patients younger than 12 years of age who underwent excisional surgery and had at least 1 year of follow-up. Outcome, measured by postoperative seizure frequency, was analyzed as a function of age at seizure onset, duration of epilepsy, presence of cognitive impairment, lobe of seizure origin, presence of a lesion, histopathology, and completeness of resection. Completeness of resection was defined on the basis of excising both the entire structural lesion if present and the region revealing prominent interictal and ictal abnormalities on intracranial EEG. RESULTS: Seventy-seven percent of patients had good outcomes (class 1 or 2), and 59% were seizure-free. Lesional status, site of resection, and pathologic diagnoses were not significant predictors of outcome except for in multilobar resection, for which overall outcome was relatively poor (44% class 3 or 4; 22% seizure-free). Completeness of resection was the only significant predictor of good outcome (p < 0.001), with 92% of patients who underwent complete resection of the epileptogenic zone achieving good outcome compared with 50% of patients who had incomplete resections. CONCLUSION: In this series of pediatric patients, complete resection of the lesion and the electrographically abnormal region was the main determinant of outcome after focal resections. Except for multilobar resections, other factors examined in this study did not significantly influence postoperative seizure prognosis and should not influence candidate selection for the surgical process.     

Neuropathological work-up of focal cortical dysplasias using the new ILAE consensus classification system - practical guideline article invited by the Euro-CNS Research Committee

FCDs are increasingly recognized in patients with drug-resistant epilepsies, and many patients benefit from tailored resection strategies. Yet, postsurgical seizure control cannot be sufficiently predicted and specification of FCD variants remains difficult during presurgical monitoring. The International League against Epilepsy (ILAE) has published a new consensus classification system for focal cortical dysplasias (FCDs). Based on a review of imaging data, electroclinical features and postsurgical seizure control correlation with neuropathological findings specify three clinico-pathological FCD subtypes: FCD Type I is characterized by aberrant radial (FCD Type Ia) or tangential lamination of the neocortex (FCD Type Ib) affecting one or multiple lobes. FCD Type II is characterized by cortical dyslamination and dysmorphic neurons without (Type IIa) or with balloon cells (Type IIb). It is important to note, that these types should not be associated with any other structural brain lesion (isolated FCD). In contrast, a new FCD Type III is introduced, which occurs in combination with hippocampal sclerosis (FCD Type IIIa), or with epilepsy-associated tumors (FCD Type IIIb). FCD Type IIIc is found adjacent to vascular malformations, whereas FCD Type IIId can be diagnosed in association with other epileptogenic lesions obtained in early life (i.e., traumatic injury, ischemic injury or encephalitis). Histopathological features are very similar to those observed in FCD Type I, but likely present postnatal development and maturation failures acquired by the principal lesion. This first international consensus classification may encourage neuropathologists to focus their attention onto this important histopathological group. Addressing more precisely defined clinico-pathological entities will also help to clarify underlying pathomechanisms and, thereby, improve treatment strategies for patients with difficult-to-treat epilepsies.     

Temporal lobe focal cortical dysplasia: MRI imaging using FLAIR shows lesions consistent with neoplasia

Focal cortical dysplasia (FCD), a form of neuronal migration disorder, is a malformative lesion of the neocortex that occurs during development of the brain. It can cause partial and generalized epilepsy. Seizures occur at an early age and are often resistant to medication. Surgical resection has been found to be beneficial in these patients. Dual pathology, in the form of mesial temporal sclerosis, has been associated with FCD. At the Children’s Hospital of Eastern Ontario, four patients with temporal lobe FCD have re-cently, been identified. This paper discusses how these children presented and how they were managed, with particular emphasis on their MRI findings and differential diagnoses. In three of the four patients neuroimaging studies showed lesions consistent with a neoplastic process because of the large volume and mass effect. Radiologically, FCD may mimic the MRI appearance of tumors, such as dysembryoplastic neuroepithelial tumors, primitive neuroectodermal tumors, gangliogliomas, oligodendrogliomas, and astrocytomas. These lesions are best visualized on fluid-attenuated inversion recovery (FLAIR) imaging, a technique that has recently become applicable in the clinical setting, as we help demonstrate in this series. With better MRI capability, milder forms of FCD and microdysplasia may be distinguished. Received: 7 September 1999