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Although great care has been taken to provide accurate and current information, neither the author(s) nor the publisher, nor anyone else associated with this publication, shall be liable for any loss, damage, or liability directly or indirectly caused or alleged to be caused by this book. The material contained herein is not intended to provide specific advice or recommendations for any specific situation.

Trademark notice: Product or corporate names may be trademarks or registered trademarks and are used only for identification and explanation without intent to infringe.

Library of Congress Cataloging-in-Publication Data

A catalog record for this book is available from the Library of Congress.

ISBN: 0-8247-4068-8

This book is printed on acid-free paper.

Headquarters

Marcel Dekker, 270 Madison Avenue, New York, NY 10016, U.S.A. tel: 212-696-9000; fax: 212-685-4540

Distribution and Customer Service

Marcel Dekker, Cimarron Road, Monticello, New York 12701, U.S.A. tel: 800-228-1160; fax: 845-796-1772

World Wide Web http:==www.dekker.com

The publisher offers discounts on this book when ordered in bulk quantities. For more information, write to Special Sales=Professional Marketing at the headquarters address above.

Copyright # 2005 by Marcel Dekker. All Rights Reserved.

Neither this book nor any part may be reproduced or transmitted in any form or by any means, electronic or mechanical, including photocopying, microfilming, and recording, or by any information storage and retrieval system, without permission in writing from the publisher.

Current printing (last digit):

10 9 8 7 6 5 4 3 2 1

PRINTED IN THE UNITED STATES OF AMERICA

Preface

This book was written to provide the clinician with practical information of visual electrophysiologic tests in an accessible and understandable format. Personnel involved in electrophysiologic testing and ophthalmic trainees may also find this book beneficial. The book is organized into two sections keeping in mind that the majority of clinical users of visual electrophysiologic tests do not perform the tests themselves. The first section consists of six chapters that discuss clinical recording techniques and physiologic origins of electroretinogram (ERG), electro-oculogram (EOG), and visual evoked potential (VEP); the section ends with a chapter that focuses on the effects of maturation and aging on these tests as well as electrophysiologic testing in infants. The second section consists of 11 chapters dealing with the clinical applications of ERG, EOG, and VEP testing. The first chapter of this section is an overview from a clinical perspective. The electrophysiologic findings of specific clinical conditions are discussed in subsequent chapters with an emphasis on when and why a specific test should be considered. The clinician

iii

iv

Preface

will find the second section particularly useful in everyday patient care.

ACKNOWLEDGMENTS

The completion of the book wouldn’t have been possible without the support of my family and colleagues. Dr. Mu Liu assisted immensely in many of the figures and tables. Rick Stratton helped substantially in the preparation of figures. Ailin Rodriguez provided excellent secretarial support. Dr. Sheridan Lam reviewed drafts and gave valuable advice.

I wish to thank my family for their support and encouragement during the writing of this book. In particular, I deeply appreciate Diane, my lifetime soul mate and best friend, for her loving sustenance. The book is dedicated to my family.

Byron L. Lam

Contents

Preface . . . . iii

I. Clinical Recording Techniques

1.Full-Field Electroretinogram . . . . . . . . . . . . . . . . 1

Clinical Use of Full-Field ERG . . . . 1 Retinal Electrical Responses . . . . 6 Clinical Recording of Full-Field ERG . . . . 6

Physiologic Origin of the Full-Field ERG . . . . 20

ERG Fluctuation Related to Circadian Rhythm . . . . 24 Negative ERG—Selective Reduction

of b-Wave . . . . 24

Advanced Clinical Full-Field ERG Topics . . . . 25 Specialized ERG Recordings and Waveforms . . . . 38 Basic Retinal Anatomy and Physiology as

Related to ERG . . . . 47

2. Focal and Multifocal Electroretinogram . . . . . 65

Focal Electroretinogram . . . . 67

Multifocal Electroretinogram . . . . 68

v

vi

Contents

Multifocal ERG Recording Environment and Patient Set-Up . . . . 70

Multifocal ERG Stimulus . . . . 71

Recording Multifocal ERG . . . . 71

First-Order ‘‘Response’’ of Multifocal ERG . . . . 74 Displaying Multifocal ERG Results . . . . 78 Physiologic Blind Spot in Multifocal ERG . . . . 79 Second-Order ‘‘Response’’ of the Multifocal ERG . . . . 81 Specialized Multifocal ERG Techniques and

Waveforms . . . . 83

3.Pattern Electroretinogram . . . . . . . . . . . . . . . . . 91

Clinical Utility of Pattern ERG . . . . 91 Basic Concepts and Physiologic Origins of

Pattern ERG . . . . 93

Clinical Recording of Pattern ERG . . . . 98 Reporting Pattern ERG Results . . . . 101

4.Electro-oculogram . . . . . . . . . . . . . . . . . . . . . . . . 105

Physiologic Origins and Characteristics of EOG . . . . 105

Clinical EOG Recording—Patient Set-Up . . . . 111 Obtaining Light Peak and Dark Trough

EOG Amplitudes . . . . 114

EOG Amplitude Ratio—Arden Ratio . . . . 116 Alternative EOG Method: Light-Peak to Dark-Adapted

Baseline Amplitude Ratio . . . . 117 Reporting the EOG Result . . . . 117 Non-photic EOG Responses . . . . 118 Fast Oscillations of the EOG . . . . 118

5.Visual Evoked Potential . . . . . . . . . . . . . . . . . . . 123

Clinical Utility of VEP . . . . 123 Specialized VEP Techniques . . . . 139

6.Maturation, Aging, and Testing in Infants . . . . 151

Maturation . . . . 152

Delayed Visual Maturation . . . . 156 Electrophysiologic Testing in Infants . . . . 156

Contents

vii

Estimating Visual Acuity in Infants . . . . 159

Amblyopia . . . . 165

Aging . . . . 167

Gender . . . . 172

II. Clinical Applications

7.Overview: Clinical Indications and

Disease Classification . . . . . . . . . . . . . . . . . . . . . 185

Clinical Indications of Visual Electrophysiologic Tests . . . . 185

Disease Classification . . . . 188

8.Retinitis Pigmentosa and Pigmentary Retinopathies . . . . . . . . . . . . . . . . . . . . . . . . . . . 191

Retinitis Pigmentosa (Rod–Cone Dystrophy) . . . . 192 Leber Congenital Amaurosis . . . . 203

Usher Syndrome . . . . 204 Bardet–Biedl Syndrome . . . . 206 Refsum Syndrome . . . . 208 Abetalipoproteinemia (Bassen–Kornzweig

Syndrome) . . . . 209

Neuronal Ceroid Lipofuscinosis . . . . 210 Kearns–Sayre Syndrome: Mitochondrial

Retinopathy . . . . 213 Rubella Retinopathy . . . . 214 Syphilitic Retinopathy . . . . 214

Enhanced S-Cone Syndrome . . . . 215 Goldmann–Favre Syndrome . . . . 218

Dominant Late-Onset Retinal Degeneration . . . . 219 Cone–Rod Dystrophy . . . . 219

Alstro¨m Syndrome . . . . 223

9.Stationary Night Blindness and Stationary Cone Dysfunction Disorders . . . . . . . . . . . . . . . . . . . . 243

Stationary Night Blindness Disorders . . . . 244 Stationary Cone Dysfunction Disorders . . . . 261

viii

Contents

10.Macular Disorders . . . . . . . . . . . . . . . . . . . . . . . 277

Age-Related Macular Degeneration . . . . 278 Macular Degeneration—Autosomal Dominant,

Recessive . . . . 279

Central Serous Chorioretinopathy . . . . 280 Doyne Honeycomb Retinal Dystrophy=

Malattia Leventinese . . . . 283 Stargardt Macular Dystrophy—

Fundus Flavimaculatus . . . . 284

Best Vitelliform Macular Dystrophy . . . . 290 Cone Dystrophy . . . . 293

Central Cone Dystrophy (Occult Macular Dystrophy) . . . . 295

Peripheral Cone Dystrophy . . . . 297 Cone Dystrophy with Supernormal and Delayed Rod ERG (Supernormal and

Delayed Rod ERG Syndrome) . . . . 297 Sorsby Fundus Dystrophy . . . . 298 Pattern Dystrophy . . . . 302

X-Linked Retinoschisis . . . . 303

Central Areolar Choroidal Dystrophy . . . . 307 North Carolina Macular Dystrophy

(Central Areolar Pigment Epithelial Dystrophy) . . . . 308

Progressive Bifocal Chorioretinal Atrophy . . . . 309 Fenestrated Sheen Macular Dystrophy . . . . 309 Familial Internal Limiting Membrane

Dystrophy . . . . 310

11.Chorioretinal Disorders . . . . . . . . . . . . . . . . . . 331

Choroideremia . . . . 331 Gyrate Atrophy . . . . 334

Hereditary Choroidal Atrophy . . . . 336 Helicoid Peripapillary Chorioretinal

Degeneration . . . . 337

Pigmented Paravenous Retinochoroidal Atrophy . . . . 339

Contents

ix

12.Vitreoretinal Disorders . . . . . . . . . . . . . . . . . . . 343

Stickler Syndrome . . . . 344 Wagner Vitreoretinopathy . . . . 345

Familial Exudative Vitreoretinopathy . . . . 346 Autosomal Dominant

Vitreoretinochoroidopathy . . . . 347 Autosomal Dominant Neovascular Inflammatory

Vitreoretinopathy . . . . 347

13.Inflammatory and Immune-Related Ocular Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351

Inflammatory Retinal Disorders . . . . 352 Zonal Inflammatory Retinal Disorders . . . . 360 Paraneoplastic and Immune-Related

Retinopathies . . . . 367

14.Ocular Vascular Disorders . . . . . . . . . . . . . . . . 377

Vascular Occlusions . . . . 379

Other Proliferative Neovascular Disorders . . . . 392 Other Ocular Vascular Disorders . . . . 397

15.Nutritional, Toxic, and Pharmacologic

Effects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407

Vitamin A Deficiency . . . . 408 Nutritional Optic Neuropathy . . . . 410 Metallic Intraocular Foreign Bodies—

Ocular Siderosis . . . . 411 Methanol Poisoning . . . . 414

Synthetic Retinoids—Isotretinoin (Accutane ) . . . . 415 Chloroquine=Hydroxychloroquine . . . . 415 Thioridazine (Mellaril ), Chlorpromazine, and

Other Phenothiazines . . . . 419 Quinine . . . . 421

Deferoxamine (Desferrioxamine) . . . . 425 Vigabatrin . . . . 428

Sildenafil (Viagra ) . . . . 431 Gentamicin . . . . 432 Ethambutol . . . . 433

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