Handbook of Clinical Electrophysiology of Vision

218 Pages·2019·14.227 MB·English

by  Minzhong Yu, Donnell Creel, Alessandro Iannaccone

Handbook of Clinical
Electrophysiology
of Vision
Minzhong Yu
Donnell Creel
Alessandro Iannaccone
Editors
123
Handbook of Clinical Electrophysiology of Vision
Minzhong Yu • Donnell Creel
Alessandro Iannaccone
Editors
Handbook of Clinical
Electrophysiology of Vision
Editors
Minzhong Yu Donnell Creel
Department of Ophthalmology Moran Eye Center
University Hospitals Eye Institute University of Utah School of Medicine
Cleveland, OH Salt Lake City, UT
USA USA
Alessandro Iannaccone
Center for Retinal Degenerations and
Ophthalmic Genetic Diseases
Duke University School of Medicine
Duke Eye Center, Department of
Ophthalmology
Durham, NC
USA
ISBN 978-3-030-30416-4 ISBN 978-3-030-30417-1 (eBook)
https://doi.org/10.1007/978-3-030-30417-1
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Preface
Over the past decade, progress in clinical electrophysiology of vision has revealed
better understanding of the relationship of retinal pathophysiology and the change
of the parameters of electrophysiologic responses and the underlying mechanisms.
This handbook, which includes recent updates of techniques, research, and data
from clinical electrophysiology of vision, was written by Ophthalmic electrophysi-
ologists/vision scientists, and ophthalmologists. We anticipate that clinical research-
ers and vision scientists will be inspired by developments toward the improvement
of diagnoses and drug screening.
Chapters 1, 2, and 3 illustrate the physiologic sources of electrophysiologic
responses, the techniques of the recording, and the analysis of electrophysiologic
signals. Chapters 3, 4, 5, 6, 7, 8, 9, 10, and 11 summarize the characteristics of the
electrophysiologic signals in a number of disorders of the retina and optic nerve,
which will help clinicians select the suitable electrophysiology tests for differentia-
tion and diagnosis of diseases.
We would like to thank Springer for giving us the opportunity to publish this
book. We are also deeply grateful to all of the chapter authors for their extraordinary
contributions. Finally, we thank Xiaoshan Yu, PharmD, RPh, and Feras Mohder,
MD, Board-Certified Internist, for their critical review of Chapters 9 and 10,
respectively.
Cleveland, OH, USA Minzhong Yu, PhD, MMed
Salt Lake City, UT, USA Donnell Creel, PhD
Durham, NC, USA Alessandro Iannaccone, MD, MS, FARVO

v
Contents
Part I B asic Theory of Electrophysiology of Vision
1 Electroretinography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Donnell Creel and Minzhong Yu
2 Visually Evoked Potentials . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19
Donnell Creel and Minzhong Yu
3 Electrooculography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
Donnell Creel and Minzhong Yu
Part II C linical Application of Electrophysiology of Vision
4 Congenital Non-Degenerative Retinal Diseases . . . . . . . . . . . . . . . . . . 37
Wajiha Jurdi Kheir, Roberto Gattegna, Minzhong Yu,
Alessandro Racioppi, Alfonso Senatore, Donnell Creel,
and Alessandro Iannaccone
5 Macular Dystrophies. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
Wajiha Jurdi Kheir, Minzhong Yu, Alfonso Senatore,
Roberto Gattegna, Alessandro Racioppi, Donnell Creel,
and Alessandro Iannaccone
6 Degenerative Night-Blinding Disorders and Cone
and Cone–Rod Dystrophies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
Wajiha Jurdi Kheir, Minzhong Yu, Alfonso Senatore,
Alessandro Racioppi, Roberto Gattegna, Donnell Creel,
and Alessandro Iannaccone
7 Syndromic Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111
Alfonso Senatore, Wajiha Jurdi Kheir, Minzhong Yu,
Alessandro Racioppi, Roberto Gattegna, Donnell Creel,
and Alessandro Iannaccone
vii
viii Contents
8 Characteristics of Visual Electrophysiology
in Inflammatory Disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147
Alessandro Iannaccone, Alfonso Senatore, Wajiha Jurdi Kheir,
Donnell Creel, and Minzhong Yu
9 Characteristics of Visual Electrophysiology
in Retinal Toxicities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173
Minzhong Yu, Alfonso Senatore, Alessandro Iannaccone,
Wajiha Jurdi Kheir, and Donnell Creel
10 Characteristics of Visual Electrophysiology in the Diseases
of Optic Nerve or Visual Pathway . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 191
Minzhong Yu and Donnell Creel
11 Characteristics of Visual Electrophysiology in Albinism . . . . . . . . . . 203
Donnell Creel, Minzhong Yu, and Alessandro Iannaccone
Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215
Contributors
Donnell Creel, PhD Moran Eye Center, University of Utah School of Medicine,
Salt Lake City, UT, USA
Roberto Gattegna, MD Center for Retinal Degenerations and Ophthalmic Genetic
Diseases, Duke University School of Medicine, Duke Eye Center, Department of
Ophthalmology, Durham, NC, USA
Retina Service, Israelitic Hospital, Rome, Italy
Alessandro Iannaccone, MD, MS, FARVO Center for Retinal Degenerations and
Ophthalmic Genetic Diseases, Duke University School of Medicine, Duke Eye
Center, Department of Ophthalmology, Durham, NC, USA
Wajiha Jurdi Kheir, MD Center for Retinal Degenerations and Ophthalmic
Genetic Diseases, Duke University School of Medicine, Duke Eye Center,
Department of Ophthalmology, Durham, NC, USA
Alessandro  Racioppi, BS Center for Retinal Degenerations and Ophthalmic
Genetic Diseases, Duke University School of Medicine, Duke Eye Center,
Department of Ophthalmology, Durham, NC, USA
University of North Carolina, Chapel Hill, NC, USA
Alfonso Senatore, MD Center for Retinal Degenerations and Ophthalmic Genetic
Diseases, Duke University School of Medicine, Duke Eye Center, Department of
Ophthalmology, Durham, NC, USA
Minzhong Yu, PhD, MMed Department of Ophthalmology, University Hospitals
Eye Institute, Cleveland, OH, USA
ix
Part I
Basic Theory of Electrophysiology
of Vision
Chapter 1
Electroretinography
Donnell Creel and Minzhong Yu
Components of ERG
In 1865, Holmgren noticed that there were electrical changes in an amphibian eye
when exposed to light [1]. By 1908, three waves, a, b, and c, of ERG had been iden-
tified. In 1933, Ragnar Granit performed several studies on cat retinae manipulating
levels of anesthetic to isolate different components contributing to the ERG [2]. He
identified the origins of ERG components: a-wave originating from retinal recep-
tors, b-wave from mid-retina (bipolar cells), and c-wave from the retinal pigment
epithelium. The 1967 Nobel Prize for Physiology and Medicine was awarded to
Ragnar Granit for this work.
Granit labeled three components, PI, PII, and PIII, in the order they were extin-
guished with deepening the levels of anesthesia in cat. When stimulating with a
2-second pulse of light, a cornea-positive component PI increases to a maxi-
mum slowly and then decreases slowly without showing an off response. PII is
also a cornea-p ositive component with short latency reflecting the b-wave, which
decreases quickly to a lower positive potential and then shows an off component.
PIII best resists anesthesia in cat. It is a cornea-negative component that quickly
reaches a minimum potential and remains negative until light stimulus ends.
PIII includes a fast negative PIII and a slow negative PIII [3, 4]. The fast PIII,
elicited by the onset of the stimulating light, is from extracellular radial current
of photoreceptors, which is caused by the closure of cGMP-gated cationic chan-
nels when the photoreceptor outer segments receive light [5, 6]. The slow PIII,
maintained when the stimulating light is kept on, is generated by the change of
D. Creel (*)
Moran Eye Center, University of Utah School of Medicine, Salt Lake City, UT, USA
e-mail: [email protected]
M. Yu
Department of Ophthalmology, University Hospitals Eye Institute, Cleveland, OH, USA
© Springer Nature Switzerland AG 2019 3
M. Yu et al. (eds.), Handbook of Clinical Electrophysiology of Vision,
https://doi.org/10.1007/978-3-030-30417-1_1