PRINCIPLES AND PRACTICE
OF CLINICAL
ELECTROPHYSIOLOGY
OF VISION
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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.
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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
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CONTENTS
Foreword |
xiii |
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Preface to the Second Edition |
xvii |
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Preface to the First Edition |
xix |
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Contributors |
xxi |
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I HISTORY AND BACKGROUND TO
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MODERN TESTING |
1 |
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1 |
History of the Electroretinogram |
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A. F. R |
3 |
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2 |
History of Electro-Oculography |
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G B. A |
11 |
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3 |
History of Visual Evoked Cortical Testing |
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G F. A. H |
15 |
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IIANATOMY 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 |
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O S |
37 |
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6 |
Functional Organization of the Retina |
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H K |
47 |
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7 |
Phototransduction and Photoreceptor Physiology |
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W. C S |
65 |
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8 |
Synaptic Transmission: Sensitivity Control Mechanisms |
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G F and R S |
79 |
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9Structure 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 |
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10 Central Disorders of Vision in Humans |
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C A. G |
109 |
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III ORIGINS OF SLOW ELECTROPHYSIOLOGICAL COMPONENTS 121
11 |
Origin and Significance of the Electro-Oculogram |
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G B. A |
123 |
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12 |
Orgins of the Electroretinogram |
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L J. F |
139 |
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13 |
The Origin of the Pattern Electroretinogram |
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M B and M B. H |
185 |
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14 |
The Multifocal Electroretinographic and Visual Evoked Potential Techniques |
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D C. H |
197 |
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15 |
Origin of the Visual Evoked Potentials |
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M F and M B |
207 |
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IV |
EQUIPMENT |
235 |
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16 |
Data Acquisition Systems for Electrodiagnostic Testing |
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C H and S N |
237 |
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17 |
Electrodes for Visual Testing |
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S G. C |
245 |
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18 |
Amplifiers and Special-Purpose Data Acquisition Systems |
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J. V O |
255 |
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vi
19 Stimulus Devices, Calibration, and Measurement of Light |
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C H |
265 |
V DATA ACQUISITION |
285 |
20 1. Introduction to the ISCEV Standards
M F. M and E Z (
) |
287 |
2.EOG Standard
M F. M and E 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 (
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) |
309 |
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21 |
Multifocal Techniques |
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D K and S P |
319 |
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22 |
The Pattern Electroretinogram |
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G E. H |
341 |
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23 |
Assessing Infant Acuity, Fusion, and Stereopsis with Visual Evoked Potentials |
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E E. B |
353 |
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24 |
Aging and Pattern Visual Evoked Cortical Potential |
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E A -U |
361 |
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25Aberrant Albino and Achiasmat Visual Pathways: Noninvasive Electrophysiological Assessment
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P. A and L. J. B |
369 |
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26 |
Clinical Psychophysical Techniques |
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K R. A |
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399 |
27 |
Measurement of Contrast Sensitivity |
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G B. A |
413 |
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vii
28 |
Suppressive Rod-Cone Interaction |
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T E. F |
417 |
29 |
The Use of Fluorescein Angiography as an Adjunct to Electrophysiological Testing |
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J R. H |
423 |
VI |
DATA ANALYSIS |
429 |
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30 |
Experimental Design and Data Analyses in Vision Function Testing |
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S N |
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431 |
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31 |
Analytical Techniques |
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L. H T and O E |
439 |
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32 |
Reverse Correlation Methods |
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B R. C and M S. L |
461 |
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33 |
Stimulus-Response Functions for the Scotopic b-Wave |
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A B. F and R . M. H |
473 |
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34 |
Kernel Analysis |
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J. V O |
479 |
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35Measuring 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 |
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36 |
Localizing Lesions in the Visual System |
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G E. H |
505 |
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37 |
Paired-Flash ERG Analysis of Rod Phototransduction and Adaptation |
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D R. P |
519 |
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38 |
Hyperabnormal (Supranormal) Electroretinographic Responses |
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J R. H and S N |
533 |
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39 |
Technical Issues in Evaluating Patients for Therapeutic Trials |
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B E , P J. F , and R G. W 541 |
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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
41Nonphotic Standing Potential Responses: Hyperosmolarity, Bicarbonate, and Diamox Responses
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K K , J T , and K W |
553 |
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42 |
Direct Current Electroretinogram |
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S E G. N |
557 |
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43 |
The Oscillatory Potentials of the Electroretinogram |
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P L C |
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565 |
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44 |
Flicker Electroretinography |
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D G. B |
581 |
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45Chromatic Recordings of Electroretinograms
K K , J T , K W , and
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Y S |
585 |
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46 |
Adaptation Effects on the Electroretinogram |
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P G and C M |
593 |
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47 |
Clinical Electrophysiological and Psychophysical Investigations into Color Defects |
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G B. A and T B |
597 |
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48 |
Causes and Cures of Artifacts |
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G B. A |
615 |
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IX PRINCIPLES OF CLINICAL TESTING AND EVALUATION OF VISUAL DYSFUNCTION FROM DEVELOPMENTAL, TOXIC,
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AND ACQUIRED CAUSES |
621 |
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49 |
Testing Levels of the Visual System |
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J R. H , R G. W , and G B. A |
623 |
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50 |
Effects of High Myopia on the Electroretinogram |
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S N |
631 |
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51 |
Electrodiagnostic Testing in Malingering and Hysteria |
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G E. H |
637 |
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52 |
Developmental Amblyopia |
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D T |
643 |
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ix
53 |
Visual Evoked Potentials in Cortical Blindness |
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E A -U |
651 |
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54 |
Drug Side Effects and Toxicology of the Visual System |
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E Z |
655 |
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55 |
Mitochondrial Diseases |
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A B. H. S and N J. N |
665 |
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X |
EVALUATION OF VASCULAR DISEASES, INFLAMMATORY |
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STATES, AND TUMORS |
673 |
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56 |
Diseases of the Middle Retina: Venous and Arterial Occlusions |
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M A. J |
675 |
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57 |
Acute Disorders of the Outer Retina, Pigment Epithelium and Choroid |
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S E. B |
683 |
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58 |
Autoimmune Retinopathy, CAR and MAR Syndromes |
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J R. H , N A , and G E. H 691 |
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59 |
Ischemic Optic Neuropathy |
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G E. H |
699 |
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XI CLINICAL DESCRIPTIONS: RETINAL PIGMENT |
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EPITHELIUM DISEASES |
703 |
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60 |
Gyrate Atrophy of the Choroid and Retina |
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R G. W |
705 |
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61 |
Dominant Drusen |
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E H , F M , and C W 717 |
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62 |
Stargardt Disease |
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D G. B 727 |
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63 |
Bietti’s Crystalline Dystrophy of the Cornea and Retina |
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R G. W and D J. W 735 |
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64 |
Leber Congenital Amaurosis |
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R K. K 745 |
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65 |
Pattern Dystrophies |
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M F. M |
757 |
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66 |
Best Vitelliform Macular Dystrophy |
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G A. F |
763 |
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x
67 Sorsby’s Fundus Dystrophy |
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M P. C and K W. M |
769 |
XII DISEASES OF THE OUTER RETINA |
775 |
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68 |
Choroideremia |
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I M. M D and M C. S 777 |
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69 |
Retinitis Pigmentosa |
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D G. B |
781 |
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70 |
Cone Dystrophies and Degenerations |
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J R. H 795 |
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71 |
Vitamin A Deficiency |
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R E. C |
803 |
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XIII DISEASES OF THE MIDRETINA (INCLUDING NEGATIVE
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WAVEFORM DISEASES) |
807 |
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72 |
Differential Diagnosis of the Electronegative Electroretinogram |
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R G. W and P J. F |
809 |
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73 |
Juvenile X-Linked Retinoschisis |
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P A. S , I M. M D , and N W. K |
823 |
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74 |
Congenital Stationary Night Blindness |
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Y M |
829 |
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75 |
Quinine Retinopathy |
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G E. H |
841 |
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XIV OPTIC NERVE AND CENTRAL NERVOUS SYSTEM DYSFUNCTION 843
76 |
Leber’s Hereditary Optic Neuropathy |
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Y O |
|
845 |
77 |
The Pattern Electroretinogram in Glaucoma and Ocular Hypertension |
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G L. T |
851 |
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78 |
Chiasmal and Retrochiasmal Lesions |
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G E. H |
857 |
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xi
79Optic Nerve and Central Nervous Dysfunctions: Parkinson’s Disease and Multiple Sclerosis
I B -W and A A |
867 |
80Diseases of Fatty Acid Storage and Metabolism: Neuronal Ceroid Lipofuscinoses and the Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency
D G. B and R G. W |
889 |
XV ANIMAL TESTING 897
81 Evaluating Retinal Function in the Mouse Retina with the Electroretinogram
S N and J R. H |
899 |
82Electroretinograms of Dog and Chicken
S P -J , N T , F M -F , and
|
N W. K |
911 |
83 |
Electroretinographic Testing in Larger Animals |
|
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K N |
923 |
84 |
Visual Evoked Potentials in Animals |
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W R |
935 |
Index 949
xii