Nervous System Disorders

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Dominique Bagnard, Ph.D. Neuropilin: From Nervous System to Vascular and Tumor Biology Neuropilin: From Nervous System to Vascular and Tumor Biology Neuropilin: From Nervous System to Vascular and Tumor Biology Edited by Dominique Bagnard, Ph.D. Maître de Conférences Université Louis Pasteur 67084 Strasbourg, France email: bagnard@neurochem.u-strasbg.fr Kluwer Academic / Plenum Publishers New York, Boston, Dordrecht, London, Moscow Neuropilin: From Nervous System to Vascular and Tumor Biology Edited by Dominique Bagnard ISBN 0-306-47416-6 AEMB volume number: 515 ©2002 Kluwer Academic / Plenum Publishers and Landes Bioscience Kluwer Academic / Plenum Publishers 233 Spring Street, New York, NY 10013 http://www.wkap.nl Landes Bioscience 810 S. Church Street, Georgetown, TX 78626 http://www.landesbioscience.com; http://www.eurekah.com Landes tracking number: 1-58706-168-6 10 987654321 A C.I.P. record for this book is available from the Library of Congress. All rights reserved. No part of this book may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, microfilming, recording, or otherwise, without written permission from the Publisher. Printed in the United States of America. Library of Congress Cataloging-in-Publication Data CIP applied for but not received at time of publication. v PREFACE Cell adhesion is one of the most important properties controlling embryonic development. Extremely precise cell-cell contacts are established according to the nature of adhesion molecules that are expressed on the cell surface. The identifica- tion of several families of adhesion molecules, well conserved throughout evolu- tion, has been the basis of a considerable amount of work over the past 20 years that contributed to establish functions of cell adhesion in almost all organs. Nowadays, cell adhesion molecules are not just considered as cellular glue but are thought to play critical roles in cell signaling. Their ability to influence cell proliferation, mi- gration, or differentiation depends on both cell surface adhesion properties and acti- vation of intracellular pathways. The next challenge will be to understand how these molecules interact with each other to ensure specific functions in the morphogen- esis of very sophisticated systems. Indeed, by exploring the cellular and molecular mechanisms of nervous system development, the group of H. Fujisawa in Japan identified in 1987 an adhesion molecule, neuropilin, highly expressed in the neuro- pile of amphibian optic tectum. Ten years later, two groups discovered that neuropilin is a receptor for guidance signals of the semaphorin family. Axon guidance is a critical step during brain development and the mechanisms ensuring growth cone navigation are beginning to be well understood. The semaphorins are bifunctional signals defining permissive or inhibitory pathways sensed by the growth cone. Moreover, a semaphorin can be repellent or attractive depending on the axonal popu- lations. The complexity of the signaling cascade triggered by the semaphorin is further illustrated by the capacity of Sema3A to be repulsive for the axon and attrac- tive for the dendrites of cortical neurons. Hence, an appropriate response of the growth cone requires the recruitment of a receptor complex enabling the integration of this varying information. The analysis of the structure of neuropilin revealed a very short intracellular domain lacking transduction capacities. Because of these works, several groups started to analyze the possible interactions of neuropilin and described various binding partners allowing semaphorin transduction. The current view considers neuropilin as the heart of a receptor Nervous System Disorders Nervous System Disorders Bởi: OpenStaxCollege A nervous system that functions correctly is a fantastically complex, well-oiled machine—synapses fire appropriately, muscles move when needed, memories are formed and stored, and emotions are well regulated Unfortunately, each year millions of people in the United States deal with some sort of nervous system disorder While scientists have discovered potential causes of many of these diseases, and viable treatments for some, ongoing research seeks to find ways to better prevent and treat all of these disorders Neurodegenerative Disorders Neurodegenerative disorders are illnesses characterized by a loss of nervous system functioning that are usually caused by neuronal death These diseases generally worsen over time as more and more neurons die The symptoms of a particular neurodegenerative disease are related to where in the nervous system the death of neurons occurs Spinocerebellar ataxia, for example, leads to neuronal death in the cerebellum The death of these neurons causes problems in balance and walking Neurodegenerative disorders include Huntington’s disease, amyotrophic lateral sclerosis, Alzheimer’s disease and other types of dementia disorders, and Parkinson’s disease Here, Alzheimer’s and Parkinson’s disease will be discussed in more depth Alzheimer’s Disease Alzheimer’s disease is the most common cause of dementia in the elderly In 2012, an estimated 5.4 million Americans suffered from Alzheimer’s disease, and payments for their care are estimated at $200 billion Roughly one in every eight people age 65 or older has the disease Due to the aging of the baby-boomer generation, there are projected to be as many as 13 million Alzheimer’s patients in the United States in the year 2050 Symptoms of Alzheimer’s disease include disruptive memory loss, confusion about time or place, difficulty planning or executing tasks, poor judgment, and personality changes Problems smelling certain scents can also be indicative of Alzheimer’s disease and may serve as an early warning sign Many of these symptoms are also common in people who 1/10 Nervous System Disorders are aging normally, so it is the severity and longevity of the symptoms that determine whether a person is suffering from Alzheimer’s Alzheimer’s disease was named for Alois Alzheimer, a German psychiatrist who published a report in 1911 about a woman who showed severe dementia symptoms Along with his colleagues, he examined the woman’s brain following her death and reported the presence of abnormal clumps, which are now called amyloid plaques, along with tangled brain fibers called neurofibrillary tangles Amyloid plaques, neurofibrillary tangles, and an overall shrinking of brain volume are commonly seen in the brains of Alzheimer’s patients Loss of neurons in the hippocampus is especially severe in advanced Alzheimer’s patients [link] compares a normal brain to the brain of an Alzheimer’s patient Many research groups are examining the causes of these hallmarks of the disease One form of the disease is usually caused by mutations in one of three known genes This rare form of early onset Alzheimer’s disease affects fewer than five percent of patients with the disease and causes dementia beginning between the ages of 30 and 60 The more prevalent, late-onset form of the disease likely also has a genetic component One particular gene, apolipoprotein E (APOE) has a variant (E4) that increases a carrier’s likelihood of getting the disease Many other genes have been identified that might be involved in the pathology Link to Learning Visit this website for video links discussing genetics and Alzheimer’s disease Unfortunately, there is no cure for Alzheimer’s disease Current treatments focus on managing the symptoms of the disease Because decrease in the activity of cholinergic neurons (neurons that use the neurotransmitter acetylcholine) is common in Alzheimer’s disease, several drugs used to treat the disease work by increasing acetylcholine neurotransmission, often by inhibiting the enzyme that breaks down acetylcholine in the synaptic cleft Other clinical interventions focus on behavioral therapies like psychotherapy, sensory therapy, and cognitive exercises Since Alzheimer’s disease appears to hijack the normal aging process, research into prevention is prevalent Smoking, obesity, and cardiovascular problems may be risk factors for the disease, so treatments for those may also help to prevent Alzheimer’s disease Some studies have shown that people who remain intellectually active by playing games, reading, 2/10 Nervous System Disorders playing musical instruments, and being socially active in later life have a reduced risk of developing the disease Compared to a normal brain (left), the brain from a patient with Alzheimer’s disease (right) shows a dramatic neurodegeneration, particularly within the ventricles and hippocampus (credit: modification of work by “Garrando”/Wikimedia Commons ...I At a Glance Introduction Basic Elements of the Nervous System Spinal Cord and Spinal Nerves Brain Stem and Cranial Nerves Cerebellum Diencephalon Telencephalon Cerebrovascular and Ventricular Systems Autonomic Nervous System Functional Systems The Eye The Ear Kahle, Color Atlas of Human Anatomy, Vol. 3 © 2003 Thieme All rights reserved. Usage subject to terms and conditions of license. Kahle, Color Atlas of Human Anatomy, Vol. 3 © 2003 Thieme All rights reserved. Usage subject to terms and conditions of license. III Kahle, Color Atlas of Human Anatomy, Vol. 3 © 2003 Thieme All rights reserved. Usage subject to terms and conditions of license. IV Color Atlas and Textbook of Human Anatomy in 3 volumes Volume 1: Locomotor System by Werner Platzer Volume 2: Internal Organs by Helmut Leonhardt Kahle, Color Atlas of Human Anatomy, Vol. 3 © 2003 Thieme All rights reserved. Usage subject to terms and conditions of license. V Thieme Stuttgart · New York Volume 3 Nervous System and Sensory Organs by Werner Kahle, M.D. Professor Emeritus Institute of Neurology University of Frankfurt/Main Frankfurt/Main, Germany Michael Frotscher, M.D. Professor Anatomical Institute I University of Freiburg Freiburg, Germany 5th revised edition 179 color plates Illustrations by Gerhard Spitzer Kahle, Color Atlas of Human Anatomy, Vol. 3 © 2003 Thieme All rights reserved. Usage subject to terms and conditions of license. VI Library of Congress Cataloging-in-Publication Data is available from the publischer 1st German edition 1976 2nd German edition 1978 3rd German edition 1979 4th German edition 1982 5th German edition 1986 6th German edition 1991 7th German edition 2001 1st English edition 1978 2nd English edition 1984 3rd English edition 1986 4th English edition 1993 1st Dutch e dition 1978 2nd Dutch edition 1981 3rd Dutch edition 1990 4th Dutch edition 2001 1st French edition 1979 2nd French edition 1993 1st Greek edition 1985 1st Hungarian edition 1996 1st Indonesian edition 1983 1st Italian edition 1979 2nd Italian edition 1987 3rd Italian edition 2001 1st Japanese edition 1979 2nd Japanese edition 1981 3rd Japanese edition 1984 4th Japanese edition 1990 1st Polish edition 1998 1st Serbo-Croatian edition 1991 1st Spanish edition 1977 2nd Spanish edition 1988 1st Turkish edition 1987 This book is an authorized and revised transla- tion of the 7th German edition published and copyrighted 2001 by Georg Thieme Verlag, Stuttgart, Germany. Title of the German edition: Taschenatlas der Anatomie, Band 3: Nervensystem und Sinnes- organe Translated by Ursula Vielkind, Ph. D., C. Tran., Dundas, Ontario, Canada Some of the product names, patents and regis- tered designs referred to in this book are in fact registered trademarks or proprietary names even though specific reference to this fact is not always made in the text. Therefore, the appear- ance of a name without designation as pro- prietary is not to be construed as a representa- tion by the publisher that it is in the public domain. This book, including all parts thereof, is le- gally protected by copyright. Any use, exploita- tion or commercialization outside the narrow limits set by copyright legislation, without the publisher’s consent, is illegal and liable to pros- ecution. This applies in particular to photostat reproduction, copying, mimeographing or du- plication of any kind, translating, preparation of microfilms, and electronic data processing and storage. ᭧ 2003 Georg Thieme Verlag Rüdigerstraße 14, D-70469 Stuttgart, Germany http://www.thieme.de Thieme New York, 333 Seventh Avenue, New York, N.Y. 10001 U.S.A. http://www.thieme.com Cov National Cancer Institute 203 SEER Survival Monograph INTRODUCTION This study provides survival analysis for 19,774 histo- logically confirmed first primary brain and other cen- tral nervous system (CNS) cancers diagnosed from 1988 through 2001 from the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (NCI). The analysis performed in this study at- tempts to better understand the influence of morphologic and demographic factors on survival. Other CNS cancers include cancers of the central nervous system and malig- nant meningiomas of the brain. Benign and borderline tumors are not included in these analyses. Brain and other CNS cancers are considered to be rare compared to prostate, lung, breast, or colon cancer. It is estimated there will be 18,820 new cases diagnosed of and 12,820 deaths from brain and other CNS cancer in the United States each year (1). The average annual age- adjusted incidence rate for brain and other CNS cancer in the United States is 7.6 per 100,000 for males and 5.4 per 100,000 for females (white males: 8.3 per 100,000; white females: 5.9 per 100,000; black males: 4.9 per 100,000; black females: 3.5 per 100,000) (2). The average annual age-adjusted mortality rate is approximately 4.5 per 100,000 for all races combined, with males having a higher mortality rate as compared to females (2). Histologic type of tumor, age at diagnosis, race and treat- ment are all important predictors of survival, with a large variation in survival by histologic type of tumor (3, 4, 5, 6). The most common histologic subtypes of brain cancer are astrocytoma and glioblastoma multiforme (GBM), while the most common histologic subtypes of other CNS cancer are meningioma and ependymoma (3, 4, 7, 8, 9). Patients with GBM have the worst survival compared to any other histologic subtype (8). No risk factor accounting for a large number of brain and other CNS cancers has been identified. There has been some evidence for inherited factors, with approximately 16% of families studied having a family history of cancer (5, 10, 11). The only known risk factor for primary brain and other CNS cancers is exposure to therapeutic ion- izing radiation. Other factors have been shown to cause increased risk, including exposure to synthetic rubber manufacturing, to vinyl chloride, to petroleum refining/ production work, or to pesticides and consumption of cured foods, but the data are inconsistent (5). Exposure to filtered cigarettes, diagnostic ionizing radiation, resi- dential electromagnetic fields, formaldehyde, cell phone use and active or passive maternal tobacco smoking are not proven risk factors (5). The most common present- ing symptoms, progressive neurological deficit, motor weakness, headache and seizure, do not appear to be independent risk factors (5, 11). MATERIALS AND METHODS Exclusions Between 1988 and 2001, 29,335 adult cases of malignant brain and other CNS cancer were diagnosed and reported to the NCI SEER Program. Children (aged less than 20) were excluded because brain and other CNS cancer are different in children compared to adults in terms of incidence and survival (8, 12). Patients were followed for vital status until 2001. The survival analysis was based on relative survival rates calculated by the life-table method (13). The file:///C|/Documents%20and%20Settings/Administrator/Deskto 0BILL%20-%20BUSINESS%20AT%20THE%20SPEED%20OF%20THOUGHT.TXT BUSINESS AT THE SPEED OF THOUGHT by bill Gates ALSO By BILL GATES The Road Ahead BUSINESS AT THE SPEED OF THOUGHT: USING A DIGITAL NERVOUS SYSTEM BILL GATES WITH COLLINs HEMINGWAY 0 VMNER BOOKS A Time Warner Company To my wife, Melinda, and my daughter, Jennifer Many of the product names referred to herein are trademarks or registered trademarks of their respective owners. Copyright (D 1999 by William H. Gates, III All rights reserved. Warner Books, Inc, 1271 Avenue of the Americas, New York, NY 10020 Visit our Web site at www.warnerbooks.com 0 A Time Warner Company Printed in the United States of America First Printing: March 1999 10 9 8 7 6 5 4 3 2 1 ISBN: 0-446-52568-5 LC: 99-60040 Text design by Stanley S. Drate lFolio Graphics Co Inc Except as file:///C|/Documents%20and%20Settings/Admini SINESS%20AT%20THE%20SPEED%20OF%20THOUGHT.TXT (1 of 392)12/28/2005 5:28:51 PM file:///C|/Documents%20and%20Settings/Administrator/Deskto 0BILL%20-%20BUSINESS%20AT%20THE%20SPEED%20OF%20THOUGHT.TXT indicated, artwork is by Gary Carter, Mary Feil-jacobs, Kevin Feldhausen, Michael Moore, and Steve Winard. ACKNOWLEDGMENTS I first want to thank my collaborator, Collins Hemingway, for his help in synthesizing and developing the material in this book and for his overall management of this project. I want to thank four CEOs who read a late draft of the manuscript and offered valuable thoughts on how to make it more meaningful for business leaders: Paul O'Neill, Alcoa; Ivan Seidenberg, Bell Atlantic; Tony Nicely, GEICO Insurance; and Ralph Larsen, Johnson & Johnson. Details on the use of technology by business and public agencies came from worldwide travel and research by Collins and by Jane Glasser. Barbara Leavitt, Evelyn Vasen,and Ken Linarelli researched one or more chapters. The book gained from the careful editing of Erin O'Connor during manuscript development. Anne Schott served as combination research assistant and project coordinator. I want to thank Bob Kruger and Tren Griffin who offered thoughtful comments on many chapters as the book progressed. And Steve Ballmer, Bob Herbold, and Jeff Raikes for their thoughts about the book's organization and focus. David Vaskevitch, Rich Tong, Gary Voth, and Mike Murray helped shape important ideas. For their review comments thanks to Mich Mathews and John Pinette. Thanks also to Larry Kirshbaum, chairman and CEO of Time Warner Trade Publishing, and Rick Horgan, VP and executive editor of Warner Books, for their incisive feedback. Thanks to Kelli Jerome, who has now managed the worldwide marketing of both of my books in a smooth and professional manner, and to Lee Anne Staller for her help in sales. At Warner, thanks also to Harvey-Jane Kowal, VP and executive managing editor, and Bob Castillo, senior pro duction editor, aswell as Sona Vogel, copy editor, for their editorial assistance. With all the search capabilities provided by technology, the researchers at the Microsoft Library remained an in valuable resource: Laura Bain, Kathy Brost, Jill Burger, Lynne Busby, Peggy Crowley, Erin Fields, April Hill, Susan Hoxie, Jock McDonald, Tammy Pearson, K.C. file:///C|/Documents%20and%20Settings/Admini SINESS%20AT%20THE%20SPEED%20OF%20THOUGHT.TXT (2 of 392)12/28/2005 5:28:51 PM file:///C|/Documents%20and%20Settings/Administrator/Deskto 0BILL%20-%20BUSINESS%20AT%20THE%20SPEED%20OF%20THOUGHT.TXT Rich, Deborah Robinson, Christine Shannon, Mary Taylor, Dawn Zeh, and Brenda Zurbi. For their general assistance, thanks to Christine Turner and Gordon Lingley This work gained ... who specialize in disorders of the nervous system They diagnose and treat disorders such as epilepsy, stroke, dementia, nervous system injuries, Parkinson’s disease, sleep disorders, and multiple... the nervous system may be damaged in a patient Mental Illnesses Mental illnesses are nervous system disorders that result in problems with thinking, mood, or relating with other people These disorders. .. Neurodevelopmental Disorders Neurodevelopmental disorders occur when the development of the nervous system is disturbed There are several different classes of neurodevelopmental disorders Some,