Neurodegenerative Disorders

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1 Neurodegenerative Disorders

2

3 Orla Hardiman Colin P. Doherty Editors Marwa Elamin Peter Bede Associate Editors Neurodegenerative Disorders A Clinical Guide Second Edition

4 Editors Orla Hardiman Academic Unit of Neurology Trinity Biomedical Sciences Institute Dublin Colin P. Doherty Academic Unit of Neurology Trinity Biomedical Sciences Institute Dublin Associate Editors Marwa Elamin Academic Unit of Neurology Trinity Biomedical Sciences Institute Dublin Peter Bede Academic Unit of Neurology Trinity Biomedical Sciences Institute Dublin ISBN DOI / ISBN (ebook) Library of Congress Control Number: Springer Cham Heidelberg New York Dordrecht London Springer International Publishing Switzerland 2016 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. Printed on acid-free paper Springer International Publishing AG Switzerland is part of Springer Science+Business Media (

5 Contents 1 Common Themes in the Pathogenesis of Neurodegeneration Marina A. Lynch, Orla Hardiman, Marwa Elamin, Janine Kirby, and Lewis P. Rowland 2 Imaging Biomarkers in Neurodegenerative Conditions Parameswaran Mahadeva Iyer, Colin P. Doherty, and Peter Bede 3 Role of Neuropsychology in Neurodegeneration Marwa Elamin, Thomas H. Bak, Colin P. Doherty, Niall Pender, and Sharon Abrahams 4 Alzheimer s Disease Damien Gallagher, Robert F. Coen, and Brian A. Lawlor 5 Dementia and Cerebrovascular Disease Joseph Harbison, Sean P. Kennelly, and Rose Anne Kenny 6 Parkinson s Disease Diana A. Olszewska, Stanley Fahn, Richard A. Walsh, and Tim Lynch 7 Fronto-Temporal Dementia (FTD) Marwa Elamin, Taha Omer, Siobhan Hutchinson, Colin P. Doherty, and Thomas H. Bak 8 Amyotrophic Lateral Sclerosis Orla Hardiman, Matthew C. Kiernan, and Leonard H. van den Berg 9 Huntington s Disease Tom Burke, Colin P. Doherty, Walter Koroshetz, and Niall Pender 10 Parkinsonism-Plus Syndromes Seán O Dowd, Daniel Healy, and David Bradley v

6 vi Contents 11 Medical Mimics of Neurodegenerative Diseases Lewis P. Rowland, Peter Bede, Marwa Elamin, and Orla Hardiman 12 Prion Diseases Simon Mead and Peter Rudge 13 Managing Neuropsychiatric Symptoms of Neurodegenerative Diseases Joseph Trettel, Zeina Chemali, and Kirk R. Daffner 14 HIV and Other Infectious Causes of Dementia Patricia McNamara, Lilia Zaporojan, Colin P. Doherty, Robert F. Coen, and Colm Bergin 15 Neuroinflammatory Disorders Lisa Costelloe, Jean Fletcher, and Denise Fitzgerald 16 Clinical Trials in Neurodegeneration Orla Hardiman, Julie A. Kelly, Thomas H. Bak, Marwa Elamin, Dragos L. Mihaila, Pamela J. Shaw, Hiroshi Mitsumoto, and Jeremy M. Shefner 17 Palliative Care and End of Life Care David Oliver, Sinead Maguire, Orla Hardiman, and Peter Bede Index

7 Contributors Sharon Abrahams Human Cognitive Neuroscience-Psychology, Euan MacDonald Centre for Motor Neurone Disease Research, Centre for Cognitive Aging and Epidemiology, University of Edinburgh, Edinburgh, UK Thomas H. Bak The Anne Rowling Regenerative Neurology Clinic, Centre for Clinical Brain Sciences (CCBS), University of Edinburgh, Edinburgh, UK Centre for Cognitive Ageing and Cognitive Epidemiology, University of Edinburgh, Edinburgh, UK Peter Bede Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Leonard H. van den Berg Department of Neurology, University Medical Center Utrecht, Utrecht, The Netherlands Colm Bergin Department of Infectious Diseases, St. James s Hospital, Department of Clinical Medicine, Trinity College, David Bradley Department of Neurology, St. Jame s Hospital, Tom Burke Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Zeina Chemali Departments of Neurology and Psychiatry, Massachusetts General Hospital, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA Robert F. Coen Memory Clinic, Mercer s Institute For Research on Aging, St. James s Hospital, Department of Gerontology, Trinity College Dublin, Lisa Costelloe Department of Neurology, Beaumont Hospital, vii

8 viii Contributors Kirk R. Daffner Center for Brain/Mind Medicine, Department of Neurology, Brigham and Women s Hospital, Harvard Medical School, Boston, MA, USA Colin P. Doherty Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Department of Neurology, St. James s Hospital, Marwa Elamin Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Stanley Fahn Department of Neurology, Columbia University Medical Center, New York, NY, USA Denise Fitzgerald Queens University, Belfast, Jean Fletcher Schools of Medicine and Biochemistry and Immunology, Trinity College Dublin, Damien Gallagher Geriatric Psychiatry, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, ON, Canada Joseph Harbison Department of Medical Gerontology, Mercer s Institute for Successful Ageing, St James s Hospital, Department of Medical Gerontology, Trinity College, Dublin, Dublin, Orla Hardiman Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Daniel Healy Department of Neurology, St. James s Hospital, Royal College of Surgeons in, Siobhan Hutchinson Cognitive Neurology Clinic, St. James s Hospital, Parameswaran Mahadeva Iyer Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Julie A. Kelly The Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Trinity College Dublin, Sean P. Kennelly Medical Gerontology, Trinity College, Dublin, Acute Medical Unit, Tallaght Hospital, Rose Ann Kenny Department of Medical Gerontology, Mercer s Institute for Successful Ageing, St James s Hospital, Department of Medical Gerontology, Trinity College, Dublin 2, Dublin,

9 Contributors ix Matthew C. Kiernan Brain and Mind Centre, Sydney Medical School, University of Sydnes, Royal Prince Alfred Hospital, Camperdown, NSW, Australia Janine Kirby Department Neurology, Sheffield Institute of Translational Neuroscience, Sheffield University, Sheffield, UK Walter Koroshetz Neurological Disorders and Stroke Institute, National Institutes of Health, Bethesda, MD, USA Brian A. Lawlor Department of Psychiatry, Trinity College Dublin, Marina A. Lynch Trinity College Institute of Neuroscience, Lloyd Building, Trinity College, Tim Lynch Department of Neurology, Dublin Neurological Institute, Mater Misericordiae University Hospital, Sinead Maguire Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Patricia McNamara Academic Unit of Neurology, Trinity Biomedical Sciences Institute, Simon Mead MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, UK NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, UK Dragos L. Mihaila Department of Neurology, SUNY Upstate Medical University, Syracuse, New York, USA Hiroshi Mitsumoto Department of Neurology, Columbia University Medical Center, New York, USA Seán O Dowd Department of Neurology, St. James s Hospital, Trinity College Medical School, David Oliver Wisdom Hospice, Consultant in Palliative Medicine, High Bank, Rochester, UK University of Kent, Kent, UK Diana A. Olszewska Department of Neurology, Dublin Neurological Institute, Mater Misericordiae University Hospital, Niall Pender Department of Neuropsychology, Beaumont Hospital, Academic Unit of Neurology, Trinity College Dublin,

10 x Contributors Lewis P. Rowland Department of Neurology, The Neurological Institute of New York, Columbia University Medical Center, New York, USA Peter Rudge MRC Prion Unit, Department of Neurodegenerative Disease, UCL Institute of Neurology, Queen Square, London, UK NHS National Prion Clinic, National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, UK Pamela J. Shaw Sheffield Institute for Translational Neuroscience (SITraN), University of Sheffield, Sheffield, UK Jeremy M. Shefner Barrow Neurological Institute, University of Arizona College of Medicine, Phoenix, AZ, USA Joseph Trettel Department of Neurobehavioral Medicine, Gaylord Hospital, Wallingford, CT, USA Richard A. Walsh Department of Neurology, Tallaght Hospital, Lilia Zaporojan Academic Unit of Neurology, Trinity College Dublin, Taha Omer Academic Unit of Neurology, Trinity Biomedical Sciences Institute,

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