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Objective:To analyze the fetal ultrasonographic features of malformation of cortical development (MCD) during the second trimester, and explore and summarize the relevant diagnostic clues, so as to improve the ability of diagnosis and differential diagnosis of fetal MCD.Methods:A total of 313 fetuses with brain abnormalities suspected on ultrasound in Chengdu Women′s and Children′s Central Hospital from April 2018 to August 2022 were retrospectively analyzed. The fetuses were examined using MRI. The ultrasonographic characteristics of fetal MCD were summarized, and the fetal ultrasound and MRI imaging data were compared for fetal MCD.Results:Nineteen fetuses were diagnosed with MCD from 313 fetuses(6.07%, 19/313). Seventeen cases of MCD were identified by ultrasonography and subsequently validated by fetal MRI, including 6 cases of schizencephaly, 2 cases of hemimegalencephaly(HMEG), 3 cases of periventricular nodular heterotopia(PVNH), 3 cases of lissencephaly, 2 cases of microcephaly and 1 case of polymicrogyria(PMG). There were 3 cases with two concurrent MCD, 1 case of HMEG, and MRI increased the diagnosis of left parietal PMG; 1 case of lissencephaly, and MRI increased the diagnosis of PVNH. The other case was PMG, and MRI increased the diagnosis of lissencephaly. Two cases of fetal MCD were not indicated by ultrasonography, one of which was diagnosed as tuberous sclerosis and another one as schizencephaly by MRI, both due to ventriculomegaly.Conclusions:Various types of MCD in the second trimester have ultrasonographic characteristics. Abnormal lateral ventricles, intracranial structural changes such as sulci and gyrus can provide reliable ultrasound diagnostic clues for fetal MCD.
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ABSTRACT Focal Cortical Dysplasia (FCD) is a group of focal developmental malformations of the cerebral cortex cytoarchitecture. FCD usually manifests as medically intractable epilepsy, especially in young children. Live patients are diagnosed by radiological examination such as magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG PET), magnetoencephalography (MEG), diffusion-tensor imaging (DTI), and intracranial electroencephalogram (EEG). While some cases can be missed by radiological examination, they are usually diagnosed on the histopathological examination of the surgically removed specimens of medically intractable epilepsy patients. We report a case of a young girl with cerebral palsy, mental retardation, and seizure disorder who died in her sleep. The deceased was diagnosed with FCD type III with hippocampal sclerosis on histopathological examination at autopsy. H & E stain and NeuN immunohistochemistry neuronal cell marker were used to demonstrate the findings of FCD.
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Focal cortical dysplasias (FCDs) represent the third most frequent cause of drug-resistant focal epilepsy in adults (after hippocampal sclerosis and tumours) submitted to surgery, and the most common in the pediatric age group. The International League Against Epilepsy (ILAE) classification of focal cortical dysplasia is still a reference and consists of a three-tiered system: FCD type I refers to isolated abnormalities in cortical layering; FCD type II refers to cases with abnormalities in cortical architecture and dysmorphic neurons with or without balloon cells; and FCD type III refers to abnormalities in cortical layering associated with other lesions. Recent studies have demonstrated that somatic mutations occurring post-zygotically during embryonal development and leading to mosaicism, underlie most brain malformations. The molecular pathogenesis of FCD type II is associated with activation of the mTOR pathway. Pathogenic variants in this pathway are recognized in up to 63% of cases and may occur both through single activating variants in activators of the mTOR signaling pathway or double-hit inactivating variants in repressors of the signaling pathway. The newly described mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy, has been found to show recurrent pathogenic variants in SLC35A2 with mosaicism. The present review describes the lesions of FCD and discusses the molecular pathogenesis and proposal for a revised classification.
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@#Introduction: Epilepsy is the common condition encountered in both adults and pediatric population. It occurs as a result of various spectrum of etiology ranging from infections to tumors. EEG and Neurosonogram can characterize the type of epilepsy; however, imaging is the only tool to identify the lesion, its location, and extent and resection possibility. CT was the only modality before the era of MRI. However, CT was only used to identify the lesion with hemorrhage and calcification. It is having the disadvantage of having poor spatial resolution and using radiation. The era of MRI has changed the imaging due to its higher spatial resolution, gray white matter differentiation, status of myelination and non-utilization of radiation. Purpose: The aim of study was to detect and characterize various lesions causing epilepsy in pediatric age group (0-12 years) and also to detect frequency with which they occurred using MRI. Methods: The study was performed on 50 children under the age of 12 years over a period of 1 year who presented with epilepsy. Patients with trauma and febrile seizure disorders were excluded. Conventional and contrast MRI was performed in all cases and lesions were characterized in location, signal intensity, and other features. Results: The mean age group of the study population was 1-5 years. Generalized seizures constituted the major seizure group. Our study shows infection as the most common etiology followed by mesial temporal sclerosis and Focal cortical dysplasia. It was followed by neoplastic etiology, phacomatosis and demyelinating diseases. Conclusion: MRI is the imaging modality of choice in the evaluation of pediatric patients presenting with epilepsy. Proper MRI seizure protocol helps to establish the correct diagnosis, plan the management according to diagnosis as well as helps in prognosis.
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In 2011, the International League against Epilepsy (ILAE) proposed a first international consensus of the classification of focal cortical dysplasia (FCD). This FCD classification had been widely used in worldwide. In this review paper, the authors would like to give helpful comments for better understanding of the current FCD classification. Especially, the basic concepts of FCD type I, such as “radial”, “tangential” and “microcolumn” will be discussed with figures. In addition, the limitations, genetic progress and prospect of FCD will be suggested.
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Humans , Classification , Consensus , Embryology , Epilepsy , Malformations of Cortical Development , PathologyABSTRACT
Objective: To propose a multimodal epilepsy assessment system to guide the surgical treatment of patients with focal cortical dysplasia (FCD)-related epilepsy, and to explore its significance in improving the prognosis of patients with FCD-related epilepsy. Methods: Twenty-two epilepsy patients with FCD confirmed by postoperative pathology, who underwent surgical treatment at Second Hospital of Lanzhou University from Oct. 2016 to Nov. 2018, were enrolled. All patients were evaluated by multimodal epilepsy assessment system during the perioperative period. The assessment techniques included onset symptom assessment, structural imaging, long-term scalp video electroencephalogram monitoring, positron emission tomography-computed tomography, image fusion analysis, and magnetic resonance imaging (MRI). Engel efficacy grading system was used to assess the prognosis. Engel I grade was defined as good control for epilepsy and Engel II to IV as poor control. Fisher exact test was used to explore the influences of gender, age, pathological type, MRI examination, lesion location, duration of disease, and electroencephalogram-clinical characteristic-anatomical localization consistency on the prognosis of patients. Results: Twenty-two patients (13 males and 9 females) underwent surgical resection of the lesions, and were followed up for 4 to 28 months. There were 18 cases of Engel I grade, 2 cases of Engel II grade, 1 case of Engel III grade, and 1 case of Engel IV grade, with the good control rate being 81.8% (18/22). Fisher exact test analysis showed that disease duration and electroencephalogram-clinical characteristic-anatomical localization consistency were independent factors of efficacy of FCD-related epilepsy (P = 0.045 and 0.005). While age, lesion location, gender, pathological type and MRI findings had no significant effect on prognosis (all P < 0.05). Conclusion: The multimodal epilepsy assessment system can more accurately locate epileptogenic foci of patients with FCD-related epilepsy, providing a theoretical basis for epilepsy surgery. It is a comprehensive and accurate assessment method for epileptogenic foci.
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In 2011, the International League against Epilepsy (ILAE) proposed a first international consensus of the classification of focal cortical dysplasia (FCD). This FCD classification had been widely used in worldwide. In this review paper, the authors would like to give helpful comments for better understanding of the current FCD classification. Especially, the basic concepts of FCD type I, such as “radial”, “tangential” and “microcolumn” will be discussed with figures. In addition, the limitations, genetic progress and prospect of FCD will be suggested.
Subject(s)
Humans , Classification , Consensus , Embryology , Epilepsy , Malformations of Cortical Development , PathologyABSTRACT
Objective To investigate the clinical pathologic features of a distinct variant of focal cortical dysplasia (FCD) characterized by neuronal loss of layer four.Methods Between 2005 and 2017,approximately 3 000 surgeries were performed for the treatment of intractable epilepsy at Xuanwu Hospital,Capital Medical University and Yuquan Hospital,Tsinghua University.Retrospective analysis of clinic-pathological data of patients with epilepsy surgery was made and histological manifestations of neuronal loss of cortical layer four were included in this study.Results In this cohort,25 patients (22 males and three females) were identified with early onset pharmaco-resistant epilepsy and regionally circumscribed neuronal loss of cortical layer four in surgical specimens from the occipital lobe.Histologically,except for neuronal loss in cortical layer four in all cases,glial scar lesions were found in some patients.Thus the histology of those cases can be subdivided into two groups:group A (13 cases):neuronal loss of cortical layer four without glial scar lesions;and group B (12 cases):neuronal loss of cortical layer four with glial scar lesions.Due to the prominent horizontal disorganization of cortical layering and lack of any other microscopically visible principle lesion,group A should be classified hitherto as FCD International League Against Epilepsy (ILAE) type Ⅰ b,however,group B with scar lesions and cortical dysplasia around the main leision,should be classified as FCD ILAE type Ⅲd.This retrospective analysis of clinical histories revealed a perinatal distress in 20 patients (80％),suggesting an acquired pathomechanism.Magnetic resonance imaging revealed abnormal signals in the occipital lobe in all patients,and signal changes suggestive of encephalomalacia were found in 18 patients.Surgical treatment achieved favorable seizure control (Engel class Ⅰ and Ⅱ) in 18 patients (75％ among 24 available follow up).Comparion of the two groups with age at epilepsy onset (group A:5.00±2.76,group B:5.01±3.78),the proportion of perinatal distress (group A:11/13,group B:9/12) and the follow-up results (favorable seizure control of the two groups was 9/13,9/11 respectively) showed that there was no statistically significant difference between the two groups.Conculsion Neuronal loss of cortical layer four in the occipital lobe should be classified as a distinct variant of FCD ILAE type Ⅲd.
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BACKGROUND AND PURPOSE: This study investigated the seizure recurrence rate and potential predictors of seizure recurrence following antiepileptic drug (AED) withdrawal after resective epilepsy surgery in children with focal cortical dysplasia (FCD). METHODS: We retrospectively analyzed the records of 70 children and adolescents with FCD types I, II, and IIIa who underwent resective epilepsy surgery between 2004 and 2015 and were followed for at least 2 years after surgery. RESULTS: We attempted AED withdrawal in 40 patients. The median time of starting the AED reduction was 10.8 months after surgery. Of these 40 patients, 14 patients (35%) experienced seizure recurrence during AED reduction or after AED withdrawal. Half of the 14 patients who experienced recurrence regained seizure freedom after AED reintroduction and optimization. Compared with their preoperative status, the AED dose or number was decreased in 57.1% of patients, and remained unchanged in 14.3% after surgery. A multivariate analysis found that incomplete resection (p=0.004) and epileptic discharges on the postoperative EEG (p=0.025) were important predictors of seizure recurrence after AED withdrawal. Over the mean follow-up duration of 4.5 years after surgery, 34 patients (48.6% of the entire cohort) were seizure-free with and without AEDs. CONCLUSIONS: Children with incomplete resection and epileptic discharges on postoperative EEG are at a high risk of seizure recurrence after drug withdrawal. Complete resection of FCD may lead to a favorable surgical outcome and successful AED withdrawal after surgery.
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Adolescent , Child , Humans , Anticonvulsants , Electroencephalography , Epilepsy , Follow-Up Studies , Freedom , Malformations of Cortical Development , Multivariate Analysis , Recurrence , Retrospective Studies , SeizuresABSTRACT
Objective To investigate the value of magnetoencephalograph(MEG)combined neuronavigation in the operation of frontal and temporal epilepsy caused by focal cortical dysplasia(FCD). Methods The data of 44 cases of frontal and temporal epilepsy caused by FCD were analyzed retrospectively. The location of epileptogenic zone and assessment IQ ,memory and language of patients were tested before operation;MEG examination confirmed the language dominance hemisphere and clarified the scope of language function. The surgical navigation system was guided by the American medtronic steal health 7 surgical navigation system to protect the neurological function. IQ,memory and language examination were measured 1 year after operation,and the data were analyzed before and after operation. The patients were followed up for 13~ 44 months after operation,according to Engel′s classification standard,the curative effect of epilepsy was determined. Results MEG can accurately localize the location of the language functional areas and FCD Epileptogenic zone. Of the 44 language functional areas ,28 had the left hemisphere and 16 were on the right side. Statistics showed that the verbal IQ and total IQ increased(P<0.05)in 1 year after operation,and there was no significant change in memory quotient and operation IQ(P >0.05). FCD patients recovered well ,and no language and limb function damaged. The curative effect of epilepsy:18 cases of Engel′sⅠgrade,22 cases of Engel′sⅡgrade;4 cases of Engel′sⅢgrade. Conclusions MEG combined with neuronavigation plays an important role in the localization ,localization and guidance of epileptogenic zone in patients with refractory frontal and temporal epilepsy caused by FCD ,which can protect the cortex function,avoid serious postoperative complications,and improve the therapeutic effect of epilepsy.
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Purpose To study the clinicopathologic features of ganglioglioma. Methods The clinicopathologic data of the cases pathologically diagnosed as ganglioglioma that underwent resection of epileptic focus were retrospectively analyzed. Results In the 19 cases studied, the mean onset age was 9.1 years, and the duration of disease was 9.3 years. MRI images showed abnormal signals. The majority of the site was temporal lobe (14/19, 73.7％). The tumors showed heterogeneity and often accompanied by focal cortical dysplasias (13/19, 68.4％). Immunohistochemical staining showed CD34 positive in 18 cases, Nestin positive in 16 cases, and BRAF-V600E positive in 6 case. The positive expression rate of CD34 and Nestin did not have significant differences. Conclusion The diagnosis of ganglioglioma relies on pathological observations combined with clinical features and neuroradiological examinations. Differential diagnosis should be done from other tumors or cortical dysplasia. Immunohistochemical staining of CD34 and Nestin can help diagnosis.
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PURPOSE: Antiepileptic drugs (AEDs) can be discontinued in a subset of patients after surgery. We aimed to identify the factors related to successful AED withdrawal after surgery in pediatric patients with focal cortical dysplasia (FCD). METHODS: The study included 134 patients who underwent resective surgery for FCD at Severance Hospital between 2003 and 2014. Age of seizure onset, epilepsy duration, and location and histopathological classification of the FCD were compared between patients who experienced seizure recurrence and those who did not. The interval between surgery and initiation of AED reduction was also compared. RESULTS: In total, 134 patients were included. The median age at seizure onset was 1.0 year (interquartile range [IQR], 0.3–5.0). The median follow-up duration was 6.0 years (IQR, 1.0–13.0). AED withdrawal was attempted in 89 (66%), and 61 (69%) patients remained seizure-free. Of 61 patients, 38 (62%) were successfully weaned off all AEDs. Seizures recurred in 28 (31%) patients. The mean duration between surgery and initiation of AED reduction did not significantly differ between the seizure recurrence (4.5 months, IQR, 2.7–8.7) and non-recurrence groups (1.9 months, IQR, 0.5–5.4) (P<0.006). Patients who had FCD type IIb (39% vs. 7%, P=0.004) were more likely to be in the non-recurrence group than in the recurrence group (P=0.031). CONCLUSION: Surgical resection offers patients with FCD an opportunity to completely discontinue their AEDs. Early AED discontinuation may be pursued in patients with FCD in cases of complete resection.
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Humans , Anticonvulsants , Classification , Epilepsy , Follow-Up Studies , Malformations of Cortical Development , Recurrence , SeizuresABSTRACT
Objetivo: La displasia cortical focal (DCF) es una anomalía del desarrollo cortical. Representa una de las causas más frecuentes de epilepsia refractaria, siendo fundamental la resonancia magnética (RM) para su diagnóstico. Dada la importancia que ha cobrado la secuencia de tensor de difusión (DTI), el objetivo de este trabajo fue evaluar retrospectivamente los hallazgos en el mapa de fracción de anisotropía del DTI en pacientes con DCF. Observaciones: Se buscó retrospectivamente a pacientes con diagnóstico anatomopatológico de DCF en la base de datos de nuestro hospital. De un total de 74, se seleccionaron 8 casos con diagnóstico aislado de DCF y estudio de RM prequirúrgico con DTI. El foco de displasia se identificó en las imágenes anatómicas convencionales de la RM. Se evaluó el mapa de fracción de anisotropía (FA) y se definieron las alteraciones en la región de la DCF. Se observó una disminución de la FA en la sustancia blanca adyacente a la DCF en 7 de los 8 pacientes (87,5%). Discusión: Los hallazgos con el DTI brindan información complementaria en relación con la RM. En la mayoría de nuestros pacientes no hubo cambios de señal en la sustancia blanca en las imágenes convencionales, pero sí se identificó una disminución de la FAen el DTI. Se desconoce si esto refleja cambios estructurales o únicamente funcionales, secundarios a la lesión primaria. Creemos que el DTI puede agregar información complementaria de valor para el diagnóstico y valoración de la extensión de esta patología.
Objective: Focal cortical dysplasia (DCF) in an anomaly of cortical development. It represents one of the most frequent causes of drug resistant epilepsy and the magnetic resonance imaging (MRI) is trivial for its diagnosis. In the last years the use of diffusion tensor imaging (DTI) has increased in this kind of pathology. The purpose of this work was to evalúate retrospectively DTI findings on fractional anisotropy maps in patients with FCD. Observations: We retrospectively searched patients with confirmed anatomo-pathological diagnosis of FCD in our hospital datábase. From a total of 74, 8 patients, with isolated diagnosis of FCD and preoperative MRI with DTI, were selected. The FCD was identified in conventional anatomical MRI in all patients. Fractional anisotropy (FA) maps were evaluated and changes in the región of FCD were defined. Decreased FA was observed in white matter adjacent to the FCD, in 7 of 8 patients (87.5%). Discussion: Findings of DTI gives us complementary information to those of conventional MRI. Most of our patients showed no signal changes of white matter in conventional sequences and they presented decreased FA in DTI. We don't actually know if these DTI findings represent structural changes in white matter or just functional changes secondary to the adjacent FCD. We think DTI can give valuable complementary information for the diagnosis and determination of the extensión of this pathology.
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Objective To investigate the potential pathogenesis of Focal cortical dysplasia (FCD), we performed cDNA microarray analysis to obtain gene expression profile of FCD. Methods Three FCD samples and three normal controls were enrolled. Total RNA of the brain tissues were extracted. The difference gene expressions between FCD group and control group was detected using Affymetrix gene chip. The up and down-regulated genes were confirmed by Real-time PCR. Further, the related signal pathways involved in the pathogenic mechanisms of FCD were predicted by bioinformatics. Result In FCD, two up-regulated genes C21orF2 and AU152162 and 5 down-regulated genes ENPP2, ANLN, IP6K3, UGT8, and AZGP were found. Compared the FCD samples with the normal controls , there were significantly different in all down-regulated genes (P 0.05). Using bioinformatics analysis, the ENPP2 , UGT8 , and AZGP1 protein which located in the cell membrane or secreted into the extracellular matrix may be involved in the formation of the myelin sheath and the development of the nervous system by the lipid metabolism and LPA signaling pathway. Conclusion ENPP2, UGT8 and AZGP1 may be involved in pathogenesis of FCD through the process of myelin sheath formation and LPA signal pathway , which warrants further study to know their roles in the pathogenesis of FCD.
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Objective To explore the correlations of the MRI findings and its pathological typing in the focal cortical dysplasia (FCD) .Methods MR images of 74 patients with FCD confirmed by operation and histopathologic examination were analysed retro‐spectively .MRI findings with FCD were divided into three subtypes including radial band type ,hyperintensity type and mild type . The correlation of the FCD MRI findings and pathological typing is analysed .Results In 74 patients with FCD ,there were radial band type in 12 cases ,hyperintensity type in 32 cases ,and mild type in 30 cases respectively .M RI finding of radial band type FCD showed a tail of increased T2WI/FLAIR signal tapering down to the lateral ventricle .Hyperintensity type FCD showed increased T2 WI/FLAIR signal in the cortex and subcortical white matter ,accompanied with focal cortical thickening .Mild type FCD showed T2 WI/FLAIR subtle hyperintense signal in cortex with or without focal cortical thickening ,but there was no hyperintense signal in subcortical white matter .Most of radial band type FCD were ⅡB in pathology .Most of hyperintensity FCD were ⅡA and ⅡB .Mild type FCD was more found to beⅠA orⅠB .Conclusion Analysing MRI features would improve the accurate diagnosis of FCD and help to infer the pathological type .
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Objetivo: Evaluar la detección de displasias corticales utilizando un método semiautomático de cuantificación morfométrica basado en superficie mediante la localización de zonas con espesor cortical anormal. Materiales y métodos: Se seleccionó un grupo de pacientes remitidos por diagnóstico de epilepsia refractaria para la detección de lesiones cerebrales. El espesor cortical se midió utilizando algoritmos automáticos de morfometría basado en superficie de imágenes de resonancia magnética en cada uno de los pacientes, los cuales fueron comparados con un grupo control de sujetos sanos pareados por edad. Resultados: Se realizó la cuantificación de espesor cortical en 4 de los 5 pacientes seleccionados. Se encontraron áreas de engrosamiento cortical en las zonas de displasia cortical conocidas que se relacionaron con las zonas detectadas previamente por el radiólogo en la secuencia FLAIR de cada paciente. Se hallaron diferencias en los mapas de espesor cortical de cada paciente respecto al grupo control. Conclusión: La cuantificación morfométrica de espesor cortical es una técnica que promete ser de utilidad como asistencia computarizada al diagnóstico de las displasias corticales.
Objective: To evaluate the detection of cortical dysplasias using a semi-automatic surface-based morphometric quantification by locating areas with abnormal cortical thickness. Materials and methods: A group selected of patients referred for refractory epilepsy diagnosis to detect brain injury. The cortical thickness was measured using automatic algorithms based morphometry surface of magnetic resonance imaging in each of the patients, which were compared with a control group of age-matched healthy subjects. Results: The quantification was performed cortical thickness in 4 of the 5 patients selected. We found areas of cortical thickening in areas of cortical dysplasia known and related to areas previously identified by the radiologist FLAIR sequence in each patient. We found differences in cortical thickness maps of each patient in the control group. Conclusion: morphometric quantification of cortical thickness is a technique that promises to be useful as diagnostic support to scan cortical dysplasias.
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ObjectivesTo explore the neuropathological characteristics of children with temporal intractable epilepsy. MethodsA retrospective analysis of the pathological characteristics was conducted in 38 children who underwent epilepsy surgery from December 2011 to December 2013.ResultsAmong 38 patients, aged 2.5-14 years, 21 boys and 17 girls, 21 cases had focal cortical dysplasia (FCD), 1 case had FCDⅠa, 2 cases had FCDⅠb, 1 case had FCDⅡa, 3 cases had FCDⅡb, 5 cases had CDⅢa, 1 case had FCDⅢb, 1 case had FCDⅢc, 6 cases had FCDⅢd. Three cases had microdysgenesis, 3 cases had simple hippocampal sclerosis, 7 cases had neoplasms, 5 cases had dysembryoplastic neuroepithelial tumor, 2 cases had astroglioma, 2 cases had encephalitis, 2 cases had arachnoid cyst. ConclusionsFCD is the most common form that causes temporal lobe intractable ep-ilepsy in children. FCDIIId is the most common subtype in FCD.
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OBJECTIVE: Cortical dysplasia (CD) is one of the common causes of epilepsy surgery. However, surgical outcome still remains poor, especially with frontal lobe epilepsy (FLE), despite the advancement of neuroimaging techniques and expansion of surgical indications. The aim of this study was to focus on surgical strategies in terms of extent of resection to improve surgical outcome in the cases of FLE with CD. METHODS: A total of 11 patients of FLE were selected among 67 patients who were proven pathologically as CD, out of a total of 726 epilepsy surgery series since 1992. This study categorized surgical groups into three according to the extent of resection : 1) focal corticectomy, 2) regional corticectomy, and 3) partial functional lobectomy, based on the preoperative evaluation, in particular, ictal scalp EEG onset and/or intracranial recordings, and the lesions in high-resolution MRI. Surgical outcome was assessed following Engel's classification system. RESULTS: Focal corticectomy was performed in 5 patients and regional corticectomy in another set of 5 patients. Only 1 patient underwent partial functional lobectomy. Types I and II CD were detected with the same frequency (45.45% each) and postoperative outcome was fully satisfactory (91%). CONCLUSION: The strategy of epilepsy surgery is to focus on the different characteristics of each individual, considering the extent of real resection, which is based on the focal ictal onset consistent with neuroimaging, especially in the practical point of view of neurosurgery.
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Humans , Classification , Electroencephalography , Epilepsy , Epilepsy, Frontal Lobe , Magnetic Resonance Imaging , Malformations of Cortical Development , Neuroimaging , Neurosurgery , ScalpABSTRACT
Objective To screen the differential proteins in the brain (neocortex and hippocampus) between the rats with cortical dysplasia (CD) and control ones,and investigate the role of their alteration in the development of epilepsy in CD.Methods Cortical dysplasia was induced in rat pups via in utero delivery of BCNU.A two-dimensional electrophoresis (2-DE)-based approach was used to construct the expression profiles of proteins in both the neocortex and hippocampus at different age groups (postnatal day 7 and 60) and to detect proteome changes between CD rats and control ones.Following gel image analysis,protein spots that differed in abundance between CD and control rats were identified by using Matrx-assisted laser desorption/ionization (MALDI) mass spectrometry (MS) and MS/MS.Results A total of 57 kinds of protein were screened out (P ＜ 0.05),in which 35 were found up-regulated and 22 were down-regulated compared with the control,35 from neonatal stage (postnatal day 7) and others from adult stage.Finally,12 among them were identified,including tubulin,alpha-lB,Beta-actin,tubulin beta-2A,GAP-43,UbCKmit,GAPDH and TMBr-3,etc.Conclusions Changed expression of specific proteins which were found in our study are involved in construction of brain 's cytoskeleton,synaptic function,mitochondrial function and so forth.Thus,they may be related to the pathogenic mechanisms of epileptogenicity of CD.
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OBJECTIVE: The aim of this study was to devise an objective clustering method for magnetoencephalography (MEG) interictal spike sources, and to identify the prognostic value of the new clustering method in adult epilepsy patients with cortical dysplasia (CD). METHODS: We retrospectively analyzed 25 adult patients with histologically proven CD, who underwent MEG examination and surgical resection for intractable epilepsy. The mean postoperative follow-up period was 3.1 years. A hierarchical clustering method was adopted for MEG interictal spike source clustering. Clustered sources were then tested for their prognostic value toward surgical outcome. RESULTS: Postoperative seizure outcome was Engel class I in 6 (24%), class II in 3 (12%), class III in 12 (48%), and class IV in 4 (16%) patients. With respect to MEG spike clustering, 12 of 25 (48%) patients showed 1 cluster, 2 (8%) showed 2 or more clusters within the same lobe, 10 (40%) showed 2 or more clusters in a different lobe, and 1 (4%) patient had only scattered spikes with no clustering. Patients who showed focal clustering achieved better surgical outcome than distributed cases (p=0.017). CONCLUSION: This is the first study that introduces an objective method to classify the distribution of MEG interictal spike sources. By using a hierarchical clustering method, we found that the presence of focal clustered spikes predicts a better postoperative outcome in epilepsy patients with CD.