Neuro-Ophthalmology
Desmond P. Kidd Neuro-Ophthalmology Illustrated Case Studies
Desmond P. Kidd Department of Clinical Neurosciences Royal Free Hospital London UK ISBN 978-1-4471-2409-2 DOI 10.1007/978-1-4471-2410-8 Library of Congress Control Number: 2017933279 ISBN 978-1-4471-2410-8 (ebook) Springer-Verlag London Limited 2017 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 This Springer imprint is published by Springer Nature The registered company is Springer-Verlag London Ltd. The registered company address is: 236 Gray s Inn Road, London WC1X 8HB, United Kingdom
For Catherine and Iain
Foreword This is an educational entertainment, not a textbook. Neurologists and ophthalmologists often approach neuro-ophthalmology with apprehension since it requires a little extra learning to understand the principles, yet having done so it proves to be not only reasonably straightforward but also remarkably fascinating and stimulating. The purpose here, therefore, is to allow the reader to acquire extra knowledge about the subject relatively easily and without the need to spend hours of study, simply by reading a number of clinical cases. Each case is set out in a realistic scenario, with the history and examination first, then a pause for consideration leading to investigations. The results of these are set out and where necessary a further pause follows to allow the reader to reflect on the information provided before the denouement arrives. A short summary of important points which allow an understanding of where our knowledge of the pathogenesis and treatment of the disease currently exists finishes each case. There are 50 cases, each of which is real and none of which has been altered, which have been picked because they are interesting or provide good examples of the clinical or radiological or pathological aspects of the disease, and allow a reasonably comprehensive run through the major topics of neuro-ophthalmology. We start at the orbit and work backwards. There is also an appendix which gives background information if required, and tips on how best to perform the neuro- ophthalmic examination. I wish to thank the production team at Springer Verlag, in particular Joanna Renwick, who was very patient as the project evolved haphazardly from a multiauthor collaboration to the work of an overworked and rather harassed but nonetheless very keen neuro-ophthalmologist. vii
Part I The Orbit and Muscles Case 1......................................................... 3 History....................................................... 3 Examination................................................... 3 Evaluation.................................................... 4 Investigations.................................................. 4 Discussion.................................................... 5 References.................................................... 7 Case 2......................................................... 9 History....................................................... 9 Examination................................................... 9 Evaluation................................................... 10 Investigations.................................................. 10 Management.................................................. 10 Discussion.................................................... 11 References.................................................... 13 Case 3......................................................... 15 History....................................................... 15 Examination................................................... 15 Evaluation.................................................... 16 Investigations.................................................. 16 Discussion.................................................... 17 References.................................................... 18 Case 4......................................................... 19 History....................................................... 19 Examination................................................... 19 Evaluation.................................................... 20 ix
x Investigation.................................................. 20 Discussion.................................................... 22 References.................................................... 23 Case 5......................................................... 25 History....................................................... 25 Examination................................................... 25 Clinical Evaluation............................................. 26 Family History................................................. 26 Investigations.................................................. 26 Discussion.................................................... 27 References.................................................... 27 Case 6......................................................... 29 History....................................................... 29 Examination................................................... 29 Clinical Evaluation............................................. 30 Investigations.................................................. 30 Management.................................................. 30 Discussion.................................................... 31 References.................................................... 32 Case 7......................................................... 33 History....................................................... 33 Examination................................................... 33 Investigation and Management.................................... 34 Discussion.................................................... 36 References.................................................... 37 Part II The Nerve Case 8......................................................... 41 History....................................................... 41 Examination................................................... 41 Evaluation.................................................... 42 Investigations.................................................. 42 Discussion.................................................... 43 References.................................................... 44 Case 9......................................................... 45 History....................................................... 45 Examination................................................... 45 Investigation.................................................. 47 Clinical Evaluation............................................. 49 Discussion.................................................... 49 References.................................................... 50
xi Case 10........................................................ 51 History....................................................... 51 Examination................................................... 51 Investigation.................................................. 52 Evaluation.................................................... 52 Management.................................................. 53 Examination................................................... 53 Further Evaluation and Management............................... 53 Discussion.................................................... 54 References.................................................... 55 Case 11........................................................ 57 History....................................................... 57 Examination................................................... 57 Evaluation.................................................... 58 Examination................................................... 60 Evaluation.................................................... 60 Discussion.................................................... 62 References.................................................... 63 Case 12........................................................ 65 History....................................................... 65 Examination................................................... 65 Evaluation.................................................... 66 Management.................................................. 67 Discussion.................................................... 67 Treatment..................................................... 68 References.................................................... 68 Case 13........................................................ 69 History....................................................... 69 Examination................................................... 69 Evaluation.................................................... 70 Investigations.................................................. 70 Treatment..................................................... 71 Discussion.................................................... 72 References.................................................... 72 Case 14........................................................ 73 History....................................................... 73 Examination................................................... 73 Evaluation.................................................... 74 Investigations.................................................. 75 Management.................................................. 78 Discussion................................................... 78 References................................................... 79
xii Case 15....................................................... 81 History...................................................... 81 Examination.................................................. 81 Evaluation................................................... 82 Investigations................................................. 82 Treatment and Progress......................................... 85 Discussion................................................... 85 Reference.................................................... 86 Case 16....................................................... 87 History...................................................... 87 Examination.................................................. 87 Evaluation................................................... 89 Investigations................................................. 90 Discussion................................................... 90 References.................................................... 92 Case 17....................................................... 93 History...................................................... 93 Examination.................................................. 93 Evaluation................................................... 94 Investigations.................................................. 95 Discussion.................................................... 96 References.................................................... 97 Case 18........................................................ 99 History....................................................... 99 Examination................................................... 99 Evaluation................................................... 100 Investigation................................................. 100 Management................................................. 101 Discussion................................................... 101 References................................................... 103 Case 19....................................................... 105 History...................................................... 105 Examination.................................................. 105 Evaluation................................................... 106 Investigations................................................. 107 Management................................................. 108 Discussion................................................... 110 References................................................... 111 Case 20....................................................... 113 History...................................................... 113 Examination.................................................. 113 Evaluation................................................... 114
xiii Investigations................................................. 115 Evaluation................................................... 116 Papilloedema................................................. 116 Pseudopapilloedema........................................... 116 Discussion................................................... 117 References................................................... 118 Part III The Apex to the Saddle Case 21....................................................... 121 History...................................................... 121 Examination.................................................. 121 Clinical Evaluation............................................ 122 Investigations................................................. 122 Discussion................................................... 124 References................................................... 124 Case 22....................................................... 125 History and Examination........................................ 125 Further Investigations.......................................... 126 Further Management........................................... 127 Discussion................................................... 128 References................................................... 128 Case 23....................................................... 131 History...................................................... 131 Examination.................................................. 131 Evaluation................................................... 132 Investigation................................................. 132 Management................................................. 135 Discussion................................................... 136 References................................................... 137 Case 24....................................................... 139 History...................................................... 139 Examination.................................................. 139 Evaluation................................................... 140 Investigations................................................. 140 Management................................................. 140 Discussion................................................... 142 References................................................... 145 Case 25....................................................... 147 History...................................................... 147 Examination.................................................. 147 Initial Investigations........................................... 148 Clinical Course............................................... 148 Clinical Evaluation............................................ 148
xiv Further Investigations.......................................... 149 Management................................................. 150 Discussion................................................... 151 References................................................... 152 Case 26....................................................... 153 History...................................................... 153 Examination.................................................. 153 Evaluation................................................... 154 Investigation................................................. 154 Management................................................. 155 Discussion................................................... 156 References................................................... 158 Case 27....................................................... 159 History and Examination........................................ 159 Discussion................................................... 161 References................................................... 162 Case 28....................................................... 163 History...................................................... 163 Examination.................................................. 163 Investigations................................................. 164 Evaluation................................................... 165 Discussion................................................... 166 References................................................... 168 Case 29....................................................... 169 History...................................................... 169 Neurological Examination....................................... 169 Clinical Evaluation............................................ 170 The Sympathetic Pathway....................................... 171 Investigations................................................. 172 Management................................................. 173 Discussion................................................... 174 References................................................... 174 Case 30....................................................... 177 History...................................................... 177 Examination.................................................. 177 Clinical Evaluation............................................ 178 Investigation................................................. 178 Discussion................................................... 180 References................................................... 180 Case 31....................................................... 181 History...................................................... 181 Examination.................................................. 181
xv Clinical Evaluation............................................ 182 Investigations................................................. 182 Management................................................. 184 Discussion................................................... 185 References................................................... 186 Case 32....................................................... 187 History and Examination........................................ 187 Clinical Evaluation............................................ 189 Discussion................................................... 191 References................................................... 193 Case 33....................................................... 195 History...................................................... 195 Examination.................................................. 195 Evaluation................................................... 196 Investigations................................................. 197 Management................................................. 197 Discussion................................................... 198 References................................................... 198 Part IV The Brain Stem Case 34....................................................... 201 History...................................................... 201 Examination.................................................. 201 Clinical Evaluation............................................ 202 Investigations................................................. 203 Management................................................. 204 Discussion................................................... 204 References................................................... 205 Case 35....................................................... 207 History...................................................... 207 Examination.................................................. 207 Clinical Evaluation............................................ 208 Investigations................................................. 208 Discussion................................................... 209 References................................................... 211 Case 36....................................................... 213 History...................................................... 213 Examination.................................................. 213 Clinical Evaluation............................................ 214 Acquired Pendular Nystagmus................................... 215 References................................................... 216
xvi Case 37....................................................... 217 History...................................................... 217 Examination.................................................. 217 Clinical Evaluation............................................ 218 Investigations................................................. 218 Disease Progression............................................ 219 Discussion................................................... 219 References................................................... 220 Case 38....................................................... 221 History...................................................... 221 Examination.................................................. 221 Clinical Evaluation............................................ 222 Investigation................................................. 222 Discussion................................................... 223 References................................................... 225 Case 39....................................................... 227 History...................................................... 227 Examination.................................................. 227 Evaluation................................................... 228 Vascular Syndromes Involving the Brain Stem... 230 Management................................................. 231 Discussion................................................... 232 References................................................... 232 Case 40....................................................... 233 History...................................................... 233 Examination.................................................. 233 Clinical Evaluation............................................ 234 Investigations................................................. 236 Discussion................................................... 236 References................................................... 237 Case 41....................................................... 239 History...................................................... 239 Examination.................................................. 239 Clinical Evaluation............................................ 240 Investigation................................................. 240 Causes of Downbeating Nystagmus............................... 241 Discussion................................................... 241 References................................................... 241 Case 42....................................................... 243 History...................................................... 243 Examination.................................................. 244
xvii Clinical Evaluation............................................ 244 Horizontal Gaze Palsy.......................................... 245 Skew Deviation............................................... 245 Ocular Tilt Reaction........................................... 245 Investigations................................................. 246 Discussion................................................... 247 References................................................... 248 Part V The Cortex Case 43....................................................... 251 History...................................................... 251 Examination.................................................. 251 Evaluation................................................... 252 Investigations................................................. 252 Management................................................. 255 Discussion................................................... 256 Causes of RCVS.............................................. 257 References................................................... 257 Case 44....................................................... 259 History...................................................... 259 Examination.................................................. 259 Evaluation................................................... 260 Differential Diagnosis.......................................... 260 Investigations................................................. 261 Clinical Course............................................... 262 Discussion................................................... 263 References................................................... 264 Case 45....................................................... 265 History...................................................... 265 Examination.................................................. 265 Clinical Evaluation............................................ 266 Investigations................................................. 266 Discussion................................................... 267 References................................................... 269 Case 46....................................................... 271 History...................................................... 271 Examination.................................................. 271 Clinical Evaluation............................................ 272 Investigations................................................. 272 Management................................................. 273 Discussion................................................... 274 References................................................... 275
xviii Case 47....................................................... 277 History...................................................... 277 Examination.................................................. 277 Evaluation................................................... 279 Investigations................................................. 279 Discussion................................................... 281 Reference.................................................... 281 Case 48....................................................... 283 History...................................................... 283 Examination.................................................. 283 Investigations................................................. 285 Clinical Evaluation............................................ 286 Management................................................. 287 Discussion................................................... 287 References................................................... 288 Case 49....................................................... 289 History...................................................... 289 Examination.................................................. 289 Evaluation................................................... 293 Discussion................................................... 294 References................................................... 295 Case 50....................................................... 297 History...................................................... 297 Examination.................................................. 297 Clinical Evaluation............................................ 298 Imaging..................................................... 299 References................................................... 301 Appendix 1.................................................... 303 Visual Acuity................................................. 303 Visual Field Examination... 304 Appendix 2.................................................... 307 Examination of the Pupils... 307 Pharmacological Testing... 309 Appendix 3.................................................... 313 The Ocular Motor Nerves... 313 Causes of Ocular Motor Paresis... 314 Examination of Diplopia... 316 Superior Orbital Fissure, Orbital Apex and Cavernous Sinus Syndromes... 318
xix Appendix 4.................................................... 321 The Visual Pathway and Cortical Apperception of Vision... 321 Disorders of the Ventral Pathway... 324 Disorders of the Dorsal Pathway... 326 Positive Visual Phenomena... 328