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Resumen La aparición de convulsiones es frecuente durante el periodo neonatal debido a las características de inma durez funcional del cerebro es este periodo. La aparición de estas convulsiones puede llevar a un diagnóstico de epilepsia neonatal, que suele estar asociado a alteracio nes estructurales del cerebro durante el neurodesarrollo. Aproximadamente el 50% de las personas con epilepsia activa padecen al menos un trastorno médico comórbi do, y esto hace que cambie la evolución de la epilepsia. La presencia de trastornos neurológicos que preceden a la aparición de la epilepsia indica que alteraciones es tructurales y/o funcionales del cerebro subyacentes pue den ser causa de la predisposición a padecer epilepsia y de los procesos comórbidos de manera independiente. En esta revisión describimos los procesos cerebrales estructurales y funcionales que subyacen a la aparición de epilepsia neonatal y sus comorbilidades.
Abstract The occurrence of seizures is frequent during the neonatal period due to the functional immaturity of the brain.The presence of these seizures may lead to a diagnosis of neonatal epilepsy, which is usually as sociated with structural alterations of the brain during neurodevelopment. Approximately 50% of people with active epilepsy have at least one comorbid medical di sorder, and the existence of a comorbid process changes the course of the epilepsy. The presence of neurologic disorders preceding the onset of epilepsy indicates that underlying neurobiological alterations may indepen dently cause the predisposition to epilepsy and comor bid processes. In this review we describe the structural and functional brain processes underlying the onset of neonatal epilepsy and its comorbidities.
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Objective:To summarize the clinical characteristics of patients with epilepsy caused by focal cortical dysplasia (FCD), and identify the influencing factors for postoperative seizure controls.Methods:Fifty-seven patients with epilepsy caused by FCD admitted to Department of Neurosurgery, Third Affiliated Hospital of Zhengzhou University from July 2019 to November 2023 were chosen; standard preoperative evaluation, surgery, postoperative management and follow-up were performed. A retrospective study of clinical data, imaging and video electroencephalogram (VEEG) data, surgical approaches, pathological findings, and follow-up data was performed; influencing factors for postoperative seizure controls were analyzed.Results:In these 57 patients with epilepsy caused by FCD, 29 were males (50.88%) and 28 were females (49.12%). Onset age was 30.00 (8.00, 74.50) months, and surgery age was 95.00 (50.00, 138.50) months. Focal to bilateral tonic-clonic seizures (42/57; 73.68%) and epileptic spasms (13/57; 22.81%) were common seizure types. Cranial MRI was positive in 34 patients (59.65%), mainly manifested as abnormal cortical gyri/sulci morphology (17/57; 29.82%). In 43 patients accepted PET-CT, hypometabolic sites were detected in 40 (93.02%), and complete agreement between PET/MRI fusion results and actual lesion sites was noted in 40 (93.02%). FCD type I was noted in 16 patients (28.07%), type II in 39 (68.42%), and type III in 2 (3.51%). By December 2023, 44 (77.19%) had Engel grading I, 4 (7.02%) had grading II, 4 (7.02%) had grading III, and 5 (8.77%) had grading IV. Children with good prognosis (Engel grading I+II) and those with poor prognosis (Engel grading III+IV) showed significant differences in terms of time from first seizure to surgery, positive/negative MRI, and regularity of postoperative ASMs ( P<0.05). Conclusions:Focal to bilateral tonic-clonic seizure is the most common seizure type in patients with epilepsy caused by FCD, and abnormal cortical gyri/sulci morphology is the most common MRI manifestation; PET/MRI fusion imaging is superior to PET-CT or MRI in identifying epileptogenic foci. Poor seizure control can be noted in patients with long onset time to surgery, with negative cranial MRI results, or with irregular postoperative ASMs.
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Objective:To investigate the diagnostic value and imaging characteristics of MRI combined with 18F-fluorodeoxyglucose (FDG) positron-emission tomography (PET)/CT in focal cortical dysplasia (FCD) complicated with refractory epilepsy. Methods:A retrospective analysis was performed on 42 patients with FCD complicated with refractory epilepsy who were admitted to the Affiliated Hospital of Jining Medical University from January 2017 to December 2022. All patients underwent preoperative MRI and 18F-FDG PET/CT, and PET/MRI fusion was performed on the images. Chi-square test and Kappa consistency test were used to compare the localization diagnostic efficacy of PET/CT, MRI and PET/MRI fusion for epileptic foci. The patients were categorized based on gender, lesion location, pathological type, seizure type, and efficacy. Independent sample t-test and analysis of variance were used to compare maximum standardized uptake (SUVmax) values and asymmetry index (AI) of the patients between different groups. Results:Among the 42 patients, the positive rates of MRI, PET/CT, PET/MRI fusion examinations were 85.7%(36/42), 95.2%(40/42), 100.0%(42/42), the lateral localization rates were 71.4%(30/42), 92.9%(39/42), 95.2%(40/42), and the localization rates were 57.1%(24/42), 81.0%(34/42), 88.1%(37/42), respectively. There were significant differences in the lateral localization rates and localization rates of epileptogenic foci between MRI and PET/CT (χ 2=6.574, P=0.010; χ 2=5.570, P=0.018). There were significant differences in the positive rates of lesions, the lateral localization rates and the localization rates of epileptogenic foci between MRI and PET/MRI fusion (χ 2=6.385, P=0.012; χ 2=8.571, P=0.003; χ 2=10.118, P=0.001). There were no significant differences in the positive rates of lesions between MRI and PET/CT, and in the positive rates of lesions, the lateral localization rates and localization rates of epileptogenic foci between PET/CT and PET/MRI fusion (χ 2=2.184, P=0.139; χ 2=2.024, P=0.155; χ 2=0.210, P=0.647; χ 2=0.819, P=0.365). The Kappa consistency test of PET/CT and PET/MRI fusion imaging was performed for the location of epileptogenic foci, and the Kappa=0.721 was obtained, indicating that they were consistent in the location of epileptogenic foci. The SUVmax values of patients with temporal lobe epilepsy were lower, and the AI values were higher than that of patients with extra temporal lobe epilepsy (7.4±1.3 vs 9.6±1.6, 15.5±2.6 vs 12.9±2.4; t=5.154, 6.083; P=0.001, 0.001). The SUVmax values of patients with good efficacy (according to the Engel efficacy grading system, grades Ⅰ-Ⅱ indicating good efficacy) were higher, and the AI values were lower than that of patients with poor efficacy (according to the Engel efficacy grading system, grades Ⅲ-Ⅳ indicating poor efficacy; 9.5±1.9 vs 7.9±2.1, 13.5±3.3 vs 14.8±3.0; t=2.789, 3.722; P=0.042, 0.029). There were no significant differences in SUVmax and AI values among different genders, pathological types and seizure types (all P>0.05). Conclusions:The imaging characteristics of patients with different types of FCD complicated with refractory epilepsy are different. PET/MRI fusion is better than MRI in the diagnosis of FCD complicated with refractory epilepsy, and is consistent with PET/CT in the location of epileptogenic foci.
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Resumen Las crisis atónicas focales son poco reconocidas como fenómenos ictales, pueden corresponder tanto a una epilepsia generalizada como a una epilepsia focal. Las áreas del cerebro implicadas en la gestión de este tipo de crisis son: el área motora negativa y las cortezas motora primaria y somatosensitiva primaria, aunque aún la neurofisiología que las genera no está aclarada. Presentamos el caso de un paciente con crisis atónicas focales farmacorresistentes en miembro superior iz quierdo. Se realizó resonancia de cerebro con diagnóstico de displasia cortical parietal, se monitoreó con video EEG de scalp y luego a video EEG con electrodos profundos. Se definieron el área epileptógena y su relación con áreas elocuentes, se realizó resección quirúrgica de la lesión, logrando el control completo de las crisis.
Abstract Focal atonic seizures are recognized rarely as ictal phenomena, they can correspond to both generalized epilepsy and focal epilepsy. The areas of the brain in volved in the management of this type of seizure are: the negative motor area and the primary motor and primary somatosensory cortices, although the neurophysiology that generates them is still unclear. We present the case of a patient with focal atonic seizures in the left upper limb, refractory to drug treatment. Neuroimaging was performed, a parietal cortical lesion was diagnosed. A scalp Video EEG and then a Stereo EEG was performed, defining the epileptogenic area and its relationship with eloquent areas. Surgical resection of the lesion was performed, achieving complete seizure control.
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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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Objective:To investigate the clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia (FCD) IIId in children.Methods:Forty-nine children with epilepsy accepted surgical treatment in our hospital from January 2008 to July 2017 were chosen in our study; these patients were pathologically diagnosed as having FCD IIId. These children were divided into 7 groups according to different brain injuries: cerebral hemorrhage ( n=13), central nervous system infectious disease ( n=19), cerebral infarction ( n=1), intrapartum hypoxia ( n=5), two kinds of early brain injury ( n=2), febrile seizure ( n=6), traumatic brain injury ( n=3). Two years after surgery, Engel grading was used to evaluate the surgical efficacy of the children. The clinical and pathological characteristics of these children were retrospectively analyzed. Results:The average age of brain injury was 0 d-3.8 years, the average age of epilepsy was 4-5.3 years, and the average age of surgery was 7-10.2 years. The incidence of multiple lobe brain injury in the cerebral hemorrhage group (100%, 13/13) was significantly higher than that in the central nervous system infectious diseases group (36.8%, 7/19,) and febrile seizure group (50%, 3/6, P<0.05). There was a significant difference in Engel grading I ratio between cerebral hemorrhage group (84.6%, 11/13) and central nervous system infectious disease group (42.1%, 8/19, P<0.05); Engel grading I and II was noted in 80.0% children (4/5) of the intrapartum hypoxia group. All children had pathological changes and pathological characteristics of FCD after early brain injury. Conclusion:FCD IIId children with cerebral hemorrhage or intrapartum hypoxia have good surgical effect, which may be related to the early occurrence of early brain damage, clear pathological changes, and clear boundary between the focus and the surrounding brain tissues.
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Objective:To investigate the clinical and pathological characteristics and surgical efficacy of early brain injury with focal cortical dysplasia (FCD) IIId in children.Methods:Forty-nine children with epilepsy accepted surgical treatment in our hospital from January 2008 to July 2017 were chosen in our study; these patients were pathologically diagnosed as having FCD IIId. These children were divided into 7 groups according to different brain injuries: cerebral hemorrhage ( n=13), central nervous system infectious disease ( n=19), cerebral infarction ( n=1), intrapartum hypoxia ( n=5), two kinds of early brain injury ( n=2), febrile seizure ( n=6), traumatic brain injury ( n=3). Two years after surgery, Engel grading was used to evaluate the surgical efficacy of the children. The clinical and pathological characteristics of these children were retrospectively analyzed. Results:The average age of brain injury was 0 d-3.8 years, the average age of epilepsy was 4-5.3 years, and the average age of surgery was 7-10.2 years. The incidence of multiple lobe brain injury in the cerebral hemorrhage group (100%, 13/13) was significantly higher than that in the central nervous system infectious diseases group (36.8%, 7/19,) and febrile seizure group (50%, 3/6, P<0.05). There was a significant difference in Engel grading I ratio between cerebral hemorrhage group (84.6%, 11/13) and central nervous system infectious disease group (42.1%, 8/19, P<0.05); Engel grading I and II was noted in 80.0% children (4/5) of the intrapartum hypoxia group. All children had pathological changes and pathological characteristics of FCD after early brain injury. Conclusion:FCD IIId children with cerebral hemorrhage or intrapartum hypoxia have good surgical effect, which may be related to the early occurrence of early brain damage, clear pathological changes, and clear boundary between the focus and the surrounding brain tissues.
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Objective:To explore the surgical outcomes of focal cortical dysplasia (FCD) patients with "difficult to locate" intractable epilepsy and their influencing factors.Methods:Thirty-five FCD patients with "difficult to locate" intractable epilepsy, underwent surgical treatment after intracranial electroencephalogram (iEEG) evaluation in our hospital from January 2011 to December 2018, were chosen in our study. Engel grading was used to evaluate the surgical efficacies of these patients, and they were divided into a satisfied efficacy group (Engel grading I) and an incomplete satisfied efficacy group (Engel grading II-IV). The clinical data of patients from the 2 groups were compared. Multivariate Logistic regression analysis was performed to explore the influencing factors for surgical outcomes of FCD patients with "difficult to locate" intractable epilepsy.Results:Of these 35 patients, 26 patients (74.3%) achieved satisfied efficacy, and 4 had incomplete satisfied efficacy. As compared with those in the satisfied efficacy group, patients in the incomplete satisfied efficacy group had significantly lower total resection rate of epileptogenic foci ( P<0.05). Multivariate Logistic regression analysis showed that incomplete resection of epileptogenic foci was the influencing factor for surgical outcomes of FCD patients with "difficult to locate" intractable epilepsy ( P=0.014, OR=0.050, 95%CI: 0.005-0.547). Conclusion:The FCD patients with "difficult to locate" intractable epilepsy can achieve satisfactory results by surgical resection of epileptogenic zones after iEEG monitoring; these FCD patients with "difficult to locate" intractable epilepsy with incomplete resection of epileptogenic foci often have poor surgical outcomes.
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Objective:To explore the value of triple fusion positron emission tomography (PET)/MRI in location of epileptogenic focus in patients with focal cortical dysplasia (FCD).Methods:Three patients with refractory partial epilepsy, admitted to our hospital from December 2016 to June 2017, were chosen in our study. The raw MRI and PET images of these patients were processed using Freesurfer and FSL image processing softwares. After extraction and coregistration, precise PET/MRI fusion images were obtained; and the grey-white matter dividing line was highlighted on this fusion image to form triple-fusion images to observe the hypometabolic area and clarify the location.Results:Triple-fusion images of these 3 patients were acquired. In patient 1, a marked decrease in metabolism was noted in the gyrus region delineated by the gray-white matter boundary in the right cingulate gyrus. In patient 2, the area with slightly increased local signal in the right superior frontal gyrus (MRI FLAIR sequence) was the area with reduced metabolism. In patient 3, an area of local decreased metabolism was noted in the right cingulate gyrus. The preoperative evaluation of all 3 patients showed that the above areas were epileptic foci; the patients were followed up for 2 years after surgical resection, no clinical seizures occurred in all patients, and antiepileptic drugs were gradually stopped. All 3 patients were diagnosed as having FCD by postoperative pathology.Conclusion:Triple fusion PET/MRI is a powerful way to assist FCD diagnosis, especially for those FCD cases which are difficult to be diagnosed by other imaging methods, and has a potential clinical application value in epilepsy patients.
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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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@#Objective To explore the value of high-resolution MRI in in the preoperative evaluation of children with type Ⅰ focal cortical dysplasia (FCD). Methods The MRI and related clinical data of 52 children with FCD Ⅰ confirmed by pathology were retrospectively analyzed,Compare the detection rates of major MRI signs of type Ⅰ FCD (focal demarcation of gray and white matter,focal cortical structure abnormalities,abnormal white matter signal lesions,and local atrophy / hypoplasia) of high-resolution imaging and conventional MRI sequences,and comparisons in the assessment of lesion extent,borders,and completeness were perfomed. Results The detection rate of high-resolution imaging for blurring of gray-white matter boundaries,focal cortical structural abnormalities,and abnormal white matter abnormalities was higher than that of conventional sequences (P<0.05),there was no significant difference in the detection rate of local atrophy/dysplasia (P>0.05). 3D-T1WI showed the range and boundary of the lesion better than the conventional T1WI sequence,3D-T2FLAIR was more complete than the conventional T2FLAIR sequence. Conclusion The high-resolution MRI could improve the detection rate of various major signs of type Ⅰ FCD,could more fully display the lesion and accurately assess the scope and boundary of the lesion,and was the preferred MRI imaging sequence before surgery.
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@#Objective The molecular mechanisms of the pathogenesis underlying focal cortical dysplasias (FCD) remain unclear.The aims of this study is to find out the potential gene markers and drugs for FCD.Methods We analyzed the GSE62019 datasets,including tissue from five FCD patients and three controls,to identify differentially expressed genes.Afterwards,the gene ontology and signaling pathway enrichment analyses of these DEGs were performed using online software.Protein and protein interaction networks were constructed and the significant gene modules were chosen for further gene-drug interaction analysis.Furthermore,the existing drugs target to these module genes were screen to explore the therapeutic effect for FCD.Results We identified 777 DEGs,including 364 down-regulated genes and 413 up-regulated genes,respectively.One core module of DEGs was selected.Moreover,the significant module genes in PPI networks were C3,SAA1,ANXA1,CXCL2 and CCL25,and several existing drugs have targeted to those genes.Conclusion We identified 5 potential genes and several existing drugs for FCD,which might be used as targets for the study of FCD.
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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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Humanos , Clasificación , Consenso , Embriología , Epilepsia , Malformaciones del Desarrollo Cortical , PatologíaRESUMEN
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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Adolescente , Niño , Humanos , Anticonvulsivantes , Electroencefalografía , Epilepsia , Estudios de Seguimiento , Libertad , Malformaciones del Desarrollo Cortical , Análisis Multivariante , Recurrencia , Estudios Retrospectivos , ConvulsionesRESUMEN
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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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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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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Humanos , Clasificación , Consenso , Embriología , Epilepsia , Malformaciones del Desarrollo Cortical , PatologíaRESUMEN
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.