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It furthers the University’s objective of excellence in research, scholarship, and education by publishing worldwide. Oxford is a registered trade mark of Oxford University Press in the UK and in certain other countries © Oxford University Press 2012 The moral rights of the authors have been asserted First Edition published 2007 Second Edition published 2012 Impression: 1 All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, without the prior permission in writing of Oxford University Press, or as expressly permitted by law, by licence or under terms agreed with the appropriate reprographics rights organization. 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Dedication For our families: Pip, Beth, and Ellen; Judith, Sarah, Michael, and Jennifer And from all the contributors: Thanks to our own families, and those we meet through our work, who support us and teach us so much. vi Preface to the second edition The very gratifying response to the fi rst edition of this book has justifi ed our belief in the value of a team approach: contributors with their fi ngers on the pulse of advances in our fi eld, steadied at the helm by two editors with experience and perspective. It has, again, been a great privilege: never has Lord Acton’s advice to ‘learn as much from writing books as from reading them’ been better heeded! We have appreciated the very constructive suggestions for improvement of the fi rst edition and followed them where we can. We have added respiratory consults to Chapter 5, and included more neuroradiology, diagrams, and images in situations where they offer clarity. A section on late-onset metabolic disease is added with an emphasis on how this group of disorders might catch us out. With an ever-increasing list of genes and autoantibodies to think about it is important to remember those everyday skills we carry, honed as juniors in our specialty: listening to what is truly being said, careful clinical examination, focused investigation, and above all the communication of understanding, reassurance, and hope to families and young people facing challenges they never dreamed existed. You will fi nd due emphasis on this in the text. We trust this book will become a trusted companion. Please continue to tell us how it can be improved! RF RN 2012 vii Preface to the fi rst edition Medicine is a communal discipline, and this book has benefi ted greatly from being written in community by trainees (who remember the recent struggle to grasp a complex area) and older colleagues who can add par- ticular emphases and perspective. We have striven to provide a combina- tion of practical advice on clinical approach, and ‘at a glance’ oversights and aides-memoire to topic areas. We also wanted to address a number of practical issues that occupy a lot of time in practice, but that are rarely addressed in more conventional textbooks. RF RN 2007 viii Acknowledgements We are grateful to colleagues Hilary Cass and Chris O’Brien for their con- tributions to the sections on b p. 114 and b p. 254, respectively. Robert MacFarland reviewed the section on mitochondrial disease and Simon Bailey commented on the CNS tumour section. We thank Susan Kafka, Clinical Pharmacist at Royal Manchester Children’s Hospital for her expert review of the pharmacopoeia. Stavros Stivaros, also of Royal Manchester Children’s Hospital provided some MR images. Hilary Reidpath, Senior Technician in the Neurophysiology Department at the Royal Hospital for Sick Children Glasgow and Dr Ming Lai, Consultant Neurophysiologist at the Royal Victoria Infi rmary, Newcastle kindly supplied EEG illustrations. We are very grateful to Nuno Cordeiro, Christian de Goede, and Omar Kwaja who contributed so generously to the fi rst edition. We are particularly grateful to Anna Basu, Ram Kumar, and Kate Riney for detailed proof-reading but any remaining errors are of course our responsibility. RF RN 2012 ix Contents Detailed contents xi Contributors xv Symbols and abbreviations xvi 1 Clinical approach 1 2 Neurodiagnostic tools 51 3 Signs and symptoms 103 4 Specifi c conditions 209 5 Consultation with other services 469 6 Emergencies 533 7 Pharmacopoeia 573 Index 621 This page intentionally left blank xi Detailed contents Contributors xv Symbols and abbreviations xvi 1 Clinical approach 1 The consultation 2 What, where, and when 4 History taking 6 Examination 8 Higher cognitive function 13 Cranial nerves 16 Peripheral nervous system 25 Neonatal neurological examination 41 Real world examination sequences 45 Synthesis 49 2 Neurodiagnostic tools 51 Principles of investigation 52 Principles of neuroradiology 54 Neuroradiological anatomy 64 Brainstem anatomy 69 Principles of neurophysiology 70 Peripheral neurophysiological tests 76 Neurophysiological testing of central sensory pathways 82 Specialist investigations 85 Practical procedures 94 Neuropsychological testing 99 3 Signs and symptoms 103 Agitation and confusion 104 Back pain 106 Behaviour disorders 107 Developmental impairment 109 Exercise limitation and muscle pain 117 Eye movement abnormalities 119 xii DETAILED CONTENTS Facial movement abnormalities 126 Facial sensation abnormalities 130 The fl oppy infant 132 Foot deformities 135 Funny turns: episodic events 136 Funny turns: likely epilepsy? 141 Gait abnormalities 144 Head shape abnormalities 146 Head size abnormalities 148 Headache 151 Hearing loss 155 Incontinence 157 Movement