CLINICAL AND GENETIC ASPECTS OF EPILEPSY Edited by Zaid Afawi Clinical and Genetic Aspects of Epilepsy Edited by Zaid Afawi Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2011 InTech All chapters are Open Access articles distributed under the Creative Commons Non Commercial Share Alike Attribution 3.0 license, which permits to copy, distribute, transmit, and adapt the work in any medium, so long as the original work is properly cited. After this work has been published by InTech, authors have the right to republish it, in whole or part, in any publication of which they are the author, and to make other personal use of the work. Any republication, referencing or personal use of the work must explicitly identify the original source. Statements and opinions expressed in the chapters are these of the individual contributors and not necessarily those of the editors or publisher. No responsibility is accepted for the accuracy of information contained in the published articles. The publisher assumes no responsibility for any damage or injury to persons or property arising out of the use of any materials, instructions, methods or ideas contained in the book. Publishing Process Manager Dragana Manestar Technical Editor Teodora Smiljanic Cover Designer Jan Hyrat Image Copyright yalayama, 2011. Used under license from Shutterstock.com First published August, 2011 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechweb.org Clinical and Genetic Aspects of Epilepsy, Edited by Zaid Afawi p. cm. ISBN 978-953-307-700-0 free online editions of InTech Books and Journals can be found at www.intechopen.com Contents Preface IX Part 1 Mechanisms Underlying Epileptic Seizures 1 Chapter 1 A Functional Role for Microglia in Epilepsy 3 Martine M. Mirrione and Stella E. Tsirka Chapter 2 The Blood-Brain Barrier in Epilepsy 23 Björn Bauer, Juli Schlichtiger, Anton Pekcec and Anika M.S. Hartz Chapter 3 New Tools for Understanding Epilepsy 55 Fatima Shad Kaneez and Faisal Khan Chapter 4 Epilepsy: Selenium and Aging 75 Caroline Rocourt, Ying Yu and Wen-Hsing Cheng Part 2 Molecular Genetics of Epilepsy 93 Chapter 5 The Molecular Genetics of the Benign Epilepsies of Infancy 95 Sarah E. Heron and John C. Mulley Part 3 Animal Models 113 Chapter 6 Audiogenic Seizures - Biological Phenomenon and Experimental Model of Human Epilepsies 115 Inga I. Poletaeva, Irina B. Fedotova, Natalia M. Sourina and Zoya A. Kostina Part 4 Ion and Channels 149 Chapter 7 Ionic Imbalance 151 John Robert Cressman, Christine Drown and Monica Gertz VI Contents Part 5 New Treatments 173 Chapter 8 Antiepileptic Medicinal Plants used in Traditional Medicine to Treat Epilepsy 175 E. Ngo Bum, G.S. Taiwe, F.C.O. Moto, G.T. Ngoupaye, R.R.N. Vougat, V.D. Sakoue, C. Gwa, E.R. Ayissi, C. Dong, A. Rakotonirina and S.V. Rakotonirina Chapter 9 The Potential Role of ATP-sensitive Potassium Channels in Treating Epileptic Disorders 193 Chin-Wei Huang Preface This book on Epilepsy was conceived and produced as a source of information on wide range of issues in epilepsy. We hope that it will help health care providers in daily practices and increase their understanding on diagnosis and treatment of epilepsies. The book was designed as an update for neuroscientists who are interested in epilepsy, primary care physicians and students in health care professions. This epilepsy book is the result of a collaborative effort of investigators who have used a wide range of experimental preparations and recording techniques. Authors from a variety of backgrounds have contributed significantly with papers from their respective fields. I believe they have provided a comprehensive description of key issues and important developments within each filed of research. The studies included in this book are drawn from several disciplines of modern neuroscience and include various Chapters distributed in sections on Mechanisms Underlying Epileptic Seizures, Molecular Genetics of Epilepsy, Animal Models, Ion and Channels and New Treatments Zaid Afawi Tel-Aviv Medical Center Tel-Aviv, Israel [...]... hyperactivation and release of toxic factors, such as nitric oxide (NO), 4 Clinical and Genetic Aspects of Epilepsy leading to over production of excess peroxynitrite, and reactive oxygen and nitrogen species (ROS, RNS), may result in an unmanageable level of oxidative stress causing degeneration in nearby ‘bystander’ cells Oxidative stress can cause further neuroinflammation as well by recruitment of peripheral... camera Other helpful properties of these radiotracers (radioligands) are that they have low nonspecific binding (that they do not bind randomly to other molecules), and are they are not converted into ‘active’ metabolites, which can bind to some other unwanted target in the brain and perturb the analysis 12 Clinical and Genetic Aspects of Epilepsy The first major radioligand that has been used to study... suggesting that neuronal degeneration continues to occur as a result of 6 Clinical and Genetic Aspects of Epilepsy ongoing seizure activity (Beach et al., 1995) A recent study confirmed the presence both of activated microglial and also immunoreactive leukocytes in tissue resected from patents with intractable medial temporal lobe epilepsy, and in kainic acid (KA) treated mice, either associated with blood... increases and HSVTK is highly expressed In a cell which has taken up GCV, and is expressing HSVTK, GCV is phosphorylated by 10 Clinical and Genetic Aspects of Epilepsy HSVTK and endogenous kinases (phosphorylation represented by red circle’s labeled ‘P’) This turns GCV into a toxic triphosphate which competes with endogenous thymine for DNA synthesis Ultimately, DNA replication is disrupted and the cell... edema, and the numbers of activated microglia after febrile seizures, thereby reducing the risk of subsequent spontaneous seizures (Jung et al., 2011) Another report showed that levetiracetam reduced the harmful spread of excitation during seizures by restoring gap junctions in astrocytes co-cultured with microglia (Stienen et al., 2011) The investigators 14 Clinical and Genetic Aspects of Epilepsy. .. JP, Matys T, Skrzypiec AE, Strickland S (2005) Ethanol-withdrawal seizures are controlled by tissue plasminogen activator via modulation of NR2Bcontaining NMDA receptors Proc Natl Acad Sci U S A 102:443-448 20 Clinical and Genetic Aspects of Epilepsy Persson M, Sandberg M, Hansson E, Ronnback L (2006) Microglial glutamate uptake is coupled to glutathione synthesis and glutamate release Eur J Neurosci... cells and seal the intercellular space (Brightman & Reese, 1969; Reese & Karnovsky, 1967) With this, the molecular structure responsible for barrier function was identified and the barrier was localized to the brain capillary endothelium 24 Clinical and Genetic Aspects of Epilepsy 1885 1900 1938 1967 1975 1989 1995 2010 Present Fig 1 Evolution of Blood-Brain Barrier Methodology/History The History of. .. the context of seizures and potentially exploited as neuroprotective modulators 2 A functional role for microglia in seizures: The ‘bad’ and the ‘good’ A growing body of evidence suggests that microglia have a functional role in the pathology and symptoms of CNS diseases including ischemia and Alzheimer’s disease In this section, we discuss the evidence linking microglia to seizures and epilepsy We... 72:652-660 Werhahn KJ, Landvogt C, Klimpe S, Buchholz HG, Yakushev I, Siessmeier T, Muller-Forell W, Piel M, Rosch F, Glaser M, Schreckenberger M, Bartenstein P (2006) Decreased 22 Clinical and Genetic Aspects of Epilepsy dopamine D2/D3-receptor binding in temporal lobe epilepsy: an [18F]fallypride PET study Epilepsia 47:1392-1396 Wine RN, McPherson CA, Harry GJ (2009) IGF-1 and pAKT signaling promote... expression of two phospholipase D isoforms during experimentally induced hippocampal mossy fiber outgrowth Glia 46:74-83 2 The Blood-Brain Barrier in Epilepsy Björn Bauer1,2, Juli Schlichtiger3, Anton Pekcec4 and Anika M.S Hartz1,2 1University of Minnesota, College of Pharmacy Barriers Research Center, University of Minnesota, College of Pharmacy 3Ludwig-Maximilians University, Department of Nuclear . CLINICAL AND GENETIC ASPECTS OF EPILEPSY Edited by Zaid Afawi Clinical and Genetic Aspects of Epilepsy Edited by Zaid Afawi . hyperactivation and release of toxic factors, such as nitric oxide (NO), Clinical and Genetic Aspects of Epilepsy 4 leading to over production of excess peroxynitrite, and reactive oxygen and nitrogen. Understanding Epilepsy 55 Fatima Shad Kaneez and Faisal Khan Chapter 4 Epilepsy: Selenium and Aging 75 Caroline Rocourt, Ying Yu and Wen-Hsing Cheng Part 2 Molecular Genetics of Epilepsy