Добавил:
kiopkiopkiop18@yandex.ru t.me/Prokururor I Вовсе не секретарь, но почту проверяю Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз: Предмет: Файл:

Ординатура / Офтальмология / Английские материалы / Electrodiagnosis of Retinal Disease_Miyake_2005

.pdf
Скачиваний:
0
Добавлен:
28.03.2026
Размер:
7.17 Mб
Скачать

Yozo Miyake

Electrodiagnosis of Retinal Diseases

Yozo Miyake

Electrodiagnosis of

Retinal Diseases

With 258 Figures, Including 93 in Color

Yozo Miyake

Professor Emeritus, Nagoya University

Head of the National Institute of Sensory Organs National Hospital Organization Tokyo Medical Center 2-5-1 Higashigaoka, Meguro-ku, Tokyo 152-8902, Japan

Library of Congress Control Number: 2005932804

ISBN-10 4-431-25466-8 Springer-Verlag Tokyo Berlin Heidelberg New York ISBN-13 978-4-431-25466-9 Springer-Verlag Tokyo Berlin Heidelberg New York

Printed on acid-free paper

This work is subject to copyright. All rights are reserved, 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 other ways, and storage in data banks.

The use of registered names, trademarks, 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.

Product liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature.

Springer is a part of Springer Science+Business Media springeronline.com

© Springer-Verlag Tokyo 2006 Printed in Japan

Typesetting: SNP Best-set Typesetter Ltd., Hong Kong

Printing and binding: Nikkei Printing, Japan

Preface

Soon it will be time for me to retire from my position as professor of ophthalmology in the Department of Ophthalmology, Nagoya University School of Medicine. I have therefore decided to summarize my experience of more than 30 years of studies on the clinical electrophysiology of vision. These studies were performed in our department through the hard work and good ideas of many co-workers, and the chapters of this book cover the rationale and results of our studies. Because excellent textbooks on clinical electroretinography already exist, my wish is not to write another textbook; instead, this book is in the form of essays that include my beliefs and philosophy on the clinical electrophysiology of vision. As such, this book does not include every clinical disease but only those that we have identified or studied in detail.

In addition to full-field electroretinography (ERG), we developed the techniques and instrumentation to record focal macular ERG more than two decades ago. Using these techniques, we were able to determine several new physiological properties of the normal macula, many new pathophysiological mechanisms of known diseases, and some new clinical entities with unique functional properties.

Fortunately, the recent advances of macular surgery and imaging are such that the macular configuration can easily be determined. During the process of surgery, we have obtained considerable information on the correlation of macular function and macular morphology using focal macular ERGs and optical coherence tomography (OCT). The development of multifocal ERGs followed our focal macular ERGs, and this technique has allowed the objective examination of macular function throughout the world.

There has been no similar occasion in the past where the advancement of technology has allowed us to discover such correlations and, more importantly, to determine the mechanisms for many retinal diseases. As one who has dedicated his life to this area, I hope that this book will inspire young researchers and clinicians to enjoy the pleasure and fulfillment that can come from studying the retina. Most importantly, there are many more challenges that have to be taken on and conquered.

Yozo Miyake

Nagoya

March 2005

Acknowledgments

There are many people who have helped make this book a reality; however, some merit special attention. Dr. Hiroko Terasaki has contributed very much with her skills in the new surgical procedures and in psychophysical measurements in normal subjects and patients with retinal diseases who underwent retinal surgery. Dr. Mineo Kondo has always been with me to analyze the clinical data and to perform the animal experiments during the past 10 years. Dr. Masayuki Horiguchi worked with me for a long time before he moved to Fujita Health University as chairman and professor of the Department of Ophthalmology. He had many unique ideas and contributed greatly to our studies. Dr. Yoshihiro Hotta opened a new field of molecular genetics in our department and Dr. Makoto Nakamura has provided important molecular genetic data of our patients. It would not have been possible to complete this book without the invaluable contribution of these investigators.

Special thanks go to Dr. Tatsuo Hirose of Boston,who was a great inspiration to me and who also pointed out the importance of focal macular ERGs when I was a young investigator. I learned basic retinal electrophysiology from Dr. Genyo Mitarai in Nagoya, and he has been an important teacher in my life.

Finally, I would like to express my sincere gratitude to Dr. Duco Hamasaki of Miami, my close friend, for his kind revision and many valuable suggestions regarding the English content of this book. During the past 10 years, he has made an enormous contribution to Japanese ophthalmology by revising many manuscripts written by Japanese investigators that eventually were published in English journals. On behalf of the Japanese Society of Ophthalmology, I would like to thank him again.

Yozo Miyake

Contents

Preface V

Acknowledgments VII

1

Principles and Methods

1

1.1

Full-Field Electroretinograms

2

1.2

Focal Macular ERGs

20

 

1.3

Multifocal ERGs

33

 

1.4

Electrooculography

41

 

1.5

Optical Coherence Tomography

42

2

Hereditary Retinal and Allied Diseases 43

2.1

Retinitis Pigmentosa

44

 

 

2.2

Crystalline Retinopathy (Bietti)

55

 

2.3

Batten Disease

58

 

 

 

2.4

Kearns-Sayre Syndrome

61

 

 

2.5

Choroideremia

64

 

 

 

2.6

Gyrate Atrophy

66

 

 

 

2.7

Enhanced S-Cone Syndrome

68

 

2.8

X-Linked Retinoschisis

72

 

 

2.9

Nettleship-Falls X-Linked Ocular Albinism

87

2.10

Complete and Incomplete Types of CSNB

90

2.11

Fundus Albipunctatus

114

 

 

2.12

Oguchi’s Disease

119

 

 

 

2.13

Cone Dystrophy

123

 

 

 

2.14

Rod Monochromacy

136

 

 

XContents

2.15

Blue Cone Monochromacy

138

 

2.16

Congenital Tritanopia — Differential Diagnosis of

 

Dominantly Inherited Juvenile Optic Atrophy

141

2.17 Rod–Cone Dysfunction Syndrome with an Unusual Form

of ERG

144

2.18 Association of Negative ERG with Diseases of Unknown

 

Etiology

147

 

 

 

2.19

Occult Macular Dystrophy

153

 

2.20

Stargardt’s Disease (Fundus flavimaculatus)

160

2.21

Best’s Disease

165

 

 

3

Acquired Retinal Diseases

169

3.1

Diabetic Retinopathy 170

 

3.2

Retinal Circulatory Disturbances

180

3.3

Retinal and Choroidal Detachment

183

3.4

Inflammatory Diseases of Retina and Choroid 186

4

Acquired Macular Diseases

 

199

 

4.1

Central Serous Chorioretinopathy

200

 

 

4.2

Aphakic or Pseudophakic Cystoid Macular Edema

203

4.3

Idiopathic Epimacular Membranes

209

 

 

4.4

Foveal Thickness and Focal Macular ERG

213

 

4.5

Idiopathic Macular Hole

214

 

 

 

4.6

Macular Pseudohole

221

 

 

 

4.7

Age-Related Macular Degeneration

223

 

 

Subject Index

233

Principles and Methods1

The purpose of this chapter is to present the principles and techniques used to perform clinical electroretinography—electroretinograms (ERGs) and electrooculograms (EOGs)—and to show how they are incorporated into the examination of patients. Some special proper-

ties and the origin of ERG responses are shown for normal subjects to demonstrate the appearance and properties of normal ERGs. These observations should provide a foundation for the better understanding of abnormal ERGs in the analysis of clinical cases.

21 Principles and Methods

1.1 Full-Field Electroretinograms

The development and advancement of clinical electroretinography (ERG) were the consequence of a better understanding of the cellular origins of the major components of the ERG initially demonstrated by Granit in 1934 [1]; the progressive improvement of the recording devices, as shown by introduction of the contact lens electrode by several investigators such as Riggs [2], Karpe [3], and Burian and Allen [4]; and the development of computers for improving the signal-to-noise ratio by averaging techniques.

Many Japanese investigators have contributed significantly to full-field clinical ERG. Among the representative studies are the method for distinguishing cone from rod responses pioneered by Motokawa and Mita [5], the study of human oscillatory potentials (OPs) by Yonemura et al. [6], and the study of photopic ERG by Nagata [7]. In 1968 when I became an ophthalmologist, I thought that the major works on clinical ERG had already been done. However, looking back on the past 37 years, it is now realized that many advances in clinical ERG have taken place since then that have contributed greatly not only to the electrophysiology of the eye but to the understanding of many ophthalmological diseases.

The human ERG recorded at the cornea and elicited by a full-field stimulus is a mass response generated by cells across the entire retina. To obtain reproducible amplitudes and implicit times in the responses, the stimulus and background light should be homogeneous and cover the entire retina, so all of the receptors are stimulated or adapted in a relatively homogeneous manner [8, 9]. The full-field, or

Ganzfeld, stimulator represents such a stimulus. It is composed of a large-diameter (40cm) hemispheric dome (Fig. 1.1), with a xenon stroboscopic light bulb placed at the top of the dome. This configuration allows presentation of diffuse and homogeneous stimuli and background illumination to the entire retina. This stimulus system has been recommended by the International Society of Clinical Electrophysiology for Vision (ISCEV) Standards Committee for use when obtaining clinical ERG recordings [10], and the ISCEV protocol is now being used internationally. The establishment of standardized recording conditions for full-field ERGs was an important accomplishment for the ISCEV because it allowed us to make reasonable comparisons of the ERGs recorded in any country throughout the world that record ERGs using ISCEV standards.

Fig. 1.1. Ganzfeld (full-field) dome for full-field electroretinography (ERG) recordings