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PRINCIPLES AND PRACTICE OF CLINICAL ELECTROPHYSIOLOGY OF VISION This page intentionally left blank PRINCIPLES AND PRACTICE OF CLINICAL ELECTROPHYSIOLOGY OF VISION Second Edition Editors: John R. Heckenlively and Geoffrey B. Arden Associate Editors: Steven Nusinowitz Graham E. Holder Michael Bach THE MIT PRESS CAMBRIDGE, MASSACHUSETTS LONDON, ENGLAND © 2006 Massachusetts Institute of Technology All rights reserved. No part of this book may be reproduced in any form by any electronic or mechanical means (including photocopying, recording, or information storage and retrieval) without permission in writing from the publisher. MIT Press books may be purchased at special quantity discounts for business or sales promotional use.For information,please email [email protected] or write to Special Sales Department,The MIT Press,55 Hayward Street,Cambridge,MA 02142. This book printed and bound in the United States of America. Library of Congress Cataloging-in-Publication Data Principles and practice of clinical electrophsyiology of vision / editors,John R.Heckenlively and Geoffrey B.Arden ; associate editors,Steven Nusinowitz,Graham E.Holder, and Michael Bach—2nd ed. p.;cm. Includes bibliographical references and index. ISBN 0-262-08346-9 1. Electroretinography. 2. Electrooculography. 3. Visual evoked response. I. Heckenlively,John R. II.Arden, Geoffrey B.(Geoffrey Bernard) [DNLM: 1. Electroretinography. 2. Electrooculography. 3. Electrophysiology. 4. Evoked Potentials,Visual. 5. Vision Disorders—physiopathology.WW 143 P957 2006] RE79.E4P75 2006 617.7¢1547—dc22 2006041876 10 9 8 7 6 5 4 3 2 1 CONTENTS Foreword xiii Preface to the Second Edition xvii Preface to the First Edition xix Contributors xxi I HISTORY AND BACKGROUND TO MODERN TESTING 1 1 History of the Electroretinogram A.F. R 3 2 History of Electro-Oculography G B.A 11 3 History of Visual Evoked Cortical Testing GF.A.H 15 II ANATOMY OF THE RETINA, PRINCIPLES OF CELL BIOLOGY IN THE VISUAL PATHWAYS: FUNCTIONAL, PHYSIOLOGICAL, BIOCHEMICAL, MOLECULAR, BIOLOGICAL 21 4 The Photoreceptor–Retinal Pigment Epithelium Interface GS.H and L V.J 23 v 5 Membrane Mechanisms of the Retinal Pigment Epithelium OS 37 6 Functional Organization of the Retina H K 47 7 Phototransduction and Photoreceptor Physiology W. CS 65 8 Synaptic Transmission:Sensitivity Control Mechanisms GFand RS 79 9 Structure and Function of Retinal Synapses:Role of Cell Adhesion Molecules and Extracellular Matrix W J. B,TC,MK.M,and DD.H 93 10 Central Disorders of Vision in Humans CA.G 109 III ORIGINS OF SLOW ELECTROPHYSIOLOGICAL COMPONENTS 121 11 Origin and Significance of the Electro-Oculogram GB.A 123 12 Orgins of the Electroretinogram L J. F 139 13 The Origin of the Pattern Electroretinogram MBand MB.H 185 14 The Multifocal Electroretinographic and Visual Evoked Potential Techniques DC.H 197 15 Origin of the Visual Evoked Potentials MFand MB 207 IV EQUIPMENT 235 16 Data Acquisition Systems for Electrodiagnostic Testing C H and SN 237 17 Electrodes for Visual Testing SG. C 245 18 Amplifiers and Special-Purpose Data Acquisition Systems J. V O 255 vi  19 Stimulus Devices,Calibration,and Measurement of Light CH 265 V DATA ACQUISITION 285 20 1. Introduction to the ISCEV Standards MF. M andE Z (        ) 287 2. EOG Standard MF. M andE Z (        ) 289 3. Standard for Clinical Electroretinography MF. M,G E.H,MW.S,and S Y (        ) 290 4. Standard for Pattern Electroretinography MB,M H,G E.H,MF. M, T M,V,and YM (        ) 297 5. Visual Evoked Potentials Standard J. VO,M B,CB,MB, MF. M,AP T,GE.H,and V (        ) 301 6. Guidelines for Basic Multifocal Electroretinography MF. M,DC.H,D K,M K, M W.S,and Y M (        ) 309 21 Multifocal Techniques D Kand SP 319 22 The Pattern Electroretinogram G E.H 341 23 Assessing Infant Acuity,Fusion,and Stereopsis with Visual Evoked Potentials EE.B 353 24 Aging and Pattern Visual Evoked Cortical Potential E A-U 361 25 Aberrant Albino and Achiasmat Visual Pathways: Noninvasive Electrophysiological Assessment P.A and L.J.B 369 26 Clinical Psychophysical Techniques K R.A 399 27 Measurement of Contrast Sensitivity G B.A 413  vii 28 Suppressive Rod-Cone Interaction T E.F 417 29 The Use of Fluorescein Angiography as an Adjunct to Electrophysiological Testing J R.H 423 VI DATA ANALYSIS 429 30 Experimental Design and Data Analyses in Vision Function Testing SN 431 31 Analytical Techniques L.H  T and OE 439 32 Reverse Correlation Methods BR.C and M S.L 461 33 Stimulus-Response Functions for the Scotopic b-Wave AB.F and R.M.H 473 34 Kernel Analysis J. VO 479 35 Measuring the Health of the Human Photoreceptors with the Leading Edge of the a-Wave DC.Hand D G. B 487 VII PRINCIPLES OF CLINICAL TESTING 503 36 Localizing Lesions in the Visual System G E.H 505 37 Paired-Flash ERG Analysis of Rod Phototransduction and Adaptation D R.P 519 38 Hyperabnormal (Supranormal) Electroretinographic Responses J R.H and SN 533 39 Technical Issues in Evaluating Patients for Therapeutic Trials BE,P J. F,and RG. W 541 viii  VIII OTHER PROTOCOLS FOR RECORDING OF ERG AND SLOWER POTENTIALS, TECHNICAL ISSUES, AND AUXILIARY TESTING TECHNIQUES 547 40 Early Receptor Potential GL.F 549 41 Nonphotic Standing Potential Responses:Hyperosmolarity,Bicarbonate, and Diamox Responses K K,JT,and K W 553 42 Direct Current Electroretinogram SE G. N 557 43 The Oscillatory Potentials of the Electroretinogram PLC 565 44 Flicker Electroretinography D G. B 581 45 Chromatic Recordings of Electroretinograms K K,J T,K W,and YS 585 46 Adaptation Effects on the Electroretinogram P G and CM 593 47 Clinical Electrophysiological and Psychophysical Investigations into Color Defects G B.Aand T B 597 48 Causes and Cures of Artifacts G B.A 615 IX PRINCIPLES OF CLINICAL TESTING AND EVALUATION OF VISUAL DYSFUNCTION FROM DEVELOPMENTAL, TOXIC, AND ACQUIRED CAUSES 621 49 Testing Levels of the Visual System J R.H,R G. W,and G B.A 623 50 Effects of High Myopia on the Electroretinogram SN 631 51 Electrodiagnostic Testing in Malingering and Hysteria GE.H 637 52 Developmental Amblyopia D T 643  ix

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This authoritative text is the only comprehensive reference available on electrophysiologic vision testing, offering both practical information on techniques and problems as well as basic physiology and anatomy, theoretical concepts, and clinical correlations. The second edition, of the widely used
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