scholarly journals Surgical outcome and prognostic factors in epilepsy patients with MR-negative focal cortical dysplasia

PLoS ONE ◽  
2021 ◽  
Vol 16 (4) ◽  
pp. e0249929
Author(s):  
Min Jae Seong ◽  
Su Jung Choi ◽  
Eun Yeon Joo ◽  
Young-Min Shon ◽  
Dae-Won Seo ◽  
...  
Keyword(s):  
Prognostic Factors ◽  
Surgical Outcomes ◽  
Focal Cortical Dysplasia ◽  
Cortical Dysplasia ◽  
Type I ◽  
Epileptiform Discharges ◽  
Interictal Epileptiform Discharges ◽  
Neuroimaging Data ◽  
Magnetic Resonance Imaging Mri ◽  
Drug Resistant Epilepsy

Objective Focal cortical dysplasia (FCD) represents a heterogeneous group of disorders of the cortical formation and is one of the most common causes of epilepsy. Magnetic resonance imaging (MRI) is the modality of choice for detecting structural lesions, and the surgical prognosis in patients with MR lesions is favorable. However, the surgical prognosis of patients with MR-negative FCD is unknown. We aimed to evaluate the long-term surgical outcomes and prognostic factors in MR-negative FCD patients through comprehensive presurgical data. Methods We retrospectively reviewed data from 719 drug-resistant epilepsy patients who underwent resective surgery and selected cases in which surgical specimens were pathologically confirmed as FCD Type I or II. If the epileptogenic focus and surgical specimens were obtained from brain areas with a normal MRI appearance, they were classified as MR-negative FCD. Surgical outcomes were evaluated at 2 and 5 years, and clinical, neurophysiological, and neuroimaging data of MR-negative FCD were compared to those of MR-positive FCD. Results Finally, 47 MR-negative and 34 MR-positive FCD patients were enrolled in the study. The seizure-free rate after surgery (Engel classification I) at postoperative 2 year was 59.5% and 64.7% in the MR-negative and positive FCD groups, respectively (p = 0.81). This rate decreased to 57.5% and 44.4% in the MR-negative and positive FCD groups (p = 0.43) at postoperative 5 years. MR-negative FCD showed a higher proportion of FCD type I (87.2% vs. 50.0%, p = 0.001) than MR-positive FCD. Unilobar cerebral perfusion distribution (odds ratio, OR 5.41) and concordance of interictal epileptiform discharges (OR 5.10) were significantly associated with good surgical outcomes in MR-negative FCD. Conclusion In this study, MR-negative and positive FCD patients had a comparable surgical prognosis, suggesting that comprehensive presurgical evaluations, including multimodal neuroimaging studies, are crucial for obtaining excellent surgical outcomes even in epilepsy patients with MR-negative FCD.

Dynamic Statistical Parametric Mapping for Analyzing the Magnetoencephalographic Epileptiform Activity in Patients With Epilepsy

2004 ◽  
Vol 19 (3) ◽  
pp. 363-369
Author(s):  
Hideaki Shiraishi ◽  
Steven M. Stufflebeam ◽  
Susanne Knake ◽  
Seppo P. Ahlfors ◽  
Akira Sudo ◽  
...  
Keyword(s):  
Focal Cortical Dysplasia ◽  
Epileptiform Activity ◽  
Rapid Change ◽  
Statistical Parametric Mapping ◽  
Cortical Dysplasia ◽  
Parametric Mapping ◽  
Epileptiform Discharges ◽  
Interictal Epileptiform Discharges ◽  
L2 Norm ◽  
Patients With Epilepsy

Our current purpose is to evaluate the applicability of dynamic statistical parametric mapping, a novel method for localizing epileptiform activity recorded with magnetoencephalography in patients with epilepsy. We report four pediatric patients with focal epilepsies. Magnetoencephalographic data were collected with a 306-channel whole-head helmet-shaped sensor array. We calculated equivalent current dipoles and dynamic statistical parametric mapping movies of the interictal epileptiform discharges that were based in the minimum-L2 norm estimate, minimizing the square sum of the dipole element amplitudes. The dynamic statistical parametric mapping analysis of interictal epileptiform discharges can demonstrate the rapid change and propagation of interical epileptiform discharges. According to these findings, specific epileptogenic lesion—focal cortical dysplasia could be found and patients could be operated on successfully. The presurgical analysis of interictal epileptiform discharges using dynamic statistical parametric mapping seems to be promising in patients with a possible underlying focal cortical dysplasia and might help to guide the placement of invasive electrodes. ( J Child Neurol 2005;20:363—369).

scholarly journals Automatic Detection of Focal Cortical Dysplasia Type II in MRI: Is the Application of Surface-Based Morphometry and Machine Learning Promising?

2021 ◽  
Vol 15 ◽  
Author(s):  
Zohreh Ganji ◽  
Mohsen Aghaee Hakak ◽  
Seyed Amir Zamanpour ◽  
Hoda Zare
Keyword(s):  
Neural Network ◽  
Machine Learning ◽  
Focal Cortical Dysplasia ◽  
Diagnostic System ◽  
Cortical Dysplasia ◽  
Type Ii ◽  
Magnetic Resonance Imaging Mri ◽  
Drug Resistant Epilepsy ◽  
Sensitivity Specificity ◽  
Postsurgical Outcomes

Background and ObjectivesFocal cortical dysplasia (FCD) is a type of malformations of cortical development and one of the leading causes of drug-resistant epilepsy. Postoperative results improve the diagnosis of lesions on structural MRIs. Advances in quantitative algorithms have increased the identification of FCD lesions. However, due to significant differences in size, shape, and location of the lesion in different patients and a big deal of time for the objective diagnosis of lesion as well as the dependence of individual interpretation, sensitive approaches are required to address the challenge of lesion diagnosis. In this research, a FCD computer-aided diagnostic system to improve existing methods is presented.MethodsMagnetic resonance imaging (MRI) data were collected from 58 participants (30 with histologically confirmed FCD type II and 28 without a record of any neurological prognosis). Morphological and intensity-based features were calculated for each cortical surface and inserted into an artificial neural network. Statistical examinations evaluated classifier efficiency.ResultsNeural network evaluation metrics—sensitivity, specificity, and accuracy—were 96.7, 100, and 98.6%, respectively. Furthermore, the accuracy of the classifier for the detection of the lobe and hemisphere of the brain, where the FCD lesion is located, was 84.2 and 77.3%, respectively.ConclusionAnalyzing surface-based features by automated machine learning can give a quantitative and objective diagnosis of FCD lesions in presurgical assessment and improve postsurgical outcomes.

Assessment and surgical outcomes for mild type I and severe type II cortical dysplasia: A critical review and the UCLA experience

Epilepsia ◽  
2009 ◽  
Vol 50 (6) ◽  
pp. 1310-1335 ◽  
Author(s):  
Jason T. Lerner ◽  
Noriko Salamon ◽  
Jason S. Hauptman ◽  
Tonicarlo R. Velasco ◽  
Marta Hemb ◽  
...  
Keyword(s):  
Critical Review ◽  
Surgical Outcomes ◽  
Cortical Dysplasia ◽  
Type I ◽  
Type Ii ◽  
Severe Type

scholarly journals Somatic mutation involving diverse genes leads to a spectrum of focal cortical malformations

2021 ◽  
Author(s):  
Dulcie Lai ◽  
Meethila Gade ◽  
Edward Yang ◽  
Hyun Yong Koh ◽  
Nicole M. Walley ◽  
...  
Keyword(s):  
Focal Cortical Dysplasia ◽  
Hippocampal Sclerosis ◽  
Cortical Development ◽  
Copy Number Variants ◽  
Cortical Dysplasia ◽  
Disease Genes ◽  
Type I ◽  
Type Ii ◽  
Loss Of Function ◽  
Brain Malformations

Post-zygotically acquired genetic variants, or somatic variants, that arise during cortical development have emerged as important causes of focal epilepsies, particularly those due to malformations of cortical development. Pathogenic somatic variants have been identified in many genes within the PI3K-AKT3-mTOR-signaling pathway in individuals with hemimegalencephaly and focal cortical dysplasia (type II), and more recently in SLC35A2 in individuals with focal cortical dysplasia (type I) or non-dysplastic epileptic cortex. Given the expanding role of somatic variants across different brain malformations, we sought to delineate the landscape of somatic variants in a large cohort of patients who underwent epilepsy surgery with hemimegalencephaly or focal cortical dysplasia. We evaluated samples from 123 children with hemimegalencephaly (n=16), focal cortical dysplasia type I and related phenotypes (n=48), focal cortical dysplasia type II (n=44), or focal cortical dysplasia type III (n=15) classified using imaging and pathological findings. We performed high-depth exome sequencing in brain tissue-derived DNA from each case and identified somatic single nucleotide, indel, and large copy number variants. In 75% of individuals with hemimegalencephaly and 29% with focal cortical dysplasia type II, we identified pathogenic variants in PI3K-AKT-mTOR pathway genes. Four of 48 cases with focal cortical dysplasia type I (8%) had a likely pathogenic variant in SLC35A2. While no other gene had multiple disease-causing somatic variants across the focal cortical dysplasia type I cohort, four individuals in this group had a single pathogenic or likely pathogenic somatic variant in CASK, KRAS, NF1, and NIPBL, genes associated with neurodevelopmental disorders. No rare pathogenic or likely pathogenic somatic variants in any neurological disease genes like those identified in the focal cortical dysplasia type I cohort were found in 63 neurologically normal controls (P = 0.017), suggesting a role for these novel variants. We also identified a somatic loss-of-function variant in the known epilepsy gene, PCDH19, present in a very small number of alleles in the dysplastic tissue from a female patient with focal cortical dysplasia IIIa with hippocampal sclerosis. In contrast to focal cortical dysplasia type II, neither focal cortical dysplasia type I nor III had somatic variants in genes that converge on a unifying biological pathway, suggesting greater genetic heterogeneity compared to type II. Importantly, we demonstrate that FCD types I, II, and III, are associated with somatic gene variants across a broad range of genes, many associated with epilepsy in clinical syndromes caused by germline variants, as well as including some not previously associated with radiographically evident cortical brain malformations.

scholarly journals MRI of focal cortical dysplasia

2021 ◽  
Author(s):  
Horst Urbach ◽  
Elias Kellner ◽  
Nico Kremers ◽  
Ingmar Blümcke ◽  
Theo Demerath
Keyword(s):  
Molecular Genetics ◽  
Focal Cortical Dysplasia ◽  
Cortical Development ◽  
Cortical Dysplasia ◽  
Added Value ◽  
7 Tesla ◽  
Type I ◽  
Type Ii ◽  
Malformations Of Cortical Development ◽  
7 Tesla Mri

AbstractFocal 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. Only FCD type II have distinctive MRI and molecular genetics alterations so far. Subtle FCD including FCD type II located in the depth of a sulcus are often overlooked requiring the use of dedicated sequences (MP2RAGE, FLAWS, EDGE) and/or voxel (VBM)- or surface-based (SBM) postprocessing. The added value of 7 Tesla MRI has to be proven yet.

scholarly journals Distributed Brain Co-Processor for Neurophysiologic Tracking and Adaptive Stimulation: Application to Drug Resistant Epilepsy

2021 ◽  
Author(s):  
Vladimir Sladky ◽  
Petr Nejedly ◽  
Filip Mivalt ◽  
Benjamin H. Brinkmann ◽  
Inyong Kim ◽  
...  
Keyword(s):  
Data Storage ◽  
Brain Stimulation ◽  
Open Loop ◽  
Natural Environments ◽  
Drug Resistant ◽  
Embedded Computing ◽  
Epileptiform Discharges ◽  
Interictal Epileptiform Discharges ◽  
Drug Resistant Epilepsy

AbstractElectrical brain stimulation is a proven therapy for epilepsy, but long-term seizure free outcomes are rare. Early implantable devices were developed for open-loop stimulation without sensing, embedded computing or adaptive therapy. Recent device advances include sensing and closed-loop responsive stimulation, but these clinically available devices lack adequate computing, data storage and patient interface to concisely catalog behavior, seizures, and brain electrophysiology, despite the critical importance of these details for epilepsy management. Here we describe the first application of a distributed brain co-processor providing an intuitive, bi-directional interface between device implant, patient & physician, and implement it in human and canine patients with epilepsy living in their natural environments. Automated behavioral state tracking (awake and sleep) and electrophysiologic classifiers for interictal epileptiform discharges and electrographic seizures are run on local hand-held and distributed cloud computing resources to guide adaptive electrical stimulation. These algorithms were first developed and parameterized using long-term retrospective data from 10 humans and 11 canines with epilepsy and then implemented prospectively in two pet canines and one human with drug resistant epilepsy as they naturally navigate their lives in society.One Sentence SummaryWe created a distributed brain co-processor for continuous neurophysiologic tracking and controlling adaptive brain stimulation to treat epilepsy.

scholarly journals Congruence and Discrepancy of Interictal and Ictal EEG With MRI Lesions in Pediatric Epilepsies

2020 ◽  
Vol 51 (6) ◽  
pp. 412-419
Author(s):  
Jan Rémi ◽  
Sophie Shen ◽  
Moritz Tacke ◽  
Philipp Probst ◽  
Lucia Gerstl ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Pediatric Epilepsy ◽  
Resonance Imaging ◽  
Epileptiform Discharges ◽  
Interictal Epileptiform Discharges ◽  
Ictal Eeg ◽  
Magnetic Resonance Imaging Mri ◽  
Surface Eeg ◽  
Frontal Lesions ◽  
Video Eeg

Purpose. To evaluate the congruence or discrepancy of the localization of magnetic resonance imaging (MRI) lesions with interictal epileptiform discharges (IEDs) or epileptic seizure patterns (ESPs) in surface EEG in lesional pediatric epilepsy patients. Methods. We retrospectively analyzed presurgical MRI and video-EEG monitoring findings of patients up to age 18 years. Localization of MRI lesions were compared with ictal and interictal noninvasive EEG findings of patients with frontal, temporal, parietal, or occipital lesions. Results. A total of 71 patients were included. Localization of ESPs showed better congruence with MRI in patients with frontal lesions (n = 21, 77.5%) than in patients with temporal lesions (n = 24; 40.7%) ( P = .009). No significant IED distribution differences between MRI localizations could be found. Conclusions. MRI lesions and EEG findings are rarely fully congruent. Congruence of MRI lesions and ESPs was highest in children with frontal lesions. This is in contrast to adults, in whom temporal lesions showed the highest congruency with the EEG localization of ESP. Lesional pediatric patients should be acknowledged as surgical candidates despite incongruent findings of interictal and ictal surface EEG.

Seizure Outcome After Surgery for MRI-Diagnosed Focal Cortical Dysplasia: A Systematic Review and Meta-analysis

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000013066
Author(s):  
Anna Willard ◽  
Ana Antonic-Baker ◽  
Zhibin Chen ◽  
Terence John O'Brien ◽  
Patrick Kwan ◽  
...  
Keyword(s):  
Systematic Review ◽  
Focal Cortical Dysplasia ◽  
Meta Analysis ◽  
Cortical Dysplasia ◽  
Favorable Outcome ◽  
Seizure Outcome ◽  
Drug Resistant ◽  
Resective Surgery ◽  
Post Surgery ◽  
Drug Resistant Epilepsy

Objective:Focal cortical dysplasia (FCD) has been associated with poorer post-surgical seizure outcomes compared to other pathologies. FCD surgical series have been assembled on the basis of a histological diagnosis, including patients with abnormal as well as normal pre-operative MRI. However, in clinical workflow, patient selection for surgery is based on pre-operative findings, including MRI. We performed a systematic review and meta-analysis of the literature to determine the rate and predictors of favorable seizure outcome after surgery for MRI-detected FCD.Methods:We devised our study protocol in accordance with PRISMA guidelines and registered the protocol with PROSPERO. We searched MEDLINE, EMBASE, and Web of Science for studies of patients followed for ≥12 months after resective surgery for drug-resistant epilepsy with MRI-detected FCD. Random-effects meta-analysis was used to calculate the proportion of patients attaining a favorable outcome, defined as Engel Class I, ILAE Classes 1-2, or “seizure-free” status. Meta-regression was performed to investigate sources of heterogeneity.Results:Our search identified 3,745 references. Of these, 35 studies (total of 1,353 patients) were included. Most studies (89%) followed patients for ≥24 months post-surgery. The overall post-surgical favorable outcome rate was 70% (95% CI: 64-75). There was high inter-study heterogeneity. Favorable outcome was associated with complete resection of the FCD lesion [risk ratio, RR=2.42 (95% CI: 1.55-3.76), p<0.001] and location of the FCD lesion in the temporal lobe [RR=1.38 (95% CI: 1.07-1.79), p=0013], but not lesion extent, intracranial EEG use, or FCD histological type. The number of FCD histological types included in the same study accounted for 7.6% of the observed heterogeneity.Conclusions:70% of patients with drug-resistant epilepsy and MRI features of FCD attain a favorable seizure outcome following resective surgery. Our findings can be incorporated in routine pre-operative counselling and reinforce the importance of resecting completely the MRI-detected FCD where this is safe and feasible.

scholarly journals Spontaneous seizure remission following status epilepticus in drug-resistant epilepsy due to focal cortical dysplasia

2017 ◽  
Vol 137 ◽  
pp. 73-77 ◽  
Author(s):  
Nadine Girard ◽  
Eric Guedj ◽  
Patrick Chauvel ◽  
Fabrice Bartolomei ◽  
Aileen McGonigal
Keyword(s):  
Status Epilepticus ◽  
Focal Cortical Dysplasia ◽  
Cortical Dysplasia ◽  
Drug Resistant ◽  
Spontaneous Seizure ◽  
Drug Resistant Epilepsy
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