MISCELLANEA ON ENCEPHALOPATHIES Edited by Radu Tanasescu Miscellanea on Encephalopathies Edited by Radu Tanasescu Published by InTech Janeza Trdine 9, 51000 Rijeka, Croatia Copyright © 2012 InTech All chapters are Open Access distributed under the Creative Commons Attribution 3.0 license, which allows users to download, copy and build upon published articles even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. 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. As for readers, this license allows users to download, copy and build upon published chapters even for commercial purposes, as long as the author and publisher are properly credited, which ensures maximum dissemination and a wider impact of our publications. Notice 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 chapters. 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 Molly Kaliman Technical Editor Teodora Smiljanic Cover Designer InTech Design Team First published June, 2012 Printed in Croatia A free online edition of this book is available at www.intechopen.com Additional hard copies can be obtained from orders@intechopen.com Miscellanea on Encephalopathies, Edited by Radu Tanasescu p. cm. ISBN 978-953-51-0499-5 Contents Preface IX Chapter 1 Posttransplantation Encephalopaties 1 Daniela Anghel, Laura Dumitrescu, Catalina Coclitu, Amalia Ene, Ovidiu Bajenaru and Radu Tanasescu Chapter 2 HIV Encephalopathy – Now and Then 19 Cristina Loredana Benea, Ana-Maria Petrescu and Ruxandra Moroti-Constantinescu Chapter 3 Central Nervous System Involvement in Lyme Disease – Making the Diagnosis and Choosing the Correct Treatment 55 Ruxandra Calin, Adriana Hristea and Radu Tanasescu Chapter 4 Mechanisms of Cell Death in the Transmissible Spongiform Encephalopathies 69 Fiona Lane, James Alibhai, Jean C. Manson and Andrew C. Gill Chapter 5 Molecular Pathogenesis of Prion Diseases 95 Giuseppe Legname and Gianluigi Zanusso Chapter 6 Acute Encephalopathies and Psychiatry 113 Karim Sedky, Racha Nazir and Steven Lippmann Chapter 7 Current Advances in Cerebral Malaria Associated Encephalopathy 129 Mingli Liu, Shanchun Guo, Monica Battle and Jonathan K. Stiles Chapter 8 Encephalopathy Related to Ivermectin Treatment of Onchocerciasis in Loa loa Endemic Areas: Operational Considerations 169 Takougang Innocent and Muteba Daniel Chapter 9 Past and Future of Diagnosis and Therapy of Transmissible Spongiform Encephalopathy 187 Chih-Yuan Tseng and Jack Tuszynski "There is only one good, knowledge, and one evil, ignorance" Socrates Preface Along the years the concept of encephalopathy evolved from a clinical syndrome translating a more or less diffuse and more or less reversible or even progressive structural or only functional impairment of the brain, to a multitude of syndromes corresponding to one or several overlapping etiologies, ranging from toxic (endogenous or exogenous), to metabolic (genetic or acquired), hypoxic and/or ischemic, dysimmune, infectious and neurodegenerative, and having more or less understood pathogenic mechanisms, therefore describing distinct diseases. One may argue that a book on this subject is futile: the term encephalopathies encompasses such a broad, heterogeneous and constantly expanding spectrum; the focus of the current medical practice is changing from holistic to increasingly specialized; and the current literature revolves around diseases and not syndromes. A challenge for a nowadays neurologist is to cover all the complex field of brain disturbances by mastering the clinical features, knowing the right treatment and understanding the underlying mechanisms of those conditions. It is no doubt that this somewhat exhaustive attempt is difficult, if not impossible. The quantity of information laying at our disposal makes things more difficult than simplifying them, since the Socratic quote “Information is not Knowledge” implies integration of information as a crucial step on the path of understanding. Notwithstanding, while synthesizing and sedimenting information is an individual process, gathering the ‘right kind’ of information – the one which is not only accurate or complete but most of all relevant for the topic tackled- implies having access to ‘appropriate’ information sources. Nevertheless, brain disease implies multidisciplinary approach. For incumbent mechanisms understanding integration of basic science notions is required, while for circumscribing a broader context which facilitate brain disease (such as a metabolic one), a look gathering multi-specialty angles is needed. With this particular clinical approach in mind I propose to the reader the present book as the first part of a book project on various aspects of encephalopathies. This book groups mainly but not solely encephalopathies associated to infectious conditions, while a future one will be mainly dedicated to some various aspects regarding metabolic and hypoxic encephalopathies. As the title 'Miscellanea on X Preface Encephalopathies' states, the book does not aspire to be exhaustive, nor didactic, but rather a unique collection of chapters, written by different authors, discussing several particular aspects of certain encephalopathies as understood through the own experience of each author. By contributing to a book designed to have an atypical and less rigid structure, each author was allowed to write on a chosen subject that best reflects his/hers own expertise in the field. This approach is pertinent in the contemporaneous world when the access to information is facile, and of greater value than a uniform approach of all encephalopathies. Moreover, I consider it to be in agreement with the above-mentioned increasingly specialization tendency. Though not unitary in structure and in the quantity and quality of the offered information, the present book covers many of the important aspects regarding common and also rare encephalopathies, mostly in respect to their classic and novel diagnostic techniques and up to date therapies, and thus the book tries to respond in detail to some of the questions encountered in the clinical practice. Therefore the book is less than a sum of the current information and less than a review of the literature, but at the same time is more than that, transmitting valuable information filtered through the real life clinical and research experience of the authors, to which I thank for their willingness and effort in sharing their knowledge. Since many encephalopathies appear in the setting of systemic disease (e.g. organ failure, infectious diseases) and/or sometimes of their therapy (e.g. chemotherapy), the book addresses not only neurologists, but also fellow colleagues of other specialties, and into a lesser degree, medical researchers in the fields of neuroscience, genetics, immunology and infectious diseases and medical students. With this being said, the authors and I hope that the book will become a valuable source of information allowing to a more comprehensive view on such a vast subject as encephalopathies. Radu Tanasescu University of Medicine and Pharmacy "Carol Davila" Bucharest, Department of Neurology, Colentina Hospital, Romania [...]... stuporous or comatose 14 Miscellanea on Encephalopathies Agitation and somnolence occur alternatively Confusion and agitation are associated with hypoxia; despite hypoxia correction the state of consciousness remains altered, usually correlated with septic hypotension Neurological examination should assess neck stiffness, motor responses, muscular strength, plantar and deep tendon reflexes and cranial... consist in local changes (already mentioned cellular neurotoxins and BBB deterioration) and systemic changes, which means chronic immune activation The chronic immune activation can be done by any persistent infectious or noninfectious inflammation The repercussion is an accelerated immune-mediated global vascular senescence (endothelial dysfunction with subsequent atherosclerosis) which has as consequences... since onset Confusion and altered mental status are very frequent findings and may hide other symptoms such as disturbed vision and nausea Patients can be confused, lethargic with slowed motor responses or deeply stuporous As mentioned, seizures, including nonconvulsive status epilepticus, may occur in those with PRES.Differentiating altered mental status from non-convulsive status epilepticus on clinical... (i.e.basiliximab and daclizumab), monoclonal anti-CD20 antibody (i.e rituximab) Except for muromonab their administration in transplanted patients is associated with a very low prevalence of neurologic adverse effects Muromonab-CD3 (Orthoklone OKT3) is a murine monoclonal antibody directed to the CD3 portion of the T-cell receptor It blocks T-cell function and has limited reactions with other tissues or cells... barrier is attenuated by glial cells, dexamethasone or nitric oxid syntethase inhibition Mitochondrial dysfunction, oxidative stress, and apoptosis also occur The formation of reactive oxygen species compromises cell function and survival A major consequence of oxidative stress is apoptosis Neuronal apoptosis can also be secondary to glial cell dysfunction Neurons are also vulnerable to other disturbances... encephalopathy and neurocognitive dysfunction; microemboli are either gaseous or particulate 6 Miscellanea on Encephalopathies 2 Cerebral hypoperfusion: induces injury caused by the combination of systemic hypotension and cerebral venous hypertension (traction on the superior vena cava), occuring during off-pump surgery Atherosclerosis of the aorta: atherosclerotic lesions injury during surgery can result... changes on brain MRI translating cytotoxic and subsequently vasogenic edema and blood brain barrier disruption) and by the identification of low thiamine blood levels However, since parenteral thiamine administration is cheap, has virtually no contraindications (except for prior allergic reactions) and might be a life saving intervention, its administration should be started on clinical grounds only The... infection should also be considered In the setting of CNS signs and symptoms the electroencephalogram (EEG) is a useful investigation since it can identify diffuse non-specific slowing of the normal activity translating diffuse brain injury, and it can confirm the presence of non-convulsive seizures which may be difficult to differentiate from confusion or other mental status changes on clinical grounds only... compatible with CNS disorder Remission and comorbid psychiatric disturbance criteria similar to that for ANI and MND However, if dementia persists after one month on remission of major depression, a reassessment should be conducted to reassess for dementia Table 1 Nosology of HIV-associated neurocognitive impairment (22) 24 Miscellanea on Encephalopathies 5 Clinical manifestations Neurocognitive impairment... involving : executive functions (ability of planning, decision-making, mental flexibility), concentration and complex attention ( sustained attention, divided attention, selective attention, processing speed), verbal memory, learning and memory recall (24,25) Cortical dementia is more likely to involve memory loss, language comprehension, visual- spatial dysfunction and deficient conceptual abilities Most . when infection is suspected. Early posttransplantation period (first 30 days) Subacute posttransplantation period (1-6 months) Chronic posttransplantation period (>6 months) METABOLIC. administration and bilateral decompressive craniectomy should be taken into consideration (Jantzen 2007). 3. Cranial irradiation encephalopathy Cranial irradiation is commonly used before bone marrow. dysfunction; microemboli are either gaseous or particulate. Miscellanea on Encephalopathies 6 2. Cerebral hypoperfusion: induces injury caused by the combination of systemic hypotension and