Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
Neuropsychological Neurology The Neurocognitive Impairments of Neurological Disorders
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Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
Neuropsychological Neurology The Neurocognitive Impairments of Neurological Disorders
A. J. Larner Consultant Neurologist, Cognitive Function Clinic, Walton Centre for Neurology and Neurosurgery, Liverpool, UK
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Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
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.
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Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
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)
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Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
Contents
Acknowledgements
page ix
Introduction 1
2
1
Cognitive function, neuropsychological evaluation, and syndromes of cognitive impairment 1.1 Attention 1.2 General intelligence, IQ 1.3 Memory 1.4 Language 1.5 Perception 1.6 Praxis 1.7 Executive function, ‘frontal function’ 1.8 ‘Bedside’ neuropsychological test instruments 1.9 Normal aging 1.10 Dementia, delirium, depression 1.11 Cortical versus subcortical dementias, thalamic dementia 1.12 Disconnection syndromes
6 8 10 11 15 16 19 20 22 32 33 35 37
Neurodegenerative disorders 2.1 Alzheimer’s disease (AD) 2.2 Frontotemporal lobar degenerations (FTLD) 2.3 Motor neurone disease (MND), amyotrophic lateral sclerosis (ALS) 2.4 Parkinson’s disease dementia (PDD) and dementia with Lewy bodies (DLB)
39 40 49 63
69
vii
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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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Contents
2.5 2.6 3
4
5
Prion diseases Mild cognitive impairment (MCI)
Cerebrovascular disease: vascular dementia and vascular cognitive impairment 3.1 Cortical vascular dementia, multi-infarct dementia (MID), post-stroke dementia 3.2 Subcortical vascular dementia, Binswanger’s disease, lacunar state, subcortical ischaemic vascular disease (SIVD) 3.3 Strategic infarct dementia, strategic strokes 3.4 Subarachnoid haemorrhage (SAH) 3.5 Intracranial vascular malformations 3.6 Vasculopathies 3.7 Other cerebrovascular disorders
82 87
6.3 6.4 6.5 6.6 6.7 6.8 6.9 6.10 6.11
90
93
95 97 101
6.12 6.13
104 106 112
The epilepsies 4.1 Epilepsy and cognitive impairment 4.2 Cognitive decline and epilepsy: shared aetiology 4.3 Seizures causing acquired cognitive impairment 4.4 Antiepileptic drug therapy causing cognitive impairment 4.5 Treatment of cognitive problems in epilepsy
115 115
Neurogenetic disorders 5.1 Hereditary dementias 5.2 Hereditary ataxias 5.3 Hereditary spastic paraplegia 5.4 Hereditary movement disorders 5.5 Hereditary metabolic disorders 5.6 Hereditary neurocutaneous syndromes (phakomatoses)
125 126 135 140 141 146
6.14 6.15
Inflammatory, immune-mediated, and systemic disorders 6.1 Multiple sclerosis (MS) 6.2 Acute disseminated encephalomyelitis (ADEM)
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166 168 169 170 170 171 171
173 174 176 176 177
Structural brain lesions 7.1 Brain tumours and their treatment 7.2 Hydrocephalic dementias 7.3 Other structural lesions
178 178 182 186
8
Endocrine, metabolic, and toxin-related disorders 8.1 Endocrine disorders 8.2 Metabolic disorders 8.3 Toxin-related disorders
188 188 193 200
120 122
9
Infective disorders 9.1 Encephalitides and meningoencephalitides 9.2 Meningitides 9.3 Human immunodeficiency virus (HIV) and related conditions 9.4 Other disorders of infective aetiology
204
Neuromuscular disorders 10.1 Myotonic dystrophy (Steinert disease) 10.2 Myasthenia gravis (MG)
215
205 209 210 211
155 10
6
165
7
116
123
Sarcoidosis Systemic lupus erythematosus (SLE) Sjo¨gren’s syndrome Behc¸et’s disease Rheumatoid arthritis (RhA) Scleroderma, systemic sclerosis Relapsing polychondritis Cerebral vasculitides Sydenham’s chorea, paediatric autoimmune neuropsychiatric disorders associated with streptococcal infections (PANDAS) Limbic encephalitis Hashimoto’s encephalopathy (HE) Erdheim–Chester disease Bilateral vestibulopathy
157 157 165
Index
215 216 218
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Cambridge University Press 978-0-521-71792-2 - Neuropsychological Neurology: The Neurocognitive Impairments of Neurological Disorders A. J. Larner Frontmatter More information
Acknowledgements
First and foremost I thank my wife Philippa, and children Thomas and Elizabeth, for their forbearance 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.
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