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Focal AND Cortical AND Dysplasia

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https://www.readbyqxmd.com/read/30539344/evaluation-of-dual-pathology-among-drug-resistant-epileptic-patients-with-hippocampal-sclerosis
#1
Jafar Mehvari Habibabadi, Shervin Badihian, Nasim Tabrizi, Navid Manouchehri, Mohammad Zare, Reza Basiratnia, Majid Barekatain, Houshang Moein, Amirali Mehvari Habibabadi, Payam Moein, Peyman Gookizadeh
PURPOSE: Dual pathology (DP) is defined as simultaneous presence of hippocampal sclerosis (HS) and any other pathology in the brain. Since this is a less probed concept, we aimed to evaluate the frequency and characteristics of DP among drug-resistant epileptic patients with HS. METHODS: This is a cross-sectional study conducted during 2007-2016 in Kashani Comprehensive Epilepsy Center, Isfahan, Iran. Patients with diagnosis of drug-resistant epilepsy and HS were enrolled in the study, and demographic data, seizure semiology, EEG findings, and MRI findings were collected...
December 11, 2018: Neurological Sciences
https://www.readbyqxmd.com/read/30531428/novel-use-of-responsive-neurostimulation-rns-system-in-the-treatment-of-super-refractory-status-epilepticus
#2
Lia D Ernst, Katie L Krause, Marissa A Kellogg, Ahmed M Raslan, David C Spencer
There are very few randomized controlled trials studying treatment of super refractory status epilepticus (SE), despite estimated occurrence in about 15% of SE cases and its association with high morbidity and mortality rates. Small case series and case reports have described use of neurostimulation, including vagal nerve stimulation, transcranial magnetic stimulation, and deep brain stimulation, to treat super refractory SE when medical interventions have failed. To our knowledge, this is the first reported case of responsive neurostimulation being used to successfully treat a case of super refractory SE...
December 6, 2018: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/30531097/-function-of-synaptic-vesicle-protein-2a-sv2a-as-a-novel-therapeutic-target-for-epilepsy
#3
Kentaro Tokudome, Saki Shimizu, Tadao Serikawa, Yukihiro Ohno
Epilepsy is a chronic neurologic disease characterized by recurrent seizures, affecting nearly 1% of the population. Synaptic vesicle protein 2A (SV2A) is a membrane protein specifically expressed in synaptic vesicles and is now implicated in the pathogenesis of epileptic disorders. This is because 1) Sv2a-knockout mice exhibit severe seizures, 2) SV2A serves as a specific binding site for certain antiepileptics (e.g., levetiracetam and its analogues) and 3) the SV2A expression changes under various epileptic conditions both in animals (e...
2018: Nihon Yakurigaku Zasshi. Folia Pharmacologica Japonica
https://www.readbyqxmd.com/read/30530407/rasmussen-syndrome-an-atypical-presentation-in-ten-patients
#4
Roberto H Caraballo, Gabriela Reyes Valenzuela, Juan Pociecha, Juan P Princich, Robinson Gutierrez, Lucas Beltran, Fabiana Lubieniecki, Marcelo Bartuluchi
The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls. The mean and median ages at onset of the seizures were 8.8 and 6.5 years, respectively (range: 4.6-13 years). All of the patients except one had seizures. Eight patients (80%) had epilepsia partialis continua that started at a mean age of 7...
December 10, 2018: Epileptic Disorders: International Epilepsy Journal with Videotape
https://www.readbyqxmd.com/read/30529718/age-dependent-semiology-of-frontal-lobe-seizures
#5
Mandy Hintz, Valentina Krenz, Andreas Schulze-Bonhage
INTRODUCTION: Frontal lobe epilepsy is the second most frequent origin of focal epilepsy. Various studies have discussed localizing aspects of ictal signs in frontal lobe epilepsy; the effect of age on semiological manifestations has, however, not been analyzed so far. MATERIAL AND METHODS: We retrospectively analyzed video-documented semiological signs in a cohort of 122 consecutive patients aged 0-70 years (mean age: 24.9 years) with EEG/MR evidence for frontal lobe epilepsy undergoing video-EEG telemetry assessment between 1999 und 2016...
October 23, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/30522059/long-term-seizure-outcome-in-frontal-lobe-epilepsy-surgery
#6
Roberta Morace, Sara Casciato, Pier Paolo Quarato, Addolorata Mascia, Alfredo D'Aniello, Liliana G Grammaldo, Marco De Risi, Giancarlo Di Gennaro, Vincenzo Esposito
PURPOSE: The purpose of this study was to report long-term seizure outcome in patients who underwent frontal lobe epilepsy (FLE) surgery. METHOD: This retrospective study included 44 consecutive subjects who underwent resective surgery for intractable FLE at IRCCS NEUROMED (period 2001-2014), followed up for at least 2 years (mean: 8.7 years). All patients underwent noninvasive presurgical evaluation and/or invasive electroencephalography (EEG) monitoring when nonconcordant data were obtained or epileptogenic zone was hypothesized to be close to the eloquent cortex...
December 3, 2018: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/30504559/epilepsy-surgery-for-focal-cortical-dysplasia-seizure-and-quality-of-life-qolie-89-outcomes
#7
Jitender Chaturvedi, Malla Bhaskara Rao, A Arivazhagan, Sanjib Sinha, Anita Mahadevan, M Ravindranadh Chowdary, K Raghavendra, A S Shreedhara, Nupur Pruthi, Jitender Saini, Rose Dawn Bharath, Jamuna Rajeswaran, P Satishchandra
Aim: Surgery for drug resistant epilepsy (DRE) with focal cortical dysplasia (FCD) often requires multiple non-invasive as well as invasive pre-surgical evaluations and innovative surgical strategies. There is limited data regarding surgical management of people with FCD as the underlying substrate for DRE among the low and middle-income countries (LAMIC) including India. Methodology: The presurgical evaluation, surgical strategy and outcome of 52 people who underwent resective surgery for DRE with FCD between January 2008 and December 2016 were analyzed...
November 2018: Neurology India
https://www.readbyqxmd.com/read/30504549/focal-cortical-dysplasia
#8
Varindera P Singh
No abstract text is available yet for this article.
November 2018: Neurology India
https://www.readbyqxmd.com/read/30504547/focal-cortical-dysplasias-a-histopathologically-diverse-group-of-developmental-lesions-with-unique-clinicopathological-and-molecular-genetic-aberrations
#9
https://www.readbyqxmd.com/read/30502474/multilobar-resections-for-3t-mri-negative-epilepsy-worth-the-trouble
#10
Evangelos Kogias, Barbara Schmeiser, Soroush Doostkam, Armin Brandt, Thilo Hammen, Josef Zentner, Georgia Ramantani
OBJECTIVE: Multilobar resections for MRI-negative drug-resistant epilepsy warrant attention since they account for up to one-third of MRI-negative epilepsy surgery. Despite their high relevance, data is sparse, and the risk-benefit ratio is yet under debate. This study aimed to investigate the postsurgical seizure outcomes in this particularly challenging subgroup. METHODS: We retrospectively analyzed the data of four consecutive 3T MRI-negative patients with drug-resistant focal epilepsy who underwent multilobar epilepsy surgery in our institution...
November 28, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/30502054/gsk3%C3%AE-activity-alleviates-epileptogenesis-and-limits-glua1-phosphorylation
#11
Malgorzata Urbanska, Paulina Kazmierska-Grebowska, Tomasz Kowalczyk, Bartosz Caban, Karolina Nader, Barbara Pijet, Katarzyna Kalita, Agata Gozdz, Herman Devijver, Benoit Lechat, Tomasz Jaworski, Wieslawa Grajkowska, Krzysztof Sadowski, Sergiusz Jozwiak, Katarzyna Kotulska, Jan Konopacki, Fred Van Leuven, Erwin A van Vliet, Eleonora Aronica, Jacek Jaworski
BACKGROUND: Glycogen synthase kinase-3β (GSK3β) is a key regulator of cellular homeostasis. In neurons, GSK3β contributes to the control of neuronal transmission and plasticity, but its role in epilepsy remains to be defined. METHODS: Biochemical and electrophysiological methods were used to assess the role of GSK3β in regulating neuronal transmission and epileptogenesis. GSK3β activity was increased genetically in GSK3β[S9A] mice. Its effects on neuronal transmission and epileptogenesis induced by kainic acid were assessed by field potential recordings in mice brain slices and video electroencephalography in vivo...
November 27, 2018: EBioMedicine
https://www.readbyqxmd.com/read/30476126/severe-tubb4a-related-hypomyelination-with-atrophy-of-the-basal-ganglia-and-cerebellum-novel-neuropathological-findings
#12
Kristina M Joyal, Jean Michaud, Marjo S van der Knaap, Marianna Bugiani, Sunita Venkateswaran
Hypomyelination with atrophy of the basal ganglia and cerebellum (H-ABC) is a rare hypomyelinating leukodystrophy characterized by infantile or childhood onset of motor developmental delay, progressive rigidity and spasticity, with hypomyelination and progressive atrophy of the basal ganglia and cerebellum due to a genetic mutation of the TUBB4A gene. It has only been recognized since 2002 and the full spectrum of the disorder is still being delineated. Here, we review a case report of a severely affected girl with a thorough neuropathological evaluation demonstrating novel clinical and pathological findings...
November 22, 2018: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/30473064/long-term-follow-up-in-children-with-focal-cortical-dysplasia-iiid-for-early-brain-injuries-including-neuropathological-findings
#13
Li Yan, Piao Yue-Shan, Li Yunlin, Liu Yongling, Wang Zhichao, He Yuandong
BACKGROUND: Early cerebral injury has a close relationship with epilepsy and focal cortical dysplasia Ⅲd. We investigated children with focal cortical dysplasia Ⅲd who underwent surgery for epilepsy. METHODS: We selected 49 patients from among 260 pediatric patients who had undergone epilepsy surgery, analyzing their clinical materials and pathology data. The selected patients had been followed for more than two years. RESULTS: The 49 patients were divided into seven groups based on different early brain injuries...
November 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/30472578/single-stage-epilepsy-surgery-in-children-and-adolescents-with-focal-cortical-dysplasia-type-ii-prognostic-value-of-the-intraoperative-electrocorticogram
#14
Gudrun Gröppel, Christian Dorfer, Sharon Samueli, Anastasia Dressler, Angelika Mühlebner, Daniela Prayer, Thomas Czech, Martha Feucht
OBJECTIVE: To evaluate prospectively the informative/prognostic value of epileptic discharges in the post-resection ECoGs of children with drug-resistant epilepsies and Focal Cortical Dysplasia type II (FCD-II). METHODS: Included were consecutive patients with focal epilepsies and suspected FCD-II who were planned for single-stage epilepsy surgery based on non-invasive presurgical evaluation results. Intraoperative ECoGs were recorded using a 32-channel system with strip- and/or grid-electrodes...
November 14, 2018: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/30461353/discrimination-of-epileptogenic-lesions-and-perilesional-white-matter-using-diffusion-tensor-magnetic-resonance-imaging
#15
Alexander Rau, Elias Kellner, Niels A Foit, Niklas Lützen, Dieter H Heiland, Andreas Schulze-Bonhage, Marco Reisert, Valerij G Kiselev, Marco Prinz, Horst Urbach, Irina Mader
The aim of this study was to evaluate whether ganglioglioma (GGL), dysembryoplastic neuroepithelial tumour (DNET) and FCD (focal cortical dysplasia) are distinguishable through diffusion tensor imaging. Additionally, it was investigated whether the diffusion measures differed in the perilesional (pNAWM) and in the contralateral normal appearing white matter (cNAWM). Six GGLs, eight DNETs and seven FCDs were included in this study. Quantitative diffusion measures, that is, axial, radial and mean diffusivity and fractional anisotropy, were determined in the lesion identified on isotropic T2 or FLAIR-weighted images and in pNAWM and cNAWM, respectively...
November 21, 2018: Neuroradiology Journal
https://www.readbyqxmd.com/read/30443667/ulnar-focal-cortical-indentation-a-progressive-deforming-variant-of-focal-fibrocartilaginous-dysplasia
#16
Alyssa R Goldbach, Dan A Zlotolow, Sarah D Fenerty, Jordan Rapp, Grigory Gershkovich, Sayed Ali
BACKGROUND: Focal fibrocartilaginous dysplasia is a rare growth disturbance of bone resulting in deformity. In the ulna, focal fibrocartilaginous dysplasia is particularly rare, and the characteristic fibroligamentous tether can result in progressive deformity with progressive dislocation of the radial head. The fibroligamentous tether is similar in appearance and function to the Vickers ligament seen in Madelung deformity. The imaging features of ulnar focal fibrocartilaginous dysplasia include a unilateral angular deformity in the ulna with a radiolucent cortical defect and surrounding sclerosis, with secondary radial bowing or radial head dislocation...
November 15, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/30442696/mri-magnetoencephalography-and-surgical-outcome-of-oligodendrocytosis-versus-focal-cortical-dysplasia-type-i
#17
D Mata-Mbemba, Y Iimura, L-N Hazrati, A Ochi, H Otsubo, O C Snead, J Rutka, E Widjaja
BACKGROUND AND PURPOSE: Abnormalities of oligodendrocytes have been reported in surgical specimens of patients with medically intractable epilepsy. The aim of this study was to compare the MR imaging, magnetoencephalography, and surgical outcome of children with oligodendrocytosis relative to focal cortical dysplasia I. MATERIALS AND METHODS: Oligodendrocytosis included oligodendroglial hyperplasia, oligodendrogliosis, and oligodendroglial-like cells in the white matter, gray matter, or both from children with medically intractable epilepsy...
November 15, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/30427554/homozygous-splicing-mutation-in-nup133-causes-galloway-mowat-syndrome
#18
Atsushi Fujita, Hiroyasu Tsukaguchi, Eriko Koshimizu, Hitoshi Nakazato, Kyoko Itoh, Shohei Kuraoka, Yoshihiro Komohara, Masaaki Shiina, Shohei Nakamura, Mika Kitajima, Yoshinori Tsurusaki, Satoko Miyatake, Kazuhiro Ogata, Kazumoto Iijima, Naomichi Matsumoto, Noriko Miyake
OBJECTIVE: Galloway-Mowat syndrome (GAMOS) is a neural and renal disorder, characterized by microcephaly, brain anomalies, and early-onset nephrotic syndrome. Biallelic mutations in WDR73 and the four subunit genes of the KEOPS complex are reported to cause GAMOS. Furthermore, an identical homozygous NUP107 (nucleoporin 107 kDa) mutation was identified in four GAMOS-like families, although biallelic NUP107 mutations were originally identified in steroid-resistant nephrotic syndrome. NUP107 and NUP133 (nucleoporin 133 kDa) are interacting subunits of the nuclear pore complex in the nuclear envelope during interphase, and these proteins are also involved in centrosome positioning and spindle assembly during mitosis...
November 14, 2018: Annals of Neurology
https://www.readbyqxmd.com/read/30426477/the-repertoire-of-seizure-onset-patterns-in-human-focal-epilepsies-determinants-and-prognostic-values
#19
Stanislas Lagarde, Sinziana Buzori, Agnès Trebuchon, Romain Carron, Didier Scavarda, Mathieu Milh, Aileen McGonigal, Fabrice Bartolomei
OBJECTIVE: In this study, we seek to analyze the determinants of the intracranial electroencephalography seizure onset pattern (SOP) and the impact of the SOP in predicting postsurgical seizure outcome. METHODS: To this end, we analyzed 820 seizures from 252 consecutive patients explored by stereo-electroencephalography (total of 2148 electrodes), including various forms of focal refractory epilepsies. We used a reproducible method combining visual and time-frequency analyses...
November 13, 2018: Epilepsia
https://www.readbyqxmd.com/read/30384114/invasive-monitoring-after-resection-of-epileptogenic-neocortical-lesions-in-multistaged-epilepsy-surgery-in-children
#20
Eveline Teresa Hidalgo, Hyman Gregory Frankel, Crystalann Rodriguez, Cordelia Orillac, Sophie Phillips, Neel Patel, Orrin Devinsky, Daniel Friedman, Howard L Weiner
OBJECTIVE: Incomplete resection of neocortical epileptogenic foci correlates with failed epilepsy surgery in children. We often treat patients with neocortical epilepsy with a staged approach using invasive monitoring to localize the focus, resect the seizure onset zone, and, in select cases, post-resection invasive monitoring (PRM). We report the technique and the outcomes of children treated with staged surgery including PRM. METHODS: We retrospectively reviewed the charts of pediatric patients with neocortical epilepsy who underwent resective surgery with PRM...
December 2018: Epilepsy Research
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