Focal Cortical Dysplasia

Abnormal, non-neoplastic cell proliferation of the CEREBRAL CORTEX confined to an area in any lobe. Focal cortical dysplasia in the temporal lobe is associated with TEMPORAL LOBE EPILEPSY.

Also Known As:

Cortical Dysplasia, Focal; Dysplasia, Focal Cortical; Dysplasia, Taylor; FCD, Taylor's; Focal Cortical Dysplasias; Taylor FCD; Taylors FCD; Taylor Dysplasia; Taylor Focal Cortical Dysplasia; Taylor's FCD

Networked: 291 relevant articles (5 outcomes, 28 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Seizures (Absence Seizure)
2.	Drug Resistant Epilepsy
3.	Neuroepithelial Neoplasms (Gliomatosis Cerebri)
4.	Hamartoma
5.	Epilepsy (Aura)

Experts

1.	Aronica, Eleonora: 31 articles (06/2022 - 06/2003)
2.	Yang, Hui: 16 articles (01/2022 - 08/2010)
3.	Mühlebner, Angelika: 15 articles (06/2022 - 02/2012)
4.	van Rijen, Peter C: 13 articles (10/2021 - 06/2003)
5.	Spliet, Wim G M: 10 articles (12/2019 - 11/2004)
6.	Blümcke, Ingmar: 9 articles (03/2022 - 10/2004)
7.	Vinters, Harry V: 9 articles (01/2021 - 10/2004)
8.	Crino, Peter B: 8 articles (01/2022 - 03/2002)
9.	Feucht, Martha: 8 articles (01/2022 - 02/2012)
10.	Jansen, Floor E: 8 articles (12/2021 - 06/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Focal Cortical Dysplasia:

1.	2,6-dinitrotoluene (DNT)IBA 01/01/2017 - "The DNT lesions are often related with focal cortical dysplasia (FCD) type IIIb, which is postulated to play a role in epileptogenicity. " 02/01/2010 - "One patient had dysembryoplastic neuroepithelial tumor (DNT) and three patients had focal cortical dysplasia (FCD). " 01/01/2001 - "The purpose of this study was to clarify and compare the influence of surgical strategy on relief from seizures in patients with focal cortical dysplasia (FCD) and those with dysembryoplastic neuroepithelial tumor (DNT). " 03/01/2014 - "We retrospectively investigated 88 patients (40 female, 48 male, mean age 37.2 yrs, from 12 to 69 yrs, 41 left sided lesions) with LTLE operated at our department, including 40.9% gangliogliomas (GG), 26.1% cavernomas (CM), 10.2% dysembryoplastic neuroepithelial tumours (DNT) and 11.4% focal cortical dysplasias (FCD), excluding hippocampal sclerosis. " 10/01/2006 - "The spectrum of glioneuronal lesions underlying intractable epilepsies includes malformative pathologies like focal cortical dysplasia (FCD); and neoplastic lesions like gangliogliomas (GG) and dysembryoplastic neuroepithelial tumours (DNT). "
2.	Sirolimus (Rapamycin)FDA Link 06/01/2023 - "The efficacy of the mechanistic target of rapamycin inhibitor, sirolimus, was recently reported for patients more than 6 years of age by Kato et al. We evaluated the efficacy and safety of sirolimus in a 2-year-old patient with recurrent focal seizures with impaired consciousness after focal cortical dysplasia (FCD) type IIa resection. " 01/01/2019 - "Several mosaic mutations of the mammalian/mechanistic target of rapamycin (mTOR) have recently been found in patients with cortical malformations, such as hemimegalencephaly (HME) and focal cortical dysplasia (FCD). " 01/01/2018 - "Review: Mechanistic target of rapamycin (mTOR) pathway, focal cortical dysplasia and epilepsy." 01/01/2022 - "To determine whether sirolimus, a mechanistic target of rapamycin (mTOR) inhibitor, reduces epileptic seizures associated with focal cortical dysplasia (FCD) type II. Sixteen patients (aged 6-57 years) with FCD type II received sirolimus at an initial dose of 1 or 2 mg/day based on body weight (FCDS-01). " 01/01/2018 - "Over the last decade, there has been increasing evidence that hyperactivation of the mechanistic target of rapamycin (mTOR) pathway is a hallmark of malformations of cortical development such as focal cortical dysplasia (FCD) or hemimegalencephaly. "
3.	Intravenous Immunoglobulins (IVIG)FDA Link 11/01/2017 - "Despite obvious limitations, mainly because of the small number of patients, and the selection biases, this study suggests that, based on the available data, IVIG might be effective in the treatment of intractable epilepsy secondary to focal cortical dysplasia. " 11/01/2017 - "We found four observational studies-prospective or retrospective-including patients with focal cortical dysplasia with intractable epilepsy treated with IVIG. " 11/01/2017 - "The observation of a dramatic response to intravenous immunoglobulin (IVIG) by a child from our center with intractable epilepsy due to focal cortical dysplasia prompted us to perform a meta-analysis on the efficiency of IVIG in this condition. " 11/01/2017 - "Intravenous Immunoglobulin as a Treatment for Intractable Epilepsy Secondary to Focal Cortical Dysplasia: A Meta-analysis."
4.	trans-sodium crocetinate (crocetin)IBA 06/01/2022 - "Multiple cortical tubers are easy to diagnose as TSC; however, a solitary cortical tuber without any other cutaneous or visceral organ manifestations can be confused with other conditions, particularly focal cortical dysplasia. " 02/01/2022 - "Somatic mutations involving TSC 1 and TSC2 genes in two children with focal cortical dysplasia." 12/01/2021 - "The epileptogenic zone in TSC patients harbors multiple dysplastic features, consistent with focal cortical dysplasia. " 12/01/2019 - "Changes in vascular density in resected tissue of 97 patients with mild malformation of cortical development, focal cortical dysplasia or TSC-related cortical tubers." 09/01/2016 - "There are similarities between TSC-associated CNS lesions and type IIb focal cortical dysplasia (FCD). "
5.	Proteins (Proteins, Gene)FDA Link 11/01/2022 - "Endoplasmic reticulum stress and unfolded protein accumulation correlate to seizure recurrence in focal cortical dysplasia patients." 03/01/2009 - "Increased expression of DLG3 protein was observed in the postsynaptic membrane fraction of patients with focal cortical dysplasia. " 09/01/2007 - "Expression and cellular distribution of multidrug resistance-related proteins in patients with focal cortical dysplasia." 11/01/1999 - "We have studied the localisation of several proteins in the Wnt pathway in focal cortical dysplasia (FCD). " 11/01/1999 - "Disturbance of Notch-1 and Wnt signalling proteins in neuroglial balloon cells and abnormal large neurons in focal cortical dysplasia in human cortex."
6.	Anticonvulsants (Antiepileptic Drugs)IBA 01/01/2019 - "Antiepileptic Drug Withdrawal after Surgery in Children with Focal Cortical Dysplasia: Seizure Recurrence and Its Predictors." 01/01/2019 - "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). " 10/01/2005 - "In particular, this review will focus on (i) the synaptic and non-synaptic properties of neocortical neurons along with their ability to produce synchronous activity; (ii) the anatomical and functional alterations that characterize limbic structures in patients presenting with mesial temporal lobe epilepsy; (iii) the issue of antiepileptic drug action and resistance; and (iv) the pathophysiology of seizure genesis in Taylor's type focal cortical dysplasia. " 01/01/2020 - "Temporal lobe epilepsy (TLE) and frontal lobe epilepsy (FLE) are the largest subgroup of partial epilepsy, and focal cortical dysplasias (FCDs) are highly epileptogenic brain lesions and are a frequent cause for antiepileptic drug (AED)-resistant focal epilepsies that mostly occur in the temporal and frontal lobes. " 01/01/2019 - " ABBREVIATIONS AED = antiepileptic drug; ANT = anterior nucleus of the thalamus; BOLD = blood oxygen level dependent; CCEP = cortico-cortical evoked potential; DBS = deep brain stimulation; ECoG = electrocorticography; ERSET = Early Randomized Surgical Epilepsy Trial; FCD = focal cortical dysplasia; HH = hypothalamic hamartoma; LITT = laser interstitial thermal therapy; RCT = randomized controlled trial; r-fMRI = resting-state functional MRI; RNS = responsive neurostimulation; SEEG = stereo-electroencephalography; VNS = vagus nerve stimulation."
7.	MicroRNAs (MicroRNA)IBA 04/01/2016 - "Analysis of Altered Micro RNA Expression Profiles in Focal Cortical Dysplasia IIB." 01/01/2017 - "Quantitative real-time PCR was used to measure plasma levels of three candidate microRNAs in two phases of study: an initial discovery phase with 14 patients with mesial temporal lobe epilepsy (MTLE), 13 with focal cortical dysplasia (FCD) and 16 controls; and a validation cohort constituted of an independent cohort of 65 patients with MTLE and 83 controls. " 06/01/2014 - "In this study, we investigated the role of miRNAs in the brains of immature (20-80 days) rats with liquid nitrogen lesion-induced focal cortical dysplasia. " 10/01/2020 - "Circulating MicroRNAs from Serum Exosomes May Serve as a Putative Biomarker in the Diagnosis and Treatment of Patients with Focal Cortical Dysplasia."
8.	mitomycin C-dextranIBA 08/04/2020 - "Results: Among the 58 patients, there were 47 cases of MCD (12 cases with mild malformation of cortical development (mMCD), 35 cases with focal cortical dysplasia (FCD)), and 11 cases of other types. " 09/01/2009 - "Particular emphasis will be paid to the classification system for focal cortical dysplasias (FCD), which can be histopathologically distinguished as type I and II. Also mild forms of cortical malformations (mMCD) may be present, including heterotopic neurons in white matter location. " 03/01/2019 - "Focal cortical dysplasia (FCD) and mild malformation of cortical development (mMCD) are frequent histopathologic diagnoses in patients who undergo surgery for refractory epilepsy. " 01/01/2019 - "Mild malformation of cortical development (mMCD) and focal cortical dysplasia (FCD) subtypes combined are by far the most common histological diagnoses in children who undergo surgery as treatment for refractory epilepsy. " 03/01/2022 - "Focal cortical dysplasia (FCD) are histopathologically categorized in ILAE type I to III. Mild malformations of cortical development (mMCD) including those with oligodendroglial hyperplasia (MOGHE) are to be integrated into this classification yet. "
9.	N-Methyl-D-Aspartate Receptors (NMDA Receptors)IBA 12/01/1999 - "This study was designed to quantify the relation between expressions of NMDA receptor (NMDAR) subunits (1 and 2A/B) and the epileptogenicity in human focal cortical dysplasia. " 08/01/2000 - "Epileptogenicity correlated with increased N-methyl-D-aspartate receptor subunit NR2A/B in human focal cortical dysplasia." 12/01/1999 - "NMDA-receptors 1 and 2A/B coassembly increased in human epileptic focal cortical dysplasia." 01/01/2022 - "Vascular endothelial growth factor-C modulates cortical NMDA receptor activity in cortical lesions of young patients and rat model with focal cortical dysplasia." 01/01/2008 - "To evaluate the alteration of subunits composition in NMDA receptor and the alterations of the expression and distribution of NMDA receptors and parvalbumin (PV)-positive neurons in focal cortical dysplasia (FCD) cortices. "
10.	Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link 01/01/2022 - "[18F]fluorodeoxyglucose ([18F]FDG) brain PET is used clinically to detect small areas of decreased uptake associated with epileptogenic lesions, e.g., Focal Cortical Dysplasias (FCD) but its performance is limited due to spatial resolution and low contrast. " 03/01/2000 - "The purpose of this work was to compare the use of MRI and [18F]fluorodeoxyglucose (FDG) positron emission tomography (PET) in the diagnosis of focal cortical dysplasia (FCD). " 06/01/2021 - "In this study, we used 18F-FDG PET/CT to investigate the risk factors for CCD and its prognostic value for intractable epilepsy caused by focal cortical dysplasia (FCD). " 06/01/2021 - "Crossed cerebellar diaschisis: risk factors and prognostic value in focal cortical dysplasia by 18F-FDG PET/CT." 05/01/2010 - "Evaluation of focal cortical dysplasia and mixed neuronal and glial tumors in pediatric epilepsy patients using 18F-FDG and 11C-methionine pet."

Therapies and Procedures

1.	Therapeutics 10/01/2022 - "MRI-guided laser interstitial thermal therapy (MRgLITT) is a novel treatment modality for focal cortical dysplasia (FCD). " 10/01/2022 - "Voxel-Based Morphometric MRI Postprocessing-Assisted Laser Interstitial Thermal Therapy for Focal Cortical Dysplasia-Suspected Lesions: Technique and Outcomes." 11/20/2023 - "Radiation-induced focal cortical dysplasia/neuronal gigantism is an incredibly rare complication of therapy. " 09/01/2016 - "Staged laser interstitial thermal therapy and topectomy for complete obliteration of complex focal cortical dysplasias." 02/01/2016 - "Focal cortical dysplasias (FCDs) are increasingly recognized as causes of therapy refractory epilepsies. "
2.	Lasers (Laser) 10/01/2022 - "MRI-guided laser interstitial thermal therapy (MRgLITT) is a novel treatment modality for focal cortical dysplasia (FCD). " 10/01/2022 - "Voxel-Based Morphometric MRI Postprocessing-Assisted Laser Interstitial Thermal Therapy for Focal Cortical Dysplasia-Suspected Lesions: Technique and Outcomes." 09/01/2016 - "Staged laser interstitial thermal therapy and topectomy for complete obliteration of complex focal cortical dysplasias." 01/01/2023 - "Magnetic resonance-guided laser interstitial thermal therapy (MRgLiTT) and stereoelectroencephalography-guided radiofrequency thermocoagulation (SEEG-RFTC) are two effective, minimally invasive treatments for epilepsy with focal cortical dysplasia (FCD). " 01/01/2023 - "Magnetic resonance-guided laser interstitial thermal therapy vs. stereoelectroencephalography-guided radiofrequency thermocoagulation in epilepsy patients with focal cortical dysplasia: a systematic review and meta-analysis."
3.	Operative Surgical Procedures 04/01/1997 - "Surgical procedures using the neuronavigation system were anterior temporal lobectomy, corpus callosotomy and lesionectomy of focal cortical dysplasia. " 07/01/1998 - "In the developmental group, low-grade focal cortical dysplasias had better outcome than heterotopia and hamartoma regardless of type of surgical procedure. " 06/01/2013 - "We prospectively investigated a series of 25 patients with symptomatic epilepsy due to isolated forms of focal cortical dysplasia (FCD) or hippocampal sclerosis and low-grade tumours associated with FCD, all of whom underwent tailored surgical procedures under intraoperative electrocorticography; we conducted neuropathological examinations of 92 biopsies taken from different places. "
4.	Electrodes (Electrode) 01/01/2023 - "As part of an ongoing trial, we implanted a patient with a novel focal cortex stimulation (FCS) device with a Laplacian electrode placed over a precentral focal cortical dysplasia. " 01/01/2016 - "The aim of the study was the evaluation of the added value of depth to subdural electrodes in delineating epileptogenicity of focal cortical dysplasias (FCDs) and to test the Epileptogenicity Index (EI) in this setting. " 01/01/2018 - "MRI with electrodes in situ was used for neuronavigation in all 29 who underwent resection and yielded a histopathological diagnosis of focal cortical dysplasia in 15 MRI-negative patients. " 09/01/2017 - "Characteristics of EEG Seizure-Onset Patterns Recorded From Subdural Electrodes Over MRI-Visible Frontal Focal Cortical Dysplasia Type IIb Lesions." 07/01/2011 - "The mean total number of electrodes was 118 ± 29; the mean numbers of grid, strip, and depth electrodes were 95 ± 27, 10 ± 6, and 13 ± 5, respectively The most common pathological condition was focal cortical dysplasia. "
5.	Anterior Temporal Lobectomy 04/01/1997 - "Surgical procedures using the neuronavigation system were anterior temporal lobectomy, corpus callosotomy and lesionectomy of focal cortical dysplasia. " 01/01/2020 - "Objective: This study aims to evaluate the surgical outcomes and analyze the predictors of surgical outcomes in patients undergoing anterior temporal lobectomy (ATL) for drug-resistant temporal lobe epilepsy (TLE) secondary to focal cortical dysplasia (FCD) type IIIa. "