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Neuropsychological Neurology
Neuropsychological Neurology
Neuropsychological Neurology
Neuropsychological Neurology
Neuropsychological Neurology
Neuropsychological Neurology
Neuropsychological Neurology
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Neuropsychological Neurology

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  • 1. Neuropsychological Neurology The Neurocognitive Impairments of Neurological Disorders © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 2. Neuropsychological Neurology The Neurocognitive Impairments of Neurological Disorders A. J. Larner Consultant Neurologist, Cognitive Function Clinic, Walton Centre for Neurology and Neurosurgery, Liverpool, UK © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 3. cambridge university press Cambridge, New York, Melbourne, Madrid, Cape Town, Singapore, Sa˜o Paulo, Delhi Cambridge University Press The Edinburgh Building, Cambridge CB2 8RU, UK Published in the United States of America by Cambridge University Press, New York www.cambridge.org Information on this title: www.cambridge.org/9780521717922 ª A. J. Larner 2008 This publication is in copyright. Subject to statutory exception and to the provisions of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of Cambridge University Press. First published 2008 Printed in the United Kingdom at the University Press, Cambridge A catalogue record for this publication is available from the British Library Library of Congress Cataloguing in Publication data Larner, A. J. Neuropsychological neurology : the neurocognitive impairments of neurological disorders / A.J. Larner. p. ; cm. Includes bibliographical references and index. ISBN 978-0-521-71792-2 (pbk.) 1. Cognition disorders. 2. Clinical neuropsychology. 3. Neurobehavioral disorders. I. Title. [DNLM: 1. Nervous System Diseases – complications. 2. Cognition Disorders – physiopathology. 3. Neuropsychology – methods. WL 140 L325n 2008] RC553.C64L37 2008 616.8–dc22 2007049017 ISBN 978-0-521-71792-2 paperback Cambridge University Press has no responsibility for the persistence or accuracy of URLs for external or third-party internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate. Every effort has been made in preparing this publication to provide accurate and up-to-date information which is in accord with accepted standards and practice at the time of publication. Although case histories are drawn from actual cases, every effort has been made to disguise the identities of the individuals involved. Nevertheless, the authors, editors and publishers can make no warranties that the information contained herein is totally free from error, not least because clinical standards are constantly changing through research and regulation. The authors, editors and publishers therefore disclaim all liability for direct or consequential damages resulting from the use of material contained in this publication. Readers are strongly advised to pay careful attention to information provided by the manufacturer of any drugs or equipment that they plan to use. © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 4. To Jo – because no one has dedicated a book to you before Disorders of intellect . . . happen much more often than superficial observers will easily believe. Samuel Johnson, The History of Rasselas, Prince of Abyssinia (1759) © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 5. Contents Acknowledgements page ix Introduction 1 1 Cognitive function, neuropsychological evaluation, and syndromes of cognitive impairment 6 1.1 Attention 8 1.2 General intelligence, IQ 10 1.3 Memory 11 1.4 Language 15 1.5 Perception 16 1.6 Praxis 19 1.7 Executive function, ‘frontal function’ 20 1.8 ‘Bedside’ neuropsychological test instruments 22 1.9 Normal aging 32 1.10 Dementia, delirium, depression 33 1.11 Cortical versus subcortical dementias, thalamic dementia 35 1.12 Disconnection syndromes 37 2 Neurodegenerative disorders 39 2.1 Alzheimer’s disease (AD) 40 2.2 Frontotemporal lobar degenerations (FTLD) 49 2.3 Motor neurone disease (MND), amyotrophic lateral sclerosis (ALS) 63 2.4 Parkinson’s disease dementia (PDD) and dementia with Lewy bodies (DLB) 69 vii © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 6. 2.5 Prion diseases 82 2.6 Mild cognitive impairment (MCI) 87 3 Cerebrovascular disease: vascular dementia and vascular cognitive impairment 90 3.1 Cortical vascular dementia, multi-infarct dementia (MID), post-stroke dementia 93 3.2 Subcortical vascular dementia, Binswanger’s disease, lacunar state, subcortical ischaemic vascular disease (SIVD) 95 3.3 Strategic infarct dementia, strategic strokes 97 3.4 Subarachnoid haemorrhage (SAH) 101 3.5 Intracranial vascular malformations 104 3.6 Vasculopathies 106 3.7 Other cerebrovascular disorders 112 4 The epilepsies 115 4.1 Epilepsy and cognitive impairment 115 4.2 Cognitive decline and epilepsy: shared aetiology 116 4.3 Seizures causing acquired cognitive impairment 120 4.4 Antiepileptic drug therapy causing cognitive impairment 122 4.5 Treatment of cognitive problems in epilepsy 123 5 Neurogenetic disorders 125 5.1 Hereditary dementias 126 5.2 Hereditary ataxias 135 5.3 Hereditary spastic paraplegia 140 5.4 Hereditary movement disorders 141 5.5 Hereditary metabolic disorders 146 5.6 Hereditary neurocutaneous syndromes (phakomatoses) 155 6 Inflammatory, immune-mediated, and systemic disorders 157 6.1 Multiple sclerosis (MS) 157 6.2 Acute disseminated encephalomyelitis (ADEM) 165 6.3 Sarcoidosis 165 6.4 Systemic lupus erythematosus (SLE) 166 6.5 Sjo¨gren’s syndrome 168 6.6 Behc¸et’s disease 169 6.7 Rheumatoid arthritis (RhA) 170 6.8 Scleroderma, systemic sclerosis 170 6.9 Relapsing polychondritis 171 6.10 Cerebral vasculitides 171 6.11 Sydenham’s chorea, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) 173 6.12 Limbic encephalitis 174 6.13 Hashimoto’s encephalopathy (HE) 176 6.14 Erdheim–Chester disease 176 6.15 Bilateral vestibulopathy 177 7 Structural brain lesions 178 7.1 Brain tumours and their treatment 178 7.2 Hydrocephalic dementias 182 7.3 Other structural lesions 186 8 Endocrine, metabolic, and toxin-related disorders 188 8.1 Endocrine disorders 188 8.2 Metabolic disorders 193 8.3 Toxin-related disorders 200 9 Infective disorders 204 9.1 Encephalitides and meningoencephalitides 205 9.2 Meningitides 209 9.3 Human immunodeficiency virus (HIV) and related conditions 210 9.4 Other disorders of infective aetiology 211 10 Neuromuscular disorders 215 10.1 Myotonic dystrophy (Steinert disease) 215 10.2 Myasthenia gravis (MG) 216 Index 218 viii Contents © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
  • 7. Acknowledgements First and foremost I thank my wife Philippa, and children Thomas and Elizabeth, for their forbear- ance and moral support (perhaps involuntary) in allowing me to write another book. I thank the many colleagues with whom I have worked and from whom I have learned much: Mark Doran, Eric Ghadiali, Daniel du Plessis, and Pavla Hancock in Liverpool; Alex Leff, Jonathan Schott, Nick Fox, Angus Kennedy, John Janssen, Lisa Cipolotti, Gail Robinson, Richard Wise, and Martin Rossor in London; and John Hodges in Cambridge. I thank also the colleagues and friends who have offered advice on particular topics in this book: Rustam Al-Shahi, Alasdair Coles, Kalvinder Gahir, Desmond Kidd, Michael and Sally Mansfield, and Sivakumar Sathasivam. All errors or misconceptions which remain are entirely my own work. I shall be pleased to hear from readers who detect errors or omissions. ix © Cambridge University Press www.cambridge.org Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information

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