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DEMENTIA F I F T H E D I T I O N DEMENTIA F I F T H E D I T I O N Edited by David Ames BA, MD, BS, FRCPsych, FRANZCP Emeritus Professor University of Melbourne Department of Psychiatry and National Ageing Research Institute, Parkville, Victoria, Australia John O’Brien MA DM, FRCPsych, FMedSci Foundation Professor of Old Age Psychiatry Department of Psychiatry University of Cambridge, Cambridge, UK Alistair Burns CBE, MD, FRCP, FRCPsych Professor of Old Age Psychiatry University of Manchester, Manchester, UK CRC Press Taylor & Francis Group 6000 Broken Sound Parkway NW, Suite 300 Boca Raton, FL 33487-2742 © 2017 by Taylor & Francis Group, LLC CRC Press is an imprint of Taylor & Francis Group, an Informa business No claim to original U.S. Government works Printed on acid-free paper Version Date: 20161017 International Standard Book Number-13: 978-1-4987-0310-9 (Pack - Book and Ebook) This book contains information obtained from authentic and highly regarded sources. Reasonable efforts have been made to publish reliable data and information, but the author and publisher cannot assume responsibility for the validity of all materials or the consequences of their use. The authors and publishers have attempted to trace the copyright holders of all material reproduced in this publication and apologize to copyright holders if permission to publish in this form has not been obtained. If any copyright material has not been acknowledged please write and let us know so we may rectify in any future reprint. Except as permitted under U.S. Copyright Law, no part of this book may be reprinted, reproduced, transmitted, or utilized in any form by any electronic, mechanical, or other means, now known or hereafter invented, including photocopying, microfilming, and recording, or in any information storage or retrieval system, without written permission from the publishers. For permission to photocopy or use material electronically from this work, please access www.copyright.com (http:// www.copyright.com/) or contact the Copyright Clearance Center, Inc. (CCC), 222 Rosewood Drive, Danvers, MA 01923, 978-750-8400. CCC is a not-for-profit organization that provides licenses and registration for a variety of users. For organizations that have been granted a photocopy license by the CCC, a separate system of payment has been arranged. Trademark Notice: Product or corporate names may be trademarks or registered trademarks, and are used only for identification and explanation without intent to infringe. Visit the Taylor & Francis Web site at http://www.taylorandfrancis.com and the CRC Press Web site at http://www.crcpress.com Contents Foreword ix Preface x Acknowledgements xii Contributors xiii PART 1 DEMENTIA: General aspects 1 1 Dementia: Historical overview 3 German Berrios 2 The lived experience of dementia 19 Shirley Nurock 3 Prevalence and incidence of dementia 24 Daniel W. O’Connor 4 What is dementia, and how do you assess it? Definitions, diagnostic criteria and assessment 33 Joseph P.M. Kane and Alan Thomas 5 Screening and assessment instruments for the detection and measurement of cognitive impairment 44 Leon Flicker 6 Neuropsychological assessment of dementia 52 Greg Savage 7 Neuropsychiatric aspects of dementia 59 Nilika Perera, Mehran Javeed, Constantine G. Lyketsos and Iracema Leroi 8 Measurement of behaviour disturbance, non-cognitive symptoms and quality of life 67 Ajit Shah 9 Cross-cultural issues in the assessment of cognitive impairment 79 Ajit Shah 10 Structural brain imaging 92 Robert Barber and John T. O’Brien 11 Functional brain imaging and connectivity in dementia 107 Klaus P. Ebmeier, Nicola Filippini, Clare E. Mackay, Sana Suri and Vyara Valkanova 12 Molecular brain imaging in dementia 119 Victor L. Villemagne and Christopher C. Rowe 13 Services for people with dementia 135 Sube Banerjee 14 Family carers of people with dementia 142 Katrin Seeher and Henry Brodaty 15 Dementia care in the community: Challenges for primary health and social care 161 Steve Iliffe, Jill Manthorpe and Vari Drennan 16 Managing people with dementia in the general hospital 172 Andrew Teodorczuk, Rowan Harwood and Elizabeth Sampson 17 The role of nursing in the management of dementia 183 Maree Mastwyk and Beverley Williams 18 Social work and care/case management in dementia 192 David Challis, Jane Hughes and Caroline Sutcliffe v vi Contents 19 Occupational therapy in dementia care 199 Alissa Westphal 20 Speech and language therapy in dementia assessment and management 208 Bronwyn Moorhouse and Caroline A. Fisher 21 Role of the physiotherapist in the management of dementia 220 Sue Lord and Lynn Rochester 22 Therapeutic effects of music in persons with dementia 229 Linda A. Gerdner and Ruth Remington 23 Psychological, behavioural and psychosocial interventions for neuropsychiatric symptoms in dementia: What works, what does not and what needs more evidence? 235 Gill Livingston and Claudia Cooper 24 Treating problem behaviours in dementia by understanding their biological, social and psychological causes 244 Ian James and Louisa Jackman 25 Treatments for behavioural and psychological symptoms in Alzheimer’s disease and other dementias 257 Anna Burke, William J. Burke and Pierre N. Tariot 26 Psychological approaches for the practical management of cognitive impairment in dementia 279 Anne Unkenstein 27 Sexuality and dementia 285 Joe Stratford 28 Residential care for people with dementia 292 David Conn, John Snowdon and Nitin Purandare 29 Design and dementia 304 June Andrews 30 Legal issues and dementia 308 Hugh Series and Robin Jacoby 31 Driving and dementia 314 Aoife Fallon and Desmond O’Neill 32 Quality of life in dementia: Conceptual and practical issues 325 Betty S. Black and Peter V. Rabins 33 Ethical issues 339 Julian C. Hughes and Daniel Strech 34 End-of-life and palliative care in dementia 344 Julian C. Hughes and Jenny T. van der Steen 35 Advanced dementia and care at the end of life 349 Kirsten Moore and Elizabeth Sampson 36 Alzheimer’s associations and societies 359 Henry Brodaty, Nicole Pesa and Glenn Rees 37 Health economic aspects of dementia 371 Anders A. Wimo, Linus Jönsson and Bengt Winblad 38 Global challenge of dementia: What can be done? 388 Cleusa P. Ferri, Maëlenn Guerchet and Martin Prince 39 The international challenge of dementia 402 Karim Saad 40 The pharmaceutical industry and dementia: How can clinicians, researchers and industry work together for the betterment of people with dementia and their families? Continuous vigilance and transparency is the answer 411 Martin M. Bednar and Leon Flicker PART 2 MILD COGNITIVE IMPAIRMENT 418 41 Mild cognitive impairment (MCI): A historical perspective 419 Karen Ritchie and Sylvaine Artero 42 Clinical characteristics of mild cognitive impairment 426 Yonas E. Geda, Janina Krell-Roesch, Anna Pink, Kathleen A. Spangehl and Ronald C. Petersen 43 Managing the patient with mild cognitive impairment 439 Nicola T. Lautenschlager and Alexander F. Kurz Contents vii PART 3 ALZHEIMER’S DISEASE: Clinical Aspects 446 44 Risk factors for Alzheimer’s disease 447 Patricia A. Boyle and Robert S. Wilson 45 The natural history of Alzheimer’s disease 453 Jody Corey-Bloom and Michael S. Rafii 46 The neuropathology of Alzheimer’s disease 470 Colin L. Masters 47 Neurochemistry of Alzheimer’s disease 486 Paul T. Francis 48 The central role of Aβ amyloid and tau in the pathogenesis of Alzheimer’s disease 492 Craig W. Ritchie and Colin L. Masters 49 Inflammation in Alzheimer’s disease 508 Clive Holmes 50 Genetics of Alzheimer’s disease 519 Margie Smith 51 Blood and cerebrospinal fluid biomarkers for Alzheimer’s disease 528 Simone Lista, Henrik Zetterberg, Sid E. O’Bryant, Kaj Blennow and Harald Hampel 52 Established treatments for Alzheimer’s disease 539 Alexander Kurz and Nicola T. Lautenschlager 53 Drug treatments in development for Alzheimer’s disease 554 Paul Yates and Michael Woodward 54 Vitamins and complementary therapies used to treat Alzheimer’s disease 587 Dennis Chang, Genevieve Z. Steiner, Dilip Ghosh and Alan Bensoussan 55 Prevention of Alzheimer’s disease and Alzheimer’s dementia 598 Tom C. Russ, Karen Ritchie and Craig W. Ritchie 56 Trial designs 613 Serge Gauthier PART 4 THE OVERLAP AND INTERACTION BETWEEN ALZHEIMER’S DISEASE AND CEREBROVASCULAR DISEASE 618 57 Vascular factors and Alzheimer’s disease 619 Robert Stewart PART 5 CEREBROVASCULAR DISEASE AND COGNITIVE IMPAIRMENT 629 58 What is vascular cognitive impairment? 630 Timo Erkinjuntti, Leonardo Pantoni and Susanna Melkas 59 The neuropathology of vascular dementia 643 Raj N. Kalaria 60 Therapeutic strategies for vascular cognitive disorders 660 Gustavo C. Román PART 6 DEMENTIA WITH LEWY BODIES AND PARKINSON’S DISEASE 702 61 Dementia with Lewy bodies: A clinical overview 703 Ian G. McKeith 62 Pathology of dementia with Lewy bodies 714 Glenda Halliday, Simon Lewis and Paul Ince 63 The treatment of dementia with Lewy bodies 730 John-Paul Taylor 64 Cognitive impairment and dementia in Parkinson’s disease 740 Dag Aarsland, Kolbjørn Brønnick, Milica G. Kramberger and Joana B. Pereira viii Contents PART 7 FOCAL DEMENTIAS AND OTHER NEUROPSYCHIATRIC SYNDROMES INVOLVING COGNITIVE DECLINE 748 65 Frontotemporal dementia 749 Peter J. Nestor 66 Pick’s disease: Its relationship to progressive aphasia, semantic dementia and frontotemporal dementia 759 John Hodges 67 The genetics and molecular pathology of frontotemporal lobar degeneration 771 David M.A. Mann 68 Semantic dementia 783 Julie S. Snowden and Matthew Jones 69 Primary progressive aphasia and posterior cortical atrophy 795 Jonathan D. Rohrer, Sebastian J. Crutch and Jason D. Warren 70 The cerebellum and cognitive impairment 812 Elsdon Storey and Evelyn A. Lindsay 71 Delirium: A clinical overview 823 Andrew Teodorczuk and Daniel Davis 72 Depression with cognitive impairment 832 Sarah Shizuko Morimoto, Genevieve S. Yuen, Stephen Beres and George S. Alexopoulos 73 Schizophrenia, cognitive impairment and dementia 846 Flavie Waters, Andrew Ford and Osvaldo P. Almeida 74 Dementia in intellectual disabilities 852 Jennifer Torr 75 Alcohol-related dementia and Wernicke–Korsakoff syndrome 857 Stephen C. Bowden and Simon J. Scalzo 76 Huntington’s disease 868 Phyllis Chua, Samantha Loi and Edmond Chiu 77 Uncommon dementias (including the prion diseases) 885 Matthew Jones and David Neary Index 898 Foreword The modern study of dementia began 50 years ago with the many techniques and approaches that were not dreamed of pioneering work of Martin Roth and colleagues. In the mid- 50 years ago. Computerized imaging has been available for 1960s, their ground-breaking epidemiologic study demon- 40 years, but the recent development of positron emission strated both the high prevalence of dementia in the elderly tomography (PET) imaging ligands specific for the molecu- and its association with ageing. Several years later they dem- lar abnormalities characteristic of several dementias is likely onstrated that the neuropathology first described by Alois to dramatically change the evaluation of people presenting Alzheimer in 1907 in a woman who died from dementia at the earliest symptoms of cognitive disorder, to lead to the the age of 56 is the commonest neuropathological abnor- identification of people at high risk of developing a dementia mality seen in dementia. syndrome in 10–20 years before they become symptomatic This 5th edition of Dementia demonstrates how much and, possibly, to reshape the assessment of therapeutics. The knowledge has been gained in the past half-century since ability to identify genetic variants inexpensively, accurately these studies were conducted. Epidemiological studies and quickly is already helping to identify multiple causes throughout the world have identified many risk factors for of what had appeared to be single dementia syndromes and dementia and Alzheimer’s disease and these are beginning might lead to revolutionary treatments that target the cause to translate into prospective intervention studies. Advances of dementia in specific individuals. in molecular biology and genetic medicine have identified Finally, this edition of Dementia highlights the recogni- specific protein abnormalities that underlie many of the tion that extensive overlap exists between disorders that tra- neurodegenerative causes of dementia, identified genes act- ditionally have been considered conceptually distinct. The ing in classic Mendelian patterns in some dementias and concept that ‘functional’ disorders are categorically distinct via non-Mendelian mechanisms in most, identified prions from the disorders of cognition with identifiable neuropa- as the causative ‘agent’ of Creutzfeldt–Jacob disease (CJD) thology, which is now passé as cognitive impairment and and variant CJD (vCJD); they also raised the possibility that has been identified as a central feature of schizophrenia prion-like mechanisms explain the ‘spread’ of neurodegen- and impaired cognitive performance, has been identified as eration in many diseases. present in individuals of all ages with major depression and As extraordinary as these biological discoveries are, this bipolar disorder. Even the dichotomous distinction between edition of Dementia also highlights the equally impres- Alzheimer’s dementia and vascular dementia (VaD), so cen- sive advances that have been made in treatment. Except for tral to Martin Roth’s pioneering work, is coming under HIV-AIDS dementia, no dramatic cures have been discov- question as the co-occurrence of these two cases of demen- ered, but an extensive research literature demonstrates the tia is now being shown to result from shared neurobiology. effectiveness and benefits of environmental and behavioural In summary, this 5th edition of Dementia stands as a interventions that target the symptoms of dementia, the marker of the tremendous progress that has been made in efficacy of interventions that target the emotional distress understanding the pathophysiology of the various demen- experienced by many family members and care providers, tias and of the advances in symptomatic treatment. Just as the importance of organizations that provide education importantly, it provides hope that these gains in knowledge and support and the limited but necessary role of pharma- will lead to preventative strategies that abolish or greatly cotherapeutic treatment of Behavioural and Psychological lessen the morbidity associated with these devastating Symptoms of Dementia (BPSD). disorders. In addition to highlighting these extraordinary advances, this 5th edition of Dementia reviews the development of Peter V. Rabins MD, MPH ix

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