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Hippocampal sclerosis

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https://www.readbyqxmd.com/read/29451027/a-case-of-late-onset-ocd-developing-pls-and-ftd
#1
Enrica Bersano, Maria Francesca Sarnelli, Valentina Solara, Fabiola De Marchi, Gian Mauro Sacchetti, Alessandro Stecco, Lucia Corrado, Sandra D'alfonso, Roberto Cantello, Letizia Mazzini
We describe a 64-year-old woman, suffering from late-onset obsessive-compulsive disorder (OCD) from the age of 57, who developed dysarthria and dysphagia, spastic diplegic, and proximal muscles weakness. Needle electromyography showed no active denervation. Neuropsychological evaluation showed intact cognitive functioning. We diagnosed upper motor neuron disease (MND), with no known genetic correlates. Brain magnetic resonance (MRI) detected bilateral hippocampal atrophy with sclerosis of right hippocampus...
February 16, 2018: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/29449490/dentate-gyrus-mossy-cells-control-spontaneous-convulsive-seizures-and-spatial-memory
#2
Anh D Bui, Theresa M Nguyen, Charles Limouse, Hannah K Kim, Gergely G Szabo, Sylwia Felong, Mattia Maroso, Ivan Soltesz
Temporal lobe epilepsy (TLE) is characterized by debilitating, recurring seizures and an increased risk for cognitive deficits. Mossy cells (MCs) are key neurons in the hippocampal excitatory circuit, and the partial loss of MCs is a major hallmark of TLE. We investigated how MCs contribute to spontaneous ictal activity and to spatial contextual memory in a mouse model of TLE with hippocampal sclerosis, using a combination of optogenetic, electrophysiological, and behavioral approaches. In chronically epileptic mice, real-time optogenetic modulation of MCs during spontaneous hippocampal seizures controlled the progression of activity from an electrographic to convulsive seizure...
February 16, 2018: Science
https://www.readbyqxmd.com/read/29433734/foxo-in-neural-cells-and-diseases-of-the-nervous-system
#3
Evan E Santo, Jihye Paik
The evolutionarily conserved FOXO family of transcription factors has emerged as a significant arbiter of neural cell fate and function in mammals. From the neural stem cell (NSC) state through mature neurons under both physiological and pathological conditions, they have been found to modulate neural cell survival, stress responses, lineage commitment, and neuronal signaling. Lineage-specific FOXO knockout mice have provided an invaluable tool for the dissection of FOXO biology in the nervous system. Within the NSC compartments of the brain, FOXOs are required for the maintenance of NSC quiescence and for the clearance of reactive oxygen species...
2018: Current Topics in Developmental Biology
https://www.readbyqxmd.com/read/29432809/seizure-progression-and-inflammatory-mediators-promote-pericytosis-and-pericyte-microglia-clustering-at-the-cerebrovasculature
#4
Wendy Klement, Rita Garbelli, Emma Zub, Laura Rossini, Laura Tassi, Benoit Girard, Marine Blaquiere, Federica Bertaso, Julie Perroy, Frederic de Bock, Nicola Marchi
BACKGROUND: Cerebrovascular dysfunction and inflammation occur in experimental and clinical epilepsy. Here we asked whether pericytes, a pivotal cellular component of brain capillaries, undergo pathological modifications during experimental epileptogenesis and in human epilepsy. We evaluated whether pro-inflammatory cytokines, present in the brain during seizures, contribute to pericyte morphological modifications. METHODS: In vivo, unilateral intra-hippocampal kainic acid (KA) injections were performed in NG2DsRed/C57BL6 mice to induce status epilepticus (SE), epileptogenesis, and spontaneous recurrent seizures (SRS)...
February 9, 2018: Neurobiology of Disease
https://www.readbyqxmd.com/read/29424649/letter-to-the-editor-focal-cortical-dysplasia-type-iiia-and-isolated-hippocampal-sclerosis
#5
Marco Giulioni, Gianfranco Vornetti, Gianluca Marucci
No abstract text is available yet for this article.
February 9, 2018: Journal of Neurosurgery
https://www.readbyqxmd.com/read/29409838/deciphering-the-microstructure-of-hippocampal-subfields-with-in-vivo-dti-and-noddi-applications-to-experimental-multiple-sclerosis
#6
Amandine Crombe, Vincent Planche, Gerard Raffard, Julien Bourel, Nadège Dubourdieu, Aude Panatier, Hikaru Fukutomi, Vincent Dousset, Stephane Oliet, Bassem Hiba, Thomas Tourdias
The hippocampus contains distinct populations of neurons organized into separate anatomical subfields and layers with differential vulnerability to pathological mechanisms. The ability of in vivo neuroimaging to pinpoint regional vulnerability is especially important for better understanding of hippocampal pathology at the early stage of neurodegenerative disorders and for monitoring future therapeutic strategies. This is the case for instance in multiple sclerosis whose neurodegenerative component can affect the hippocampus from the early stage...
January 31, 2018: NeuroImage
https://www.readbyqxmd.com/read/29398181/utility-of-additional-dedicated-high-resolution-3t-mri-in-children-with-medically-refractory-focal-epilepsy
#7
Raheel Ahmed, Luc Rubinger, Cristina Go, James M Drake, James T Rutka, O Carter Snead, Elysa Widjaja
PURPOSE: In patients with medically refractory epilepsy and normal magnetic resonance imaging (MRI), high-resolution dedicated MRI may identify cryptic lesions. The aim of this study was to assess improvement in lesion detection and its impact on clinical management, using additional high-resolution dedicated 3T MRI in children with medically refractory epilepsy who had normal 3T epilepsy protocol MRI. MATERIALS AND METHODS: Children who had resective epilepsy surgery and suspected focal cortical dysplasia (FCD) or normal 3T epilepsy protocol MRI were included...
February 2, 2018: Epilepsy Research
https://www.readbyqxmd.com/read/29392715/laser-ablation-is-effective-for-temporal-lobe-epilepsy-with-and-without-mesial-temporal-sclerosis-if-hippocampal-seizure-onsets-are-localized-by-stereoelectroencephalography
#8
Brett E Youngerman, Justin Y Oh, Deepti Anbarasan, Santoshi Billakota, Camilla H Casadei, Emily K Corrigan, Garret P Banks, Alison M Pack, Hyunmi Choi, Carl W Bazil, Shraddha Srinivasan, Lisa M Bateman, Catherine A Schevon, Neil A Feldstein, Sameer A Sheth, Guy M McKhann
OBJECTIVE: Selective laser amygdalohippocampotomy (SLAH) using magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) is emerging as a treatment option for drug-resistant mesial temporal lobe epilepsy (MTLE). SLAH is less invasive than open resection, but there are limited series reporting its safety and efficacy, particularly in patients without clear evidence of mesial temporal sclerosis (MTS). METHODS: We report seizure outcomes and complications in our first 30 patients who underwent SLAH for drug-resistant MTLE between January 2013 and December 2016...
February 2, 2018: Epilepsia
https://www.readbyqxmd.com/read/29384235/mir147b-a-novel-key-regulator-of-interleukin-1-beta-mediated-inflammation-in-human-astrocytes
#9
Jackelien van Scheppingen, James D Mills, Till S Zimmer, Diede W M Broekaart, Valentina Iori, Anika Bongaarts, Jasper J Anink, Anand M Iyer, Anatoly Korotkov, Floor E Jansen, Wim van Hecke, Wim G Spliet, Peter C van Rijen, Johannes C Baayen, Annamaria Vezzani, Erwin A van Vliet, Eleonora Aronica
Astrocytes are important mediators of inflammatory processes in the brain and seem to play an important role in several neurological disorders, including epilepsy. Recent studies show that astrocytes produce several microRNAs, which may function as crucial regulators of inflammatory pathways and could be used as therapeutic target. We aim to study which miRNAs are produced by astrocytes during IL-1β mediated inflammatory conditions in vitro, as well as their functional role and to validate these findings in human epileptogenic brain tissue...
January 31, 2018: Glia
https://www.readbyqxmd.com/read/29380527/the-sphingosine-1-phosphate-analogue-fty720-alleviates-seizure-induced-over-expression-of-p-glycoprotein-in-rat-hippocampus
#10
Fei Gao, Ying Gao, Fangling Meng, Chunmei Yang, Jiangfeng Fu, Yajun Li
Over-expression of P-glycoprotein (P-gp) in the brain is an important factor leading to drug-resistant epilepsy. Clinical use of P-gp inhibitors is limited by their systemic toxicity. In the present study, we tested the hypothesis that FTY720, a sphingosine 1-phosphate (S1P) analogue used for treating multiple sclerosis, modulates the up-regulation of P-gp and improves brain delivery of phenytoin (PHT) through S1P receptor 1 in the hippocampus of a pilocarpine-induced rat model of status epilepticus (SE). We administered vehicle, FTY720 or FTY720 + W146 (an S1P receptor 1 antagonist) to SE rats...
January 30, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29374846/structural-and-functional-papez-circuit-integrity-in-amyotrophic-lateral-sclerosis
#11
Ana Paula Arantes Bueno, Walter Hugo L Pinaya, Luciana M Moura, Maxime Bertoux, Ratko Radakovic, Matthew C Kiernan, Antonio Lucio Teixeira, Leonardo Cruz de Souza, Michael Hornberger, João Ricardo Sato
Cognitive impairment in amyotrophic lateral sclerosis (ALS) is heterogeneous but now recognized as a feature in non-demented patients and no longer exclusively attributed to executive dysfunction. However, despite common reports of temporal lobe changes and memory deficits in ALS, episodic memory has been less explored. In the current study, we examined how the Papez circuit-a circuit known to participate in memory processes-is structurally and functionally affected in ALS patients (n = 20) compared with healthy controls (n = 15), and whether these changes correlated with a commonly used clinical measure of episodic memory...
January 27, 2018: Brain Imaging and Behavior
https://www.readbyqxmd.com/read/29370838/extended-ftld-pedigree-segregating-a-belgian-grn-null-mutation-neuropathological-heterogeneity-in-one-family
#12
Anne Sieben, Sara Van Mossevelde, Eline Wauters, Sebastiaan Engelborghs, Julie van der Zee, Tim Van Langenhove, Patrick Santens, Marleen Praet, Paul Boon, Marijke Miatton, Sofie Van Hoecke, Mathieu Vandenbulcke, Rik Vandenberghe, Patrick Cras, Marc Cruts, Peter Paul De Deyn, Christine Van Broeckhoven, Jean-Jacques Martin
BACKGROUND: In this paper, we describe the clinical and neuropathological findings of nine members of the Belgian progranulin gene (GRN) founder family. In this family, the loss-of-function mutation IVS1 + 5G > C was identified in 2006. In 2007, a clinical description of the mutation carriers was published that revealed the clinical heterogeneity among IVS1 + 5G > C carriers. We report our comparison of our data with the published clinical and neuropathological characteristics of other GRN mutations as well as other frontotemporal lobar degeneration (FTLD) syndromes, and we present a review of the literature...
January 22, 2018: Alzheimer's Research & Therapy
https://www.readbyqxmd.com/read/29365066/structural-brain-abnormalities-in-the-common-epilepsies-assessed-in-a-worldwide-enigma-study
#13
Christopher D Whelan, Andre Altmann, Juan A Botía, Neda Jahanshad, Derrek P Hibar, Julie Absil, Saud Alhusaini, Marina K M Alvim, Pia Auvinen, Emanuele Bartolini, Felipe P G Bergo, Tauana Bernardes, Karen Blackmon, Barbara Braga, Maria Eugenia Caligiuri, Anna Calvo, Sarah J Carr, Jian Chen, Shuai Chen, Andrea Cherubini, Philippe David, Martin Domin, Sonya Foley, Wendy França, Gerrit Haaker, Dmitry Isaev, Simon S Keller, Raviteja Kotikalapudi, Magdalena A Kowalczyk, Ruben Kuzniecky, Soenke Langner, Matteo Lenge, Kelly M Leyden, Min Liu, Richard Q Loi, Pascal Martin, Mario Mascalchi, Marcia E Morita, Jose C Pariente, Raul Rodríguez-Cruces, Christian Rummel, Taavi Saavalainen, Mira K Semmelroch, Mariasavina Severino, Rhys H Thomas, Manuela Tondelli, Domenico Tortora, Anna Elisabetta Vaudano, Lucy Vivash, Felix von Podewils, Jan Wagner, Bernd Weber, Yi Yao, Clarissa L Yasuda, Guohao Zhang, Nuria Bargalló, Benjamin Bender, Neda Bernasconi, Andrea Bernasconi, Boris C Bernhardt, Ingmar Blümcke, Chad Carlson, Gianpiero L Cavalleri, Fernando Cendes, Luis Concha, Norman Delanty, Chantal Depondt, Orrin Devinsky, Colin P Doherty, Niels K Focke, Antonio Gambardella, Renzo Guerrini, Khalid Hamandi, Graeme D Jackson, Reetta Kälviäinen, Peter Kochunov, Patrick Kwan, Angelo Labate, Carrie R McDonald, Stefano Meletti, Terence J O'Brien, Sebastien Ourselin, Mark P Richardson, Pasquale Striano, Thomas Thesen, Roland Wiest, Junsong Zhang, Annamaria Vezzani, Mina Ryten, Paul M Thompson, Sanjay M Sisodiya
Progressive functional decline in the epilepsies is largely unexplained. We formed the ENIGMA-Epilepsy consortium to understand factors that influence brain measures in epilepsy, pooling data from 24 research centres in 14 countries across Europe, North and South America, Asia, and Australia. Structural brain measures were extracted from MRI brain scans across 2149 individuals with epilepsy, divided into four epilepsy subgroups including idiopathic generalized epilepsies (n =367), mesial temporal lobe epilepsies with hippocampal sclerosis (MTLE; left, n = 415; right, n = 339), and all other epilepsies in aggregate (n = 1026), and compared to 1727 matched healthy controls...
January 22, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29360612/intracranial-electrodes-monitoring-improves-seizure-control-and-complication-outcomes-for-patients-with-temporal-lobe-epilepsy-a-retrospective-cohort-study
#14
Yan-En Lyu, Xiao-Fei Xu, Shuang Dai, Xiao-Bo Dong, Shao-Ping Shen, Yao Wang, Chong Liu
BACKGROUND: Anterior temporal lobectomy (ATL) is the standard surgical treatment for temporal lobe epilepsy (TLE), but patients may suffer from recurrent seizures post-surgery. Invasive electrical monitoring plays a critical role in precisely identifying the epileptic foci. This study aimed to evaluate and compare the benefits of long-term invasive electroencephalography (EEG) monitoring and two-stage surgery with the classical approach to examine their effect on post-surgical brain function and complications...
January 19, 2018: International Journal of Surgery
https://www.readbyqxmd.com/read/29359399/the-international-consensus-classification-of-focal-cortical-dysplasia-a-critical-update-2018
#15
Imad Najm, Harvey B Sarnat, Ingmar Blümcke
The Diagnostic Methods commission of the International League against Epilepsy (ILAE) released a first international consensus classification of Focal Cortical Dysplasia (FCD) in 2011. Since that time, this FCD classification has been widely used in clinical diagnosis and research (more than 740 papers cited in Pubmed between 1/1/2012 and 7/1/2017). Herein, we review the new data that will inform and revise the FCD classification. Many recent papers described molecular-genetic characteristics in FCD Type II including multiple mutations in the mTOR pathway...
January 23, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29353399/mri-findings-in-glutamic-acid-decarboxylase-associated-autoimmune-epilepsy
#16
Jason R Fredriksen, Carrie M Carr, Kelly K Koeller, Jared T Verdoorn, Avi Gadoth, Sean J Pittock, Amy L Kotsenas
PURPOSE: Glutamic acid decarboxylase (GAD65) has been implicated in a number of autoimmune-associated neurologic syndromes, including autoimmune epilepsy. This study categorizes the spectrum of MRI findings in patients with a clinical diagnosis of autoimmune epilepsy and elevated serum GAD65 autoantibodies. METHODS: An institutional database search identified patients with elevated serum GAD65 antibodies and a clinical diagnosis of autoimmune epilepsy who had undergone brain MRI...
January 20, 2018: Neuroradiology
https://www.readbyqxmd.com/read/29341105/metabolomic-characterization-of-human-hippocampus-from-drug-resistant-epilepsy-with-mesial-temporal-seizure
#17
Julien Detour, Caroline Bund, Charles Behr, Hélène Cebula, Ercument A Cicek, Maria-Paola Valenti-Hirsch, Béatrice Lannes, Benoît Lhermitte, Astrid Nehlig, Pierre Kehrli, François Proust, Edouard Hirsch, Izzie-Jacques Namer
OBJECTIVE: Within a complex systems biology perspective, we wished to assess whether hippocampi with established neuropathological features have distinct metabolome. Apparently normal hippocampi with no signs of sclerosis (noHS), were compared to hippocampal sclerosis (HS) type 1 (HS1) and/or type 2 (HS2). Hippocampus metabolome from patients with epilepsy-associated neuroepithelial tumors (EANTs), namely, gangliogliomas (GGs) and dysembryoplastic neuroepithelial tumors (DNTs), was also compared to noHS epileptiform tissue...
January 17, 2018: Epilepsia
https://www.readbyqxmd.com/read/29339765/mutations-in-bassoon-in-individuals-with-familial-and-sporadic-progressive-supranuclear-palsy-like-syndrome
#18
Ichiro Yabe, Hiroaki Yaguchi, Yasutaka Kato, Yasuo Miki, Hidehisa Takahashi, Satoshi Tanikawa, Shinichi Shirai, Ikuko Takahashi, Mari Kimura, Yuka Hama, Masaaki Matsushima, Shinsuke Fujioka, Takahiro Kano, Masashi Watanabe, Shin Nakagawa, Yasuyuki Kunieda, Yoshio Ikeda, Masato Hasegawa, Hiroshi Nishihara, Toshihisa Ohtsuka, Shinya Tanaka, Yoshio Tsuboi, Shigetsugu Hatakeyama, Koichi Wakabayashi, Hidenao Sasaki
Clinical diagnosis of progressive supranuclear palsy (PSP) is sometimes difficult because various phenotypes have been identified. Here, we report a mutation in the bassoon (BSN) gene in a family with PSP-like syndrome. Their clinical features resembled not only those of PSP patients but also those of individuals with multiple system atrophy and Alzheimer's disease. The neuropathological findings showed a novel three + four repeat tauopathy with pallido-luysio-nigral degeneration and hippocampal sclerosis...
January 16, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29331060/regional-hippocampal-vulnerability-in-early-multiple-sclerosis-dynamic-pathological-spreading-from-dentate-gyrus-to-ca1
#19
Vincent Planche, Ismail Koubiyr, José E Romero, José V Manjon, Pierrick Coupé, Mathilde Deloire, Vincent Dousset, Bruno Brochet, Aurélie Ruet, Thomas Tourdias
BACKGROUND: Whether hippocampal subfields are differentially vulnerable at the earliest stages of multiple sclerosis (MS) and how this impacts memory performance is a current topic of debate. METHOD: We prospectively included 56 persons with clinically isolated syndrome (CIS) suggestive of MS in a 1-year longitudinal study, together with 55 matched healthy controls at baseline. Participants were tested for memory performance and scanned with 3 T MRI to assess the volume of 5 distinct hippocampal subfields using automatic segmentation techniques...
January 13, 2018: Human Brain Mapping
https://www.readbyqxmd.com/read/29321710/cytokine-polymorphism-and-hla-genotyping-in-patients-with-temporal-lobe-epilepsy-related-to-hippocampal-sclerosis
#20
Ayşe Altintaş, Çiğdem Özkara, Melis Sohtaoğlu Sevindik, Mustafa Uzan, Çiğdem Kekik Çinar, Ömer Uysal, Fatma Savran Oğuz
Objective: Hippocampal sclerosis (HS) is the most common pathological substrate associated with mesial temporal lobe epilepsy (MTLE), where inflammatory processes are known to play an increasingly important role in the pathogenesis. To further investigate the role of the immune system, both cytokine gene polymorphisms and human leukocyte antigen (HLA) genotyping in patients with MTLE-HS were investigated. Methods: The DNA samples of 100 patients with MTLE-HS and 201 healthy individuals were genotyped for cytokines (IL-6,IL-10, TNF-α, TGF-β1 and IFN-γ) and HLA using polymerase chain reaction (PCR)-SSP and SSO methods...
December 2017: Noro Psikiyatri Arsivi
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