Electrophysiologic Testing in Disorders of the Retina, Optic Nerve, and Visual Pathway / Edition 2

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An introduction and reference for student and professional ophthalmologists, covering the fundamentals of electrophysiology and its application in various retinal, optic nerve, and visual pathway disorders. Beginning sections of each chapter include information on techniques of recording and principles of photoreceptor, retinal pigment epithelial cell, or visual pathway physiology as they are reflected in the various components of the electroretinogram, electro-oculogram, and visually evoked potential. Chapters then provide information on electrophysiologic findings on various hereditary and acquired disorders of the retina, optic nerve, and visual pathway. Fishman teaches ophthalmology and directs the Electrophysiology Laboratory at the University of Illinois College of Medicine; the three other authors are affiliated with U. of Texas Southwestern Medical Center, Moorfields Eye Hospital in London, England, and Pfizer Global Research and Development, respectively. Annotation c. Book News, Inc., Portland, OR (booknews.com)
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Table of Contents

Preface xiii
Acknowledgments xvi
Chapter 1 The Electroretinogram 1
1-1 Components and Origins of the ERG 2
1-1-1 a-, b-, and c-Waves and Off-Responses 2
1-1-2 Scotopic and Photopic Threshold Responses 6
1-1-3 Early Receptor Potential 7
1-1-4 Oscillatory Potentials 8
1-2 Measurement of the ERG Components 10
1-3 Recording Procedure 11
1-4 Recording Electrodes 12
1-4-1 Burian-Allen Electrode 12
1-4-2 Dawson-Trick-Litzkow Electrode 12
1-4-3 ERG-Jet Electrode 13
1-4-4 Mylar Electrode 13
1-4-5 Skin Electrode 13
1-4-6 Cotton-Wick Electrode 13
1-4-7 Hawlina-Konec Electrode 14
1-5 The ERG Under Light- and Dark-Adapted Conditions 14
1-5-1 With a Constant-Intensity Stimulus 17
1-5-2 With Variable-Intensity Stimuli 18
1-5-3 With Different-Wavelength Stimuli 20
1-5-4 With Variable-Frequency Stimuli 21
1-6 Other Factors Affecting the ERG 23
1-6-1 Duration of Stimulus 23
1-6-2 Size of Retinal Area Illuminated 24
1-6-3 Interval Between Stimuli 24
1-6-4 Size of Pupil 25
1-6-5 Circulation and Drugs 25
1-6-6 Development of Retina 25
1-6-7 Clarity of Ocular Media 27
1-6-8 Age, Sex, and Refractive Error 27
1-6-9 Anesthesia 27
1-6-10 Diurnal Fluctuations 28
1-7 The ERG in Retinal Disorders 28
1-8 Diffuse Photoreceptor Dystrophies 29
1-8-1 Rod-Cone Dystrophies 30
1-8-1-1 Retinitis Pigmentosa 30
1-8-1-2 Allied Disorders 35
1-8-2 Cone-Rod Dystrophies 39
1-9 Stationary Cone Dysfunction Disorders 43
1-9-1 Congenital Achromatopsia 43
1-9-2 Congenital Red-Green Color Deficiency 44
1-10 Stationary Night-Blinding Disorders 45
1-10-1 Congenital Stationary Night Blindness 45
1-10-2 Oguchi Disease 50
1-10-3 Fundus Albipunctatus 50
1-10-4 Fleck Retina of Kandori 53
1-11 Hereditary Macular Dystrophies 54
1-11-1 Stargardt Macular Dystrophy 54
1-11-2 Best Macular Dystrophy 56
1-11-3 Pattern Dystrophies 58
1-11-4 North Carolina Macular Dystrophy 59
1-11-5 Progressive Bifocal Chorioretinal Atrophy 59
1-11-6 Cone Dystrophies 60
1-11-7 Sheen Retinal Dystrophies 64
1-12 Chorioretinal Dystrophies 65
1-12-1 Choroidal Atrophy 65
1-12-2 Gyrate Atrophy 65
1-12-3 Choroideremia 66
1-13 Angioid Streaks 69
1-14 Hereditary Vitreoretinal Disorders 69
1-14-1 X-linked Juvenile Retinoschisis 69
1-14-2 Goldmann-Favre Syndrome 72
1-14-3 Wagner Disease 72
1-14-4 Stickler Syndrome 73
1-14-5 Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy 73
1-14-6 Autosomal Dominant Vitreoretinochoroidopathy 73
1-14-7 Familial Exudative Vitreoretinopathy 74
1-15 Inflammatory Conditions 75
1-15-1 Birdshot Retinochoroidopathy 76
1-15-2 Multiple Evanescent White-Dot Syndrome 78
1-15-3 Acute Zonal Occult Outer Retinopathy 82
1-15-4 Pseudo-Presumed Ocular Histoplasmosis Syndrome 83
1-15-5 Behcet Disease 83
1-16 Circulatory Deficiencies 84
1-16-1 Sickle Cell Retinopathy 84
1-16-2 Takayasu Disease 84
1-16-3 Carotid Artery Occlusion 84
1-16-4 Central and Branch Artery and Vein Occlusions 85
1-16-5 Hypertension and Arteriosclerosis 88
1-17 Toxic Conditions 88
1-17-1 Chloroquine and Hydroxychloroquine 88
1-17-2 Chlorpromazine 90
1-17-3 Thioridazine 91
1-17-4 Indomethacin 93
1-17-5 Quinine 93
1-17-6 Methanol 95
1-17-7 Gentamicin 95
1-17-8 Ethyl-m-Aminobenzoic Acid Methanesulfonate 97
1-17-9 Cisplatin 97
1-17-10 Glycine 97
1-17-11 Canthaxanthin 97
1-17-12 Vigabatrin 98
1-17-13 Deferoxamine 98
1-17-14 Sildenafil 99
1-18 Vitamin A Deficiency and Retinoids 99
1-19 Optic Nerve and Ganglion Cell Disease 100
1-20 Opaque Lens or Vitreous 102
1-21 Diabetic Retinopathy 102
1-22 Miscellaneous Conditions 104
1-22-1 Retinal Detachment 104
1-22-2 Silicone Oil and Sulfurhexafluoride Gas 104
1-22-3 Thyroid and Other Metabolic Dysfunctions 105
1-22-4 Parkinson Disease 106
1-22-5 Myotonic Dystrophy 106
1-22-6 Duchenne and Becker Muscular Dystrophies 106
1-22-7 Kearns-Sayre Syndrome 107
1-22-8 Neuronal Ceroid Lipofuscinoses 110
1-22-9 Retinal Degeneration With Spinocerebellar Ataxia 112
1-22-10 Tay-Sachs Disease 114
1-22-11 Creutzfeldt-Jakob Disease 114
1-22-12 Intraocular Foreign Bodies 115
1-22-13 Myopia 116
1-22-14 Albinism 117
1-22-15 Taurine Deficiency 117
1-22-16 Bietti Crystalline Dystrophy 117
1-22-17 Cancer-Associated Retinopathy 118
1-22-18 Enhanced S-Cone Syndrome 120
1-22-19 Pigmented Paravenous Retinochoroidal Atrophy 120
1-23 Summary 124
References 124
Suggested Readings 152
Chapter 2 The Electro-Oculogram 157
2-1 Recording Procedure 158
2-2 Components and Origins of the EOG 161
2-3 The EOG in Retinal Disorders 163
2-4 Hereditary Macular Dystrophies 163
2-4-1 Stargardt Macular Dystrophy 163
2-4-2 Best Macular Dystrophy 163
2-4-3 Pattern Dystrophies 164
2-4-4 Drusen of Bruch's Membrane 164
2-4-5 Membranoproliferative Glomerulonephritis 164
2-4-6 Ectodermal Dysplasia, Ectrodactyly, and Macular Dystrophy 165
2-5 Diffuse Photoreceptor Dystrophies 165
2-5-1 Rod-Cone Dystrophies 165
2-5-2 Cone and Cone-Rod Dystrophies 165
2-6 Stationary Night-Blinding Disorders 166
2-6-1 Congenital Stationary Night Blindness 166
2-6-2 Oguchi Disease 166
2-6-3 Fundus Albipunctatus 166
2-7 Chorioretinal Dystrophies 166
2-8 Inflammatory Conditions 166
2-9 Circulatory Deficiencies 166
2-10 Toxic Conditions 167
2-10-1 Chloroquine and Hydroxychloroquine 167
2-10-2 Didanosine 167
2-10-3 Deferoxamine 168
2-11 Optic Nerve Disease 168
2-12 Miscellaneous Conditions 168
2-12-1 Retinal Detachment 168
2-12-2 Silicone Oil 168
2-12-3 Diabetic Retinopathy 169
2-12-4 Intraocular Foreign Bodies 169
2-12-5 Myopia 169
2-12-6 Choroidal Malignant Melanoma 169
2-13 Summary 170
References 170
Suggested Readings 174
Chapter 3 The Focal and Multifocal Electroretinogram 177
3-1 The Focal ERG 177
3-1-1 Recording Procedure 178
3-1-2 Normal Values 179
3-1-3 The Focal Rod ERG 179
3-1-4 The Focal Cone ERG in Retinal Disorders 180
3-2 The Multifocal ERG 182
3-2-1 Recording Procedure 183
3-2-2 Normal Values 185
3-2-2 The Multifocal ERG in Outer Retinal Disorders 185
3-2-4 The Multifocal ERG in Inner Retinal Disorders 189
3-3 Summary 192
References 192
Suggested Readings 195
Chapter 4 The Pattern Electroretinogram 197
4-1 Historical Background 197
4-2 The PERG Waveform and Its Determinants 198
4-2-1 The Normal Waveform and Its Relationship to Stimulus Parameters 198
4-2-1-1 Stimulus Rate 199
4-2-1-2 Check Size and Spatial Frequency 199
4-2-1-3 Contrast 200
4-2-1-4 Field Size 200
4-2-2 Recording the PERG 201
4-2-2-1 Recording Electrodes 201
4-2-2-2 The Reference Electrode 202
4-2-2-3 Recording Techniques and Equipment 202
4-2-3 Pupil Diameter 204
4-2-4 Age 204
4-2-5 Reliability of the PERG 204
4-2-6 Origins of the PERG 206
4-3 Clinical Applications 207
4-3-1 Macular Dysfunction 207
4-3-1-1 Macular Assessment in Retinal Degeneration 208
4-3-1-2 Other Generalized Retinal Diseases 208
4-3-1-3 Macular Dystrophies 210
4-3-1-4 Diabetes 213
4-3-1-5 Other Macular Dysfunction 213
4-3-2 Optic Nerve Disease 214
4-3-2-1 Optic Nerve Demyelination 216
4-3-2-2 Optic Nerve Compression 219
4-3-2-3 Optic Atrophy 219
4-3-2-4 Miscellaneous Optic Nerve Dysfunction 221
4-3-3 Glaucoma 221
4-3-4 VEP Abnormalities and the PERG 226
4-3-5 Nonorganic Visual Loss 226
4-4 Summary 227
References 227
Suggested Readings 235
Chapter 5 The Visual Evoked Potential 237
5-1 Origins of the VEP 239
5-2 VEP Stimulus Parameters 240
5-3 VEP Response Parameters 242
5-4 Clinical Use of the VEP in Adults 245
5-4-1 Media Opacities 245
5-4-2 Retinal Diseases 246
5-4-2-1 Central Serous Retinopathy 246
5-4-2-2 Macular Disease 246
5-4-2-3 Glaucoma 246
5-4-3 Demyelinative Optic Neuropathies 246
5-4-3-1 Optic Neuritis 246
5-4-3-2 Multiple Sclerosis 247
5-4-3-3 Leukodystrophies 248
5-4-3-4 Nutritional Amblyopia 248
5-4-4 Compressive Optic Neuropathies 249
5-4-4-1 Dysthyroid Optic Neuropathy 250
5-4-4-2 Idiopathic Intracranial Hypertension 250
5-4-5 Anterior Ischemic Optic Neuropathies 250
5-4-6 Traumatic Optic Neuropathies 251
5-4-7 Toxic Optic Neuropathies 251
5-4-7-1 Ethambutol 251
5-4-7-2 Cisplatin 251
5-4-7-3 Deferoxamine 252
5-4-8 Inflammatory Optic Neuropathies 252
5-4-9 Hereditary Optic Neuropathies 252
5-4-9-1 Autosomal Dominant Optic Atrophy 252
5-4-9-2 Leber Hereditary Optic Neuropathy 252
5-4-9-3 Mitochondrial Myopathies 253
5-4-10 Operating Room Monitoring 253
5-4-11 Neurodegenerative Diseases 254
5-4-11-1 Alzheimer Disease and Other Dementias 254
5-4-11-2 Spinocerebellar Degeneration 254
5-4-12 Functional Disorders 254
5-4-13 Disorders of the Optic Chiasm, Optic Radiations, and Visual Cortex 256
5-4-13-1 The Hemifield VEP and Paradoxical Lateralization 256
5-4-13-2 Chiasmal Compression 257
5-4-13-3 Albinism 257
5-4-13-4 Dysfunction of the Optic Radiations and Visual Cortex 257
5-4-13-5 Migraine 257
5-4-13-6 Developmental Dyslexia 258
5-4-13-7 Cortical Blindness 258
5-5 Clinical Use of the VEP in Children 258
5-5-1 Estimation of Visual Acuity in Infants 259
5-5-2 Childhood Amblyopia and Binocular Function 260
5-5-3 Oculomotor Disorders 262
5-5-4 Delayed Visual Maturation 262
5-5-5 Optic Nerve Hypoplasia 262
5-5-6 Optic Nerve Gliomas 262
5-5-7 Prognostic Value of the VEP in Neurologic Diseases 263
5-6 Practical Guidelines 264
5-6-1 Instrumentation 264
5-6-2 Patient Preparation 264
5-6-3 Adult Protocol 264
5-6-4 Pediatric Protocol 266
5-7 Summary 267
References 268
Suggested Readings 278
CME Credit 281
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