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Focal cortical dysplasia

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https://www.readbyqxmd.com/read/28919879/automated-online-quantification-method-for-18-f-fdg-positron-emission-tomography-ct-improves-detection-of-the-epileptogenic-zone-in-patients-with-pharmacoresistant-epilepsy
#1
Vanessa Cristina Mendes Coelho, Marcia E Morita, Barbara J Amorim, Celso Darío Ramos, Clarissa L Yasuda, Helder Tedeschi, Enrico Ghizoni, Fernando Cendes
AIMS: To assess the validity of an online method to quantitatively evaluate cerebral hypometabolism in patients with pharmacoresistant focal epilepsy as a complement to the visual analysis of the (18)F-FDG positron emission tomography (PET)/CT exam. METHODS: A total of 39 patients with pharmacoresistant epilepsy and probable focal cortical dysplasia [22 patients with frontal lobe epilepsy (FLE) and 17 with temporal lobe epilepsy (TLE)] underwent a presurgical evaluation including EEG, video-EEG, MRI, and (18)F-FDG PET/CT...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28899045/recent-advances-in-epilepsy-surgery-and-achieving-best-outcomes-using-high-frequency-oscillations-diffusion-tensor-imaging-magnetoencephalography-intraoperative-neuromonitoring-focal-cortical-dysplasia-and-bottom-of-sulcus-dysplasia
#2
https://www.readbyqxmd.com/read/28899007/the-spectrum-of-structural-and-functional-network-alterations-in-malformations-of-cortical-development
#3
Seok-Jun Hong, Boris C Bernhardt, Ravnoor S Gill, Neda Bernasconi, Andrea Bernasconi
Neuroimaging studies of malformations of cortical development have mainly focused on the characterization of the primary lesional substrate, while whole-brain investigations remain scarce. Our purpose was to assess large-scale brain organization in prevalent cortical malformations. Based on experimental evidence suggesting that distributed effects of focal insults are modulated by stages of brain development, we postulated differential patterns of network anomalies across subtypes of malformations. We studied a cohort of patients with focal cortical dysplasia type II (n = 63), subcortical nodular heterotopia (n = 44), and polymicrogyria (n = 34), and compared them to 82 age- and sex-matched controls...
August 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28898173/utility-of-stereoelectroencephalography-in-children-with-dysembryoplastic-neuroepithelial-tumor-and-cortical-malformation
#4
Jun T Park, Guadalupe Fernandez Baca Vaca, Jennifer Avery, Jonathan P Miller
BACKGROUND: Uncontrolled seizures in children can contribute to irreversible cognitive impairment and developmental delay, in addition to placing them at risk for sudden unexplained death in epileptic patients (SUDEP). Since its introduction at Saint Ann Hospital in Paris in the 1960s, stereoelectroencephalography (SEEG) is increasingly being utilized at epilepsy centers in the United States as an invasive tool to help localize the seizure focus in drug-resistant focal epilepsy. INDICATIONS: Children with symptomatic epilepsy, commonly due to cortical dysplasia and dysembryoplastic neuroepithelial tumor (DNET), may benefit from SEEG investigation...
2017: Neurodiagnostic Journal
https://www.readbyqxmd.com/read/28888331/hamartia-in-hippocampal-sclerosis-associated-mesial-temporal-lobe-epilepsy
#5
REVIEW
K L Gawelek, J M Gales, R A Prayson
Hamartia are small collections of rounded glioneuronal cells that are thought to be due to aberrant cell migration. Their presence has been recognized in association with mesial temporal lobe epilepsy; their prevalence among cases of hippocampal sclerosis (HS) and any potential association with patient demographics and outcomes is unknown. This study examines hamartia in a series of 292 patients with pathologically confirmed HS. Medical records were reviewed for pertinent patient clinical information (follow-up mean 5years)...
September 6, 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/28879901/braf-gene-alterations-and-enhanced-mammalian-target-of-rapamycin-signaling-in-gangliogliomas
#6
Aanchal Kakkar, Atreye Majumdar, Pankaj Pathak, Anupam Kumar, Kalpana Kumari, Manjari Tripathi, Mehar C Sharma, Vaishali Suri, Vivek Tandon, Sarat P Chandra, Chitra Sarkar
BACKGROUND: Gangliogliomas (GGs) are slow-growing glioneuronal tumors seen in children and young adults. They are associated with intractable epilepsy, and have recently been found to harbor BRAF (B- rapidly accelerated fibrosarcoma) gene mutations. However, the mammalian target of rapamycin (mTOR) signaling pathway, downstream of BRAF, has not been evaluated extensively in GGs. MATERIALS AND METHODS: GG cases were retrieved, clinical data obtained, and histopathological features reviewed...
September 2017: Neurology India
https://www.readbyqxmd.com/read/28853521/-hemispherotomy-for-treatment-of-refractory-epilepsy-in-children
#7
Jonathan Roth, Sari Nagar, Shlomi Constantini, Itzhak Fried
INTRODUCTION: Hemispherotomies are an extreme treatment for epilepsy. As opposed to focal resections, in hemispherotomies the entire hemisphere is disconnected from the remaining nervous system, including functional regions and fibers. Despite this, hemispherotomy is recommended for certain indications, with good epilepsy and functional outcomes. AIMS: To summarize the experience of hemispherotomy performed at a tertiary national center. METHODS: A retrospective study was conducted including all patients operated on between 2001 and 2014...
August 2017: Harefuah
https://www.readbyqxmd.com/read/28843774/putting-the-new-ilae-classification-of-focal-cortical-dysplasia-into-practice-in-western-china
#8
Cheng Huang, Heng Zhang, Xiao-Sa Chi, Ni Chen, Jing Gong, Qiao Zhou, Ingmar Blümcke, Dong Zhou, Jin-Mei Li
PURPOSE: To re-examine drug-resistant epilepsy cases using the revised 2011 ILAE classification of focal cortical dysplasia (FCD). METHODS: Patients with drug-resistant epilepsy who have undergone epilepsy surgery in West China Hospital between July 2012 and Jun 2014 were included. Clinical histories, pathological diagnoses, and surgical outcomes were reviewed. A questionnaire was developed to investigate the clinical practice of the new classification. A short-term training program on FCD was carried out to improve pathological diagnosis accuracy...
July 24, 2017: Seizure: the Journal of the British Epilepsy Association
https://www.readbyqxmd.com/read/28839247/spatiotemporal-changes-in-regularity-of-gamma-oscillations-contribute-to-focal-ictogenesis
#9
Yosuke Sato, Simeon M Wong, Yasushi Iimura, Ayako Ochi, Sam M Doesburg, Hiroshi Otsubo
In focal ictogenesis, gamma oscillations (30-70 Hz) recorded by electroencephalography (EEG) are related to the epileptiform synchronization of interneurons that links the seizure onset zone (SOZ) to the surrounding epileptogenic zone. We hypothesized that the synchronization of interneurons could be detected as changes in the regularity of gamma oscillation rhythmicity. We used multiscale entropy (MSE) analysis, which can quantify the regularity of EEG rhythmicity, to investigate how the regularity of gamma oscillations changes over the course of a seizure event...
August 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28833756/multinodular-and-vacuolating-neuronal-tumours-in-epilepsy-dysplasia-or-neoplasia
#10
Maria Thom, Joan Liu, Anika Bongaarts, Roy J Reinten, Beatrice Paradiso, Hans Rolf Jäger, Cheryl Reeves, Alyma Somani, Shu An, Derek Marsdon, Andrew McEvoy, Anna Miserocchi, Lewis Thorne, Fay Newman, Sorin Bucur, Mrinalini Honavar, Tom Jacques, Eleonora Aronica
Multinodular and vacuolating neuronal tumour (MVNT) is a new pattern of neuronal tumour included in the recently revised WHO 2016 classification of tumours of the CNS. There are fifteen reports in the literature to date. They are typically associated with late onset epilepsy and a neoplastic versus malformative biology has been questioned. We present a series of ten cases and compare their pathological and genetic features to better characterised epilepsy associated malformations including focal cortical dysplasia type II (FCDII) and low-grade epilepsy associated tumours (LEAT)...
August 19, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28833053/a-distinct-clinicopathological-variant-of-focal-cortical-dysplasia-iiid-characterized-by-loss-of-layer-4-in-the-occipital-lobe-in-12-children-with-remote-hypoxic-ischemic-injury
#11
Dan-Dan Wang, Yue-Shan Piao, Ingmar Blumcke, Roland Coras, Wen-Jing Zhou, Qiu-Ping Gui, Cui-Cui Liu, Jing-Xia Hu, Li-Zhen Cao, Guo-Jun Zhang, De-Hong Lu
OBJECTIVE: In 2011, the International League Against Epilepsy (ILAE) proposed a consensus classification system of focal cortical dysplasia (FCD) to distinguish clinicopathological subtypes, for example, "isolated" FCD type Ia-c and IIa-b, versus "associated" FCD type IIIa-d. The histopathological differentiation of FCD type I and III variants remains, however, a challenging issue in everyday practice. We present a unique histopathological pattern in patients with difficult-to-diagnose FCD, which highlights this dilemma, but also helps to refine the current ILAE classification scheme of FCD...
August 23, 2017: Epilepsia
https://www.readbyqxmd.com/read/28833036/treatment-with-lacosamide-impedes-generalized-seizures-in-a-rodent-model-of-cortical-dysplasia
#12
Ashley D Nemes, Rebecca O'Dwyer, Imad M Najm, Zhong Ying, Jorge Gonzalez-Martinez, Andreas V Alexopoulos
OBJECTIVE: Epilepsy is a common neurologic disorder resulting in spontaneous, recurrent seizures. About 30-40% of patients are not responsive to pharmacologic therapies. This may be due to the differences between individual patients such as etiology, underlying pathophysiology, and seizure focus, and it highlights the importance of new drug discovery and testing in this field. Our goal was to determine the efficacy of lacosamide (LCM), a drug approved for the treatment of focal seizures, in a model of generalized epilepsy with cortical dysplasia (CD)...
August 20, 2017: Epilepsia
https://www.readbyqxmd.com/read/28828916/magnetoencephalographic-identification-of-epileptic-focus-in-children-with-generalized-electroencephalographic-eeg-features-but-focal-imaging-abnormalities
#13
Garima Shukla, Jin Kazutaka, Ajay Gupta, John Mosher, Stephen Jones, Andreas Alexopoulos, Richard C Burgess
PURPOSE: Children with generalized seizures are often excluded as epilepsy surgery candidates. This prospective study was conducted to evaluate the utility of magnetoencephalography (MEG) to refine the location of the "irritative zone" in children with single lesions on magnetic resonance imaging (MRI) but with generalized ictal electroencephalographic (EEG) findings. METHODS: Patients admitted with refractory epilepsy with imaging studies showing focal or hemispheric abnormalities but scalp video EEG showing generalized or multiregional epileptiform abnormalities were included...
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28804898/epilepsy-surgery-of-low-grade-epilepsy-associated-neuroepithelial-tumors-a-retrospective-nationwide-italian-study
#14
Marco Giulioni, Gianluca Marucci, Veronica Pelliccia, Francesca Gozzo, Carmen Barba, Giuseppe Didato, Flavio Villani, Giancarlo Di Gennaro, Pier Paolo Quarato, Vincenzo Esposito, Alessandro Consales, Matteo Martinoni, Gianfranco Vornetti, Corrado Zenesini, Carlo Efisio Marras, Nicola Specchio, Luca De Palma, Raffaele Rocchi, Flavio Giordano, Giovanni Tringali, Paolo Nozza, Gabriella Colicchio, Guido Rubboli, Giorgio Lo Russo, Renzo Guerrini, Paolo Tinuper, Francesco Cardinale, Massimo Cossu
OBJECTIVE: To analyze the attitude and results of Italian epilepsy surgery centers in the surgical management of "low grade epilepsy associated neuroepithelial tumors" (LEATs). METHODS: We conducted a retrospective study enrolling 339 consecutive patients with LEATs who underwent surgery between January 2009 and June 2015 at eight Italian epilepsy surgery centers. We compared demographic, clinical, pathologic, and surgical features of patients with favorable (Engel class I) and unfavorable (Engel class II, III, and IV) seizure outcome...
August 14, 2017: Epilepsia
https://www.readbyqxmd.com/read/28762286/childhood-onset-epileptic-encephalopathy-associated-with-isolated-focal-cortical-dysplasia-and-a-novel-tsc1-germline-mutation
#15
Hannes Hoelz, Eva Coppenrath, Konstanze Hoertnagel, Timo Roser, Moritz Tacke, Lucia Gerstl, Ingo Borggraefe
Tuberous sclerosis complex (TSC) is an autosomal-dominant inheritable neurocutaneous disease due to mutations within the TSC1 and TSC2 genes. Many patients present with West syndrome, a severe epilepsy syndrome characterized by the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia (continuous slow activity with an amplitude higher than 300 µV and multiregional spikes/polyspikes/sharp waves) and developmental regression. In this study, we report on a previously healthy patient with positive family history of epilepsy with new-onset epileptic encephalopathy at the age of 9 years...
March 1, 2017: Clinical EEG and Neuroscience: Official Journal of the EEG and Clinical Neuroscience Society (ENCS)
https://www.readbyqxmd.com/read/28753112/impact-of-focal-cortical-dysplasia-type-iiia-on-seizure-outcome-following-anterior-mesial-temporal-lobe-resection-for-the-treatment-of-epilepsy
#16
Lasse Dührsen, Thomas Sauvigny, Patrick M House, Stefan Stodieck, Brigitte Holst, Jakob Matschke, Gerhard Schön, Manfred Westphal, Tobias Martens
OBJECTIVE Temporal lobe epilepsy (TLE) is the most common type of pharmacoresistant focal epilepsy, for which anterior mesial temporal lobe resection (AMTLR) is a treatment option. Focal cortical dysplasia Type IIIa (FCD IIIa), a developmental lesion resulting from defects in neuronal formation and migration into the temporal pole (FCD I) combined with hippocampal sclerosis (HS), can be a neuropathological finding. In this study, the authors investigate the impact of FCD IIIa on seizure outcome in patients with TLE who underwent AMTLR...
July 28, 2017: Journal of Neurosurgery
https://www.readbyqxmd.com/read/28749594/seizure-activity-per-se-does-not-induce-tissue-damage-markers-in-human-neocortical-focal-epilepsy
#17
Laura Rossini, Rita Garbelli, Vadym Gnatkovsky, Giuseppe Didato, Flavio Villani, Roberto Spreafico, Francesco Deleo, Giorgio Lo Russo, Giovanni Tringali, Francesca Gozzo, Laura Tassi, Marco de Curtis
OBJECTIVE: The contribution of recurring seizures to the progression of epileptogenesis is debated. Seizure-induced brain damage is not conclusively demonstrated either in humans or in animal models of epilepsy. We evaluated the expression of brain injury biomarkers on postsurgical brain tissue obtained from 20 patients with frequent seizures and a long history of drug-resistant focal epilepsy. METHODS: The expression patterns of specific glial, neuronal, and inflammatory molecules were evaluated by immunohistochemistry in the core of type II focal cortical dysplasias (FCD-II), at the FCD boundary (perilesion), and in the adjacent normal-appearing area included in the epileptogenic region...
July 27, 2017: Annals of Neurology
https://www.readbyqxmd.com/read/28745400/voxel-based-magnetic-resonance-image-postprocessing-in-epilepsy
#18
Pascal Martin, Gavin P Winston, Philippa Bartlett, Jane de Tisi, John S Duncan, Niels K Focke
OBJECTIVE: Although the general utility of voxel-based processing of structural magnetic resonance imaging (MRI) data for detecting occult lesions in focal epilepsy is established, many differences exist among studies, and it is unclear which processing method is preferable. The aim of this study was to compare the ability of commonly used methods to detect epileptogenic lesions in magnetic resonance MRI-positive and MRI-negative patients, and to estimate their diagnostic yield. METHODS: We identified 144 presurgical focal epilepsy patients, 15 of whom had a histopathologically proven and MRI-visible focal cortical dysplasia; 129 patients were MRI negative with a clinical hypothesis of seizure origin, 27 of whom had resections...
September 2017: Epilepsia
https://www.readbyqxmd.com/read/28737124/distribution-and-expression-of-brain-derived-neurotrophic-factor-nerve-growth-factor-and-neurotrophic-factor-3-in-refractory-epilepsy-associated-focal-cortical-dysplasia
#19
Feng Wang, Yuanxiang Lin, Dezhi Kang, Fuxiang Chen, Kun Lin, Xingfen Su
OBJECTIVE: To investigate the expression and distribution of brain-derived neurotrophic factor (BDNF), nerve growth factor (NGF), and neurotrophic factor-3 (NT-3) in refractory epilepsy-associated type I focal cortical dysplasia (FCD I) and FCD IIA patients, and to explore their effects on pathogenesis of FCD I and FCD IIA. MATERIALS AND METHODS: 19 subjects who received surgery at the Department of Neurosurgery, First Affiliated Hospital, Fujian Medical University, China between June 2010 and May 2012, were enrolled in this study...
July 24, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28725554/successful-surgical-management-of-new-onset-refractory-status-epilepticus-norse-presenting-with-gelastic-seizures-in-a-3%C3%A2-year-old-girl
#20
Ahmad Marashly, Sean Lew, Jennifer Koop
Gelastic seizures (GS) are typically associated with hypothalamic hamartomas and present during childhood. However it is now known that GS can be found in focal epilepsies arising from other regions in the brain, including mesial and neocortical frontal, temporal and parietal regions. GS have rarely been described as the presenting manifestation of New Onset Refractory Status Epilepticus (NORSE). In this article we describe a previously healthy 3-year-old who presented with an explosive onset of GS that were refractory to multiple anti-seizure medications...
2017: Epilepsy & Behavior Case Reports
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