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Page numbers followed by f or t indicate figures or tables, respectively.
A
AAION. See Arteritic ischemic optic neuropathy
ABCR (ABCA4) gene mutations, 727–732, 784
Abetalipoproteinemia, 803–804
Absorption filters, 271
ac (alternating current) amplifiers, 239, 245, 256–257
Accommodation
aging and, 366, 366f–367f
drug-induced changes in, 661
Acetazolamide responses, 126, 128–130, 130f, 553, 554t, 555f, 562
Acetylcholine, 86
drug/toxic effects on, 657, 657f
Achiasmat visual pathways, 369
Achromatopsia, 112–113, 795–796, 798t, 799–800
Activities of daily living, 542–543
Acute idiopathic enlarged blind spot syndrome, 334
Acute zonal occult outer retinopathy (AZOOR), 687, 688f
Adaptation effects, 593–596. See also Dark adaptation; Light adaptation
“Adaptation pool,” 406
ADC. See Analog-to-digital converter
Adenosine triphosphatase (ATPase) transporters, 39–40
Adhesion, and synapses, 97–105, 99f–100f
Adjustment, method of, 400
Adrenaline, 88
Adrenergic responses, and light peak, 149, 149f
Adult-onset foveomacular vitelliform dystrophies/degeneration (AOFVD), 765, 765t
Age-related macular degeneration
alcohol electro-oculogram in, 135, 135f
color vision in, 608–610, 609f
drusen with, 717–718, 723
multifocal ERG in, 334
Sorsby's fundus dystrophy and, 772
Aging, and visual evoked potentials, 361–367
accommodation changes with, 366, 366f–367f
amplitude changes with, 361, 363f
contrast threshold changes with, 362–364, 365f
gender differences in, 362, 364f
general changes with, 361–362
luminance threshold changes with, 364, 365f
peak latency changes with, 361–362, 364f
pupillary size and, 365
temporal frequency changes with, 362, 363f
Agnosia, 116
apperceptive, 116
associative, 116
object, 116
pure visual, 116
AIPL-1 gene, and Leber congenital amaurosis, 750, 751f
AIR. See Autoimmune retinopathy
Akinetopsia, 113, 113f
Åland Island eye disease (AIED), 810
Albinism
autosomal recessive, 373
brown oculocutaneous, 373
electrophysiological assessment of, 369–395
Forsius-Eriksson ocular, 810
foveal hypoplasia in, 369, 370f
foveation period in, 378–382
functional magnetic resonance imaging in, 374
genetic forms of, 370–373
lateral geniculate nucleus in, 370, 372f
misrouted optic nerve fibers in, 369, 371f–372f, 514–515, 516f
ocular motor disorders in, 377–382
assessment for, 377
concomitant VEP assessment in, 377–382
misalignments and instability, 377–378, 378f–380f
optic chiasm in, 369–370
phenotype of, 369, 370f
tyrosinase-negative, 373
tyrosinase-positive, 373
visual evoked potentials in, 369, 373–395, 373f
age and, 386–396, 390f–394f
averaging of, 380–382, 383f
checkerboard stimulus for, 378–382
data analysis of, 375, 376f
genetic differences in, 386, 387f–389f
hemispheric asymmetry in, 375–377, 376f, 382–395, 385f, 387f–394f
latency of, 382, 384f
methodology for, 375–377
misrouting test of, 369, 375–377, 514–515, 516f
pattern onset/offset, 378–380, 381f–384f
pattern reversal, 378–380, 381f
principal component analysis of, 373–374, 374f, 382–385
stimulus for, 375
topography of, versus normal controls, 382–395
visual pathway anomalies in, 369–374, 371f–372f
X-linked, 373, 386, 387f–389f
Alcohol
electro-oculogram effects of, 126–127, 130–133, 131f–135f
toxicity of, 659
Alexia, pure, 116–117, 117f
Aliasing, 241, 241f, 258, 281, 281f
Allesthesia, visual, 114–115, 115f
Alpha, of Type I error, 433
Alpha rhythm, of EEG, 208, 209f
Alternating current amplifiers, 239, 245, 256–257
Amacrine cell(s), 49, 50f–51f
AII, 58, 60, 63f, 86, 93–95
dopaminergic, 60, 61f, 63f, 88
embryological origins of, 25
function of, 86
GABAergic, 58–60, 59f, 62f
glycinergic, 58, 59f
image properties of, 55–56
neurotransmitters of, 86–88
ON/OFF, 58, 60f
oscillatory potentials from, 568
piggybacking by, 58, 60f
postsynaptic responses of, 85–86, 87f
starburst, 61f, 86
subtypes of, 85–86
Amacrine cell(s) (continued)
synapses of, 57–62, 59f–62f, 85–86
with bipolar cells, 58–60, 60f–61f, 63f, 86, 93–95, 95f
Amantadine, for Parkinson's disease, 879
Amblyopia, 643–647
hysterical, electrodiagnostic testing in, 637
neurophysiology of, 643–644
prevalence of, 643
screening for, 643
subcategories of, 643
visual evoked potentials in, 644–647
binocular, 646–647
binocular summation and facilitation of, 646
dichoptic stimulation of, 646–647
interocular differences in, 644
latent nystagmus and, 645, 645f
patching and, 645, 645f
pattern assessment of, 644–645
threshold measurements of, 646
Vernier acuity and, 646
Ametropia, minimal, spatial contrast loss in, 414, 415f
2-Amino-4-phosphonobutyric acid (APB)
and a-wave, 153–154, 153f
and b-wave, 162, 163f
and multifocal ERG, 332
and oscillatory potentials, 569–570, 570f
and scotopic threshold response, 170
Amnesia, visual, 117
AMPA receptors, 51, 53f–54f
Amplifiers, 238–239, 255–260, 275–278
alternating current (ac), 239, 245, 256–257
bias of, 257
characteristics of, 277–278
common mode rejection ratio of, 238, 257, 277, 559, 617–618
definition of, 275–276
differential, 238, 239f, 257, 275–276, 276f, 617–618, 617f
direct current (dc), 238–239, 256–257, 559
dynamic range of, 257, 277
electrode impedance and, 277, 278f
for ERG, ISCEV standard for, 292
filters for, 239–240, 257–258, 277, 277f
frequency response function of, 257–258, 257f
gain of, 257, 276
input and output impedance of, 257
instrumentation, 275–276
isolation, 255–256, 277–278
linearity of, 257
long-tailed pair design of, 276, 276f
for multifocal ERG, 311, 327–329, 330f
offset of, 238–239, 257
operational design of, 276, 276f
and patient safety, 255–256
for pattern ERG, 299, 341
and phase distortions, 260
phase-sensitive (lock-in), 241, 260–261
sample and hold, 241, 242f
saturation of, and artifacts, 620
for special-purpose systems, 260–263
synchronous, 456–457, 457f–458f
for VEP recording, 229, 304–305
Amplitude characteristic, 440, 441f
Amplitude fluctuations, 456
Analog-to-digital converter (ADC), 240–241, 258–261, 559
aliasing error with, 241, 241f, 258
frequency of, 240–241
resolution of, 240–241
Analytical techniques, 439–457. See also specific types
Anesthetics, for animal electroretinogram, 923–924, 925t–926t, 927
Angiography, fluorescein. See Fluorescein angiography
Angiotensin II antagonist toxicity, 658
Animal testing. See also specific animals and disorders
chicken, 916–919
dog, 911–916, 923–931
larger animal, 923–931
anesthetics for, 923–924, 925t–926t, 927
bilateral ERG in, 931, 931f
ERG-clinical correlation in, 929–931
guidelines/protocols for, 927–929, 928f, 930f
photoreceptor variations in, 923
specific procedures for, 924–927, 927f
monkey, 935–941, 943–944
mouse, 899–907, 935–939, 941–944
multifocal ERG, 331–333
reverse correlation, 465–466, 466f–467f
visual evoked potential, 935–944
Anomaloscope, 599
Anomia
color, 116–117
object, 116–117
Antibiotic toxicity, 657
Anticholinergic drugs, for Parkinson's disease, 879
Anticonvulsant toxicity, 658
Antiepileptic drug toxicity, 658
Anton syndrome, 110–111, 861
APB. See 2-Amino-4-phosphonobutyric acid (APB)
Aphasia, optic, 117
Apperceptive agnosia, 116
Apraxia, ocular motor, 113–114
Arginine restriction, for gyrate atrophy, 711
Aristotle's color theory, 597
Arousal, and visual evoked potentials, 224–226
Arrestin, 68–69, 69f
migration/translocation of, 72–75, 74f
in Oguchi's disease, 836–837
Arterial occlusions, 681. See also Branch retinal artery occlusion (BRAO); Central retinal artery occlusion (CRAO)
Arteritic ischemic optic neuropathy (AAION), 699
clinical presentation of, 699
histology of, 699
treatment of, 699
Artifact(s), 278–282, 615–620
amplifier saturation and, 620
averaging and smoothing, 317, 620
common, 279–280, 279f
definition of, 278
electrode, 245, 278–279, 282, 618–619
frame pulse, 281, 281f
localization of, dummy patient for, 282, 282f, 618–619, 618f
mains interference and, 615–618, 616f
in multifocal ERG, 311, 314, 316–317
in new clinic, 615
patient-related, 279, 619
photovoltaic effect and, 280
rejection of, 242, 261–262, 299, 311
sources of, 278
stroboscope, 619
weak signal, 316f, 317
Associative agnosia, 116
Ataxia, optic, 113–114
ATPase transporters, 39–40
Attention, and visual evoked potentials, 224–226
Autocorrelation, 451, 453f
Autoimmune retinopathy (AIR), 691–697
clinical presentation of, 691
diagnosis of, 695
electroretinogram in, 691, 692f–694f, 695
family histories in, 691–695
future diagnostic techniques in, 697
Average, statistical, 431
Averagers (equipment), 260
Averaging, 455–456
improving signal-to-noise ratio with, 455
ISCEV standards for, 294, 299, 304–305
practical considerations on, 456
response fluctuations and, 455–456
stimulus for, 455
Averaging artifacts, 317, 620
a-wave, 4–5, 557, 558f
abnormal maximal, 506–507
in central retinal artery occlusion, 681
in central retinal vein occlusion, 675–681, 676f–677f, 679f–680f
clinical assessment of, 497–500, 498f–499f
cone response in, 154–155, 154f, 156f, 487, 488f
age and, 493, 494f
high-intensity stimulation of, 487–489
model of, 492, 493f
in retinal disease, 497, 497f
S and Rmax parameters of, 497, 497f
simpler equation for, 492–493
current source density analysis of, 151, 152f
dark-adapted, 150, 150f
depth profile of, 152f
early studies of, 151–152
in juvenile X-linked retinoschisis, 824–825, 826f
laminin defect and, 99
leading edge of, 151–152
photoreceptor health model of, 487–500
alternative models of, 493–494
fitting of, 489–495
goodness-of-fit measure of, 491–492
normative values and repeat reliability of, 493, 494f
in retinal disease, 495–497, 496f–497f
light-adapted, 150, 150f, 593
mouse model of, 902–906, 902f
myopia and, 631–632, 632f
origins of, 149–156, 809
pharmacological dissection studies of, 152–155
postreceptoral contributions to, 152–155, 153f–155f, 491, 492f
receptor photocurrent and, 151–152, 151f–152f
in retinitis pigmentosa, 506–507, 790f, 791
rod response in, 154–155, 154f–155f, 157f, 487–500, 488f, 809
age and, 493, 494f
alternative models of, 493–495, 495f
high-intensity stimulation of, 487–489, 488f–490f
model of, 489–491, 490f–492f
in retinal disease, 495–497, 496f
S and Rmax parameters of, 495–497, 496f
time course of photoreceptor response in, 155–156, 157f
B
Background illumination, for ERG, 292, 593–595, 594f, 595t
Bálint syndrome, 113–114
Band-pass characteristic, 229
Band-pass filter, 257–258, 257f
Band-reject filter, 257–258, 257f
Bandwidth restriction, of oscillatory potentials, 565, 566f, 575–576
Barium (Ba2+)
and b-wave, 159, 162f
and M-wave, 167, 168f
and photopic negative response, 170
Basement membrane, 99, 100f
Basilar laminar drusen
fluorescein angiography in, 536, 538f
hyperabnormal ERG responses in, 536, 538f
Bassen-Kornzweig syndrome, 803–804
Bassoon, in synaptic transmission, 100–103, 102f
Batten-Mayou syndrome, 889
Batten's disease, 890. See also Neuronal ceroid lipofuscinoses
Bayesian approach, 544
BCD. See Bietti's crystalline dystrophy
Becker muscular dystrophy, electroretinogram in, 816
Bessel filter, 239
Bestrophin, 765
Best's disease. See Best vitelliform macular dystrophy
Best vitelliform macular dystrophy, 763–765
c-wave in, 560f, 561
electro-oculogram in, 129f, 130, 134, 505–506, 623–624, 763–765
electroretinogram in, 763, 765
fundus findings in, 763, 764f
genetics of, 765
histology of, 763
multifocal ERG in, 334
vitelliruptive stage of, 763
Beta, of Type II error, 434
Beta rhythm, of EEG, 208, 209f
Bias, in therapeutic trials, 544
Bias, of amplifier, 257
Bicarbonate responses, 128–130, 553, 554t, 555f
Bietti's crystalline dystrophy, 735–742
clinical description of, 735–740
corneal lesions of, 740, 740f
differential diagnosis of, 742
diffuse, 735, 736f–737f
electro-oculogram in, 742
electroretinogram in, 740f, 741–742, 814
fluorescein angiogram of, 423, 424f, 735, 738f–739f
fundus appearance of, 735, 736f, 738f–739f
Goldman perimetric visual fields in, 737f–739f
histopathology of, 741, 741f–742f
historical perspective on, 735
natural history of, 735–740
physiology of, 741
regional or localized, 735, 736f, 740f
relevant testing and findings in, 741–742
stages of, 735–740
Biliary cirrhosis, vitamin A deficiency in, 803, 805f
Binding hypothesis, of visual perception, 881
Bipolar cell(s), 49, 50f–51f
in b-wave generation, 83–84, 84f, 156, 159, 162f, 809
center surround organization of, 54–55, 55f, 80
in color vision, 56–57, 57f
in d-wave generation, 164–165
embryological origins of, 25
glutamate receptors of, 50–52, 53f–54f
image properties of, 52–55
light adaptation in, 84–85, 85f
light response of, 79–80, 80f
neurotransmitters of, 85–88
ON/OFF, 51–57, 53f–54f, 57f, 80–82, 80f–81f
oscillatory potentials from, 568
rod, cGMP cascade in, 82, 82f–83f
Bipolar cell(s) (continued)
in cone dystrophy with mild nyctalopia, 89f, 90–91
in inherited night blindness, 88
in melanoma-associated retinopathy, 88
signal pathways of, 85
synapses of, 50–52, 53f–54f, 57–58, 59f, 79–85, 93–95, 94f–95f
with amacrine cells, 58–60, 60f–61f, 63f, 86, 93–95, 95f
flat contact, 96, 97f
with ganglion cells, 51–52, 54f, 56, 57f, 93–95, 95f
in inner plexiform layer, 93–96, 94f–95f
invaginating, 96, 97f
in outer plexiform layer, 94f
rod versus cone, 96
synaptic gain in, 82
Birdshot chorioretinitis, 685, 687f, 818, 819f
Black box, in linear systems, 439–440, 440f
Blind infant, management of, 751–752
Blindness. See Cortical blindness; Night blindness
Blindsight, 111
Blind spot, in multifocal ERG, 317
Blind spot syndrome, multifocal ERG in, 334
Blue cone monochromatism, 795–796, 800
Blue-sensitive cones, 48, 599, 599f
pathways/synapses of, 56–57, 57f
Boyle, Robert, 599
BPAG1, in synaptic transmission, 103
Branch retinal artery occlusion (BRAO), 334
Branch retinal vein occlusion (BRVO), 680
BRAO (branch retinal artery occlusion), 334
Brodmann's area 18, 19, 109
Bruch's membrane, 27, 99, 100f
eosinophilic deposits on, 769. See also Sorsby's fundus dystrophy
extracellular deposits on, 717. See also Drusen
BRVO (branch retinal vein occlusion), 681
Bull's-eye maculopathy (BEM), 511
Burian-Allen contact lens electrodes, 245–247, 246f
Butterworth filter, 239
b-wave, 5, 557, 558f
abnormal rod-specific, 506–507
background illumination and, 593–595, 594f
barium effects on, 159, 162f
bipolar cell generation of, 83–84, 84f, 156, 159, 162f
in central retinal artery occlusion, 507, 681
in central retinal vein occlusion, 675–681, 676f–678f
chromatic recordings of, 585, 603
cone-driven, 161–162, 603
in cone dystrophy, with mild nyctalopia, 88–90, 89f
in congenital stationary night blindness, 507–510
current source density analysis of, 152f, 156–157, 159f
dc component of, 162–163, 164f
depth profile of, 152f, 156, 158f
dog model of, 912
in flicker ERG, 581, 582f–583f
intracellular recordings of, 156–157
in juvenile X-linked retinoschisis, 824–825, 825f–826f
laminin defect and, 99
light-adapted, 161–162, 163f, 593
light-evoked [K+]0 changes in, 157–159, 160f–161f
mouse model of, 900, 901f, 903, 904f, 906f
Müller cell hypothesis of, 156–157, 159f–160f
myopia and, 631–632, 632f
origins of, 156–163, 809
oscillatory potential relationship with, 569–570
in post-phototransduction dysfunction, 507–510
postreceptoral response in, 475–476, 475f
in retinitis pigmentosa, 506–507, 506f
scotopic (dark-adapted), 160–161, 163f
stimulus-response functions for, 473–476, 474f, 476f
C
Ca2+-ATPase transporter, 39
Cadherins, in synapses, 97–98, 100f
Calcium
in light adaptation, 84–85, 85f
in phototransduction, 71–72
as second messenger, 41f, 42
Calcium-ATPase transporter, 39
Calcium channel(s)
L-type, 38, 42
voltage-dependent, 38, 79
Calcium-dependent chloride channels, 38–39
Calcium-dependent potassium channels, 38
Calmodulin, in phototransduction, 72
Cancer-associated retinopathy (CAR), 691–697
antiretinal antibodies in, 695
electroretinogram in, 692f–693f
Canine. See Dog(s)
CAR. See Cancer-associated retinopathy
Carbamazepine toxicity, 658
Carbon disulfide toxicity, 659
Carbon fiber electrodes, 248f, 249–250
CAR-like syndrome, 691, 694f
Cat(s)
electroretinogram in, 923–931
anesthetics for, 923–924, 925t–926t, 927
correlation with clinical findings, 929–931
specific procedures for, 924–927
photoreceptor diseases in, 923, 924t
Cataract, spatial contrast loss with, 414, 415f
Categorization, in Parkinson's disease, 879–880
β-Catenin, in synapses, 97–98
Cathode-ray tubes (CRTs), 267–269, 267f–268f
frame pulse artifact from, 281, 281f
m-sequence control of, 321–322
for multifocal ERG, 321–322, 327
optimal trigger location and methodology for, 269
output spectra of, 270, 270f
phosphors for, 268, 268f
pitfalls with, 268–269
Ceiling effect, 543
Cell-cell adhesion, and synapses, 97–105, 99f–100f
Center surround organization, 54–55, 55f, 80
dopamine and, 869–870, 869f–871f
Central disorders of vision, 109–117, 111t
Central retinal artery occlusion (CRAO), 681
c-wave in, 561, 561f
electroretinogram in, 507, 681, 816–817
Central retinal vein occlusion (CRVO), 675–681
electroretinogram in, 675–681, 676f–680f, 816–818, 818f
amplitudes in, 675–676, 676f–677f
intensity-response analysis in, 677–678, 680f
temporal factors in, 676–677, 678f–679f
ischemic, 817, 818f
multifocal ERG in, 677
nonischemic, 817
photoreceptor function in, 679–680, 680f
Central serous chorioretinopathy, 683–685
diagnosis of, 683–685
electroretinogram in, 683–685
etiology of, 683
fluorescein angiography of, 683, 684f
multifocal ERG in, 685, 686f
Central serous retinopathy, multifocal ERG in, 334
Central vision disorders, 109–117, 111t
Cerebral achromatopsia, 112–113
CEVNet, 251–252
C-glide electrode, 248f, 249–250
cGMP. See Cyclic guanosine monophosphate
Chebyshev filter, 239
Checkerboard stimulus
in albinism, 378–382
and Fourier analysis, 451, 452f
for pattern ERG, 186, 188f, 191–193, 193f, 298
for VEP stimulation, 17, 215–216, 220
Chiasmal dysfunction (lesions), 857–860
in albinism, 369–370
etiology of, 857
misdiagnosis of, 857
pattern ERG in, 860
visual evoked potentials in, 231, 307, 505, 514–515, 516f, 857–860, 858f–860f
crossed asymmetry in, 858, 858f
for intraoperative monitoring, 859–860
treatment effects on, 859
visual field defects in, 857
Chicken(s)
electroretinogram in, 916–919
anesthetics for, 926t
delayed basal hyperpolarization in, 148, 148f
in disease, 917–918, 917f–919f
long-flash, 917, 917f
methods of recording, 916
normal, 916–917, 917f
Leber congenital amaurosis in, 749
retina of, versus human retina, 916
Chievitz, layer of, 25
Chloride channel(s), 38–39
calcium-dependent, 38–39
CIC-2, 39
in electro-oculogram, 124–126
voltage-activated, 39
Chloride-HCO3- exchanger, 40
Chloroquine toxicity, 655–656
color vision in, 610, 610f
multifocal ERG in, 335
Chondroitin 6-sulfate, in cone matrix sheaths, 26–27, 28f
Choriocapillaris, drug effects on, 655
Choriocapillaris atrophy, c-wave in, 560f, 561
Chorioretinopathy, central serous, 683–685
diagnosis of, 683–685
electroretinogram in, 683–685
etiology of, 683
fluorescein angiography of, 683, 684f
multifocal ERG in, 685, 686f
Choroidal disorders, 683–687. See also specific types
Choroidal fissure, development of, 23
Choroideremia, 777–779
clinical characteristics of, 777
differential diagnosis of, 777–779
electroretinogram in, 777
female carriers of, 777, 778f
fluorescein angiogram of, 423, 424f, 777, 778f
fundus appearance of, 777, 778f
gene function in, 779
versus gyrate atrophy, 712–713, 779
versus retinitis pigmentosa, 777
suppressive rod-cone interaction in, 419
versus Usher syndrome, 779
Chromatic constancy, 112–113, 600
Chromatic responses, 597–610. See also Color vision
early receptor potential in, 585, 589–591, 589f–590f
ERG recordings of, 585–591, 603–604
pattern ERG recordings of, 604
in protan/deutan genetic carriers, 587f, 588–589, 588f–589f
psychophysical tests of, 607–610
rapid off-response, 585–589, 586f–588f
suppressive rod-cone interaction in, 419
VEP recordings of, 218, 604–607, 605f–607f
Chromatic stimuli, visual evoked potentials to, 224
CIC-2 channel, 39
Cilia, of photoreceptors, 65–66, 66f–67f
Cinchonism. See Quinine retinopathy
Cl<συπ>−<?συπ>/HCO3- exchanger, 40
Clinical findings, correlation of test results with, 628–629, 628t
Clinical trials. See Therapeutic trials
CMMR (common mode rejection ratio), 238, 257, 277, 559, 617–618
Coats’ reaction, 427, 428f
Codon 172 RDS-related drusen, 717, 720–721, 720f, 722f
Collagen, in synaptic transmission, 99, 103
Color anomia, 116–117
Color center, human (area V4), 109, 110f, 112f, 600
damage to, syndromes caused by, 112–113
Color confusion line, 602
Color constancy, 112–113, 600
Color contrast, 602–603, 607–608
Color perception, 112, 598–599
Color selectivity, reverse correlation of, 469–470, 469f
Color space modulation, for VEP stimulation, 218
Color triangle, 602
Color vision, 597–610
in age-related macular degeneration, 608–610, 609f
Aristotle's theory of, 597
color contrast test of, 602–603, 607–608
cone specialization for, 47–49, 599–600, 599f
in congenital stationary night blindness, 834
defects or deficiency of
acquired, 600–603
early receptor potential in, 585, 589–591, 589f–590f
electrophysiology of, 585–591, 603–607
genetic carriers of, 587f, 588–589, 588f–589f
genetic mechanisms of, 599f, 600, 601f
inherited, 599–600, 600t, 601f
in males versus females, 599f, 600, 600t
psychophysical tests for, 607–610
rapid off-response in, 585–589, 586f–588f
suppressive rod-cone interaction in, 419
in diabetic retinopathy, 603, 608, 608f
drug/toxic effects on, 610, 610f, 660–661
early receptor potential in, 585, 589–591, 589f–590f
ERG recordings of, 585–591, 603–604
in glaucoma, 608, 609f, 854–855
Goethe's theory of, 597–598
historical perspective on, 597–600
Newton's theory of, 597–598
in optic atrophy, 609–610, 610f
in Parkinson's disease, 872–873
pathways of, 56–57, 57f
pattern ERG recordings of, 604
physiological aspect of, developing of, 597–599
Plato's theory of, 597
Schopenhauer's theory of, 597–599
Color vision (continued)
silent substitution test of, 602
in Sorsby's fundus dystrophy, 769
Stiles two-color technique in, 602
visual evoked potentials in, 218, 604–607, 605f–607f
Coma, visual evoked potentials in, 232
Common mode rejection ratio (CMRR), 238, 257, 277, 559, 617–618
Complex cells, 468
reverse-correlation analysis of, 467f, 468
Conditioning flash effect, and oscillatory potentials, 574
Cone(s)
biochemistry of, 29–30
blue-sensitive, 48, 599, 599f
pathways/synapses of, 56–57, 57f
cell cytology of, 28–30
cilia of, 65–66, 66f–67f
color selectivity of, 469–470, 469f
in color vision, 47–49, 599–600, 599f
currents of, and a-wave, 151–152, 151f–152f
in daylight vision, 47–49, 49f
definition of, 47
disc membranes of, 29
in duplicity theory, 404–405
embryological development of, 25–27
functional organization of, 47–48
green-sensitive, 48, 599, 599f
pathways/synapses of, 56, 57f
image properties of, 49–50
inner segments of, 24f, 25, 29, 65, 66f
long-wavelength-sensitive (L), 405, 599, 599f
microfilaments of, 65, 67f
microtubules of, 65, 67f
middle-wavelength-sensitive (M), 405, 599, 599f
morphology of, 28–30
neurotransmitter release from, 79
orientation selectivity of, 469–470, 469f
outer segments of, 24f, 25, 28–29, 28f, 47, 48f, 65, 66f
phototransduction in, 72
physiology of, 65–75
protein migration/translocation in, 72–75, 74f
red-sensitive, 48, 599, 599f
pathways/synapses of, 56–57, 57f
responses of. See Cone response
short-wavelength-sensitive (S), 405, 599, 599f
responses in normal and disease states, 603–604
spatial density of, 405
subclasses of, 29
suppressive interaction with rods, 417–420. See also Suppressive rod-cone interaction
synaptic transmission in, 79–91
types of, 48
Cone dystrophy (degeneration), 795–801
autosomal-dominant, 800–801
autosomal-recessive, 801
clinical features of, 795, 797–799, 797t
congenital, 795–796, 798t
“cookie cutter” macula appearance in, 795, 797f
crystalline deposits in, 795, 796f
diagnosis of, 795
electro-oculogram in, 624
electroretinogram in, 88–90, 89f, 507, 510f, 795–797, 799f, 813
fluorescein angiography in, 796f
foveal structure in, 801, 801f
fundus albipunctatus associated with, 835, 837f
fundus findings in, 795, 796f–797f
hereditary, 795–796, 798t
known forms of, 799–800
later onset forms of, 795–796, 798t
management of, 801
optic nerve atrophy in, 795, 798f
oscillatory potentials in, 570, 571f, 576f, 577
partial, 800, 800f
signs and symptoms of, 795, 797t
synaptic transmission in, 88–91
vascular abnormalities in, 795, 796f
X-linked, 795, 796f, 800
Cone matrix sheaths, 26–27, 28f, 30–31, 31f
Cone notches, 29
Cone response, 72, 73f–74f, 405. See also specific disorders/findings
a-wave, 154–155, 154f, 156f, 487–499, 488f
age and, 493, 494f
high-intensity stimulation of, 487–489
model of, 492, 493f
in retinal disease, 497, 497f
S and Rmax parameters of, 497, 497f
simpler equation for, 492–493
background illumination and, 593–595, 594f
b-wave, 161–162, 603
clinical assessment of, 497–500, 498f
dark-adapted, 405–409, 406f, 595
in early receptor potential, 549–551
in electroretinogram
ISCEV standard for, 293–294
isolation of, 7–8
in flicker ERG, 581, 582f–583f
hyperpolarizing, 49, 52f, 79
light-adapted, 593
in mouse, 899–900, 901f, 906, 906f
in multifocal ERG, 197
myopia and, 631, 634
receptive field of, 49, 52f
S-cone, in normal and disease states, 603–604
Cone-rod degeneration. See also Retinitis pigmentosa
electroretinogram in, 813
versus Stargardt disease, 727–732
Confidence interval, 432
Confidence limits, 432
Congenital nystagmus, in albinism, 377–378, 378f–380f
Congenital stationary night blindness (CSNB), 829–837
color vision in, 834
complete, 510, 511f, 809, 811f, 829–834
dark adaptation in, 409, 409f, 829, 831f
electro-oculogram in, 832
electroretinogram in, 507–510, 511f, 809–810, 811f, 829–832, 831f–832f
intensity series in, 832, 833f
long-flash photopic, 832, 834f
genetics of, 809–810, 834, 835f
incomplete, 510, 511f, 809, 811f, 829–834
initial patient complaints in, 829, 830t
versus melanoma-associated retinopathy, 696–697, 697f
oscillatory potentials in, 570, 571f, 577, 577f
pathogenesis of, 834
refractive error in, 829, 830f
Schubert-Bornschein type of, 829
scotopic threshold response in, 832, 833f
visual acuity in, 829, 830f
Constant stimuli, method of, 400, 401f
Contact lens electrodes, 6–7, 7f, 245–247, 246f, 252, 558–559, 559f
Contrast
color, for color vision testing, 602–603
definition of, 413
drug/toxic effects on, 660–661
spatial, 414–415, 414f
loss of, 414, 415f
testing of, 415–416
temporal, 413–414, 414f, 415
Contrast dynamics, 192–194
Contrast perimetry, in multiple sclerosis, 875–878
Contrast reversal, in evoked potential evaluation, 210, 220
Contrast sensitivity
in glaucoma, 854–855
in multiple sclerosis, 875–878, 876f–877f
in neurodegenerative disease, 867
testing of, 415–416
clinical results of, 415–416
means of producing stimuli for, 415
methods of, 415
Contrast transfer function
of pattern ERG, 194, 194f
of visual evoked potential, 194, 194f
Control condition, 432–433
Control software, 242
“Cookie cutter” macula, in cone dystrophy, 795, 797f
Correlation techniques, 451–453
Cortex. See Visual cortex
Cortical blindness
causes of, 651
clinical visual signs of, 651
definition of, 651
retrochiasmal dysfunction and, 861–863
visual evoked potentials in, 651–652
works reporting abnormal, 652
works reporting normal, 651–652
works reporting recovery of, 652
Cortical time, 15
Cortical visual areas, 109
CRAO. See Central retinal artery occlusion
Crawford masking, 408
CRB-1 gene, and Leber congenital amaurosis, 750
Criterion free, 402
Crohn's disease, vitamin A deficiency in, 803
Cross-correlation, 451, 454–455
in multifocal ERG, 322–324, 322f–324f
CRTs. See Cathode-ray tubes
Crumbs (molecule), 100f, 105
CRVO. See Central retinal vein occlusion
CRX gene
and Leber congenital amaurosis, 749–750
and retinitis pigmentosa, 781
Crystalline retinopathy, 735. See also Bietti's crystalline dystrophy
CSNB. See Congenital stationary night blindness
Current source density (CSD), 142
of a-wave, 151, 152f
of b-wave, 152f, 156–157, 159f
of d-wave, 164–165
of pattern response, 189f
c-wave, 5, 11, 123, 143–144, 144f, 557, 558f, 560–561
normal, 560–561
“off,” 561, 562f
in pigment epithelium disease, 560f, 561, 561f
second, 149
Cyclic guanosine monophosphate (cGMP)
cascade in rod bipolar cell, 82, 82f–83f
in cone dystrophy with mild nyctalopia, 89f, 90–91
in inherited night blindness, 88
in melanoma-associated retinopathy, 88
in Leber congenital amaurosis, 748–749
in phototransduction, 67, 68f, 70, 71f, 519
in retinitis pigmentosa, 783–784
Cystic fibrosis, electro-oculogram in, 136
Cytostatic toxicity, 656
D
Dalton, John, 599
Dark adaptation, 405–409, 405f
bleaching parameters and, 408
clinical evaluation of, 408–409
of cones, 405–409, 406f, 595
in congenital stationary night blindness, 409, 409f, 829, 831f
defects in, treatment for, 409
drug/toxic effects on, 660–661
early, 408
factors affecting, 407–409
in fundus albipunctatus, 829, 831f, 834
in gyrate atrophy, 707, 710f
mechanisms of, 406–407
in neuronal ceroid lipofuscinoses, 890
in Oguchi's disease, 829, 831f, 836, 837f–838f
of rods, 405–409, 406f, 520–521, 521f, 595–596f
in Sorsby's fundus dystrophy, 409, 769–771
test stimulus wavelength and, 407, 407f
in vitamin A deficiency, 803, 805f
Dark-adapted a-wave, 150, 150f
Dark-adapted b-wave, 160–161, 163f
stimulus-response functions for, 473–476, 474f, 476f
Dark-adapted electro-oculogram, 128
Dark-adapted electroretinogram, 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 595–596, 596f
Dark-adapted flash response, 520–521, 521f
Dark-adapted oscillatory potentials, 573–575, 573f
Dark choroid effect, 423, 426, 427f
Data acquisition systems, 237–244
building your own, 244
commercially available systems for, 243–244, 243t
components of, 237
overview of, 237, 238f, 261–262, 261f
patient positioning for, 237–238
selection of, questions for, 262–263
special-purpose, 260–263
general characteristics of, 261–262, 261f
Data analyses, 431–437
Daylight vision, cone specialization for, 47–49, 49f
dc. See Direct current
Decimation, of m-sequence, 321, 322f
Deconvolution analysis, 221
Delay, 440–442
Delayed basal hyperpolarization, 147–148, 148f
Delayed rectifier ion channels, 37
Dependent variable, 436
Depth selectivity, reverse correlation of, 469–470
Descriptive statistics, 431–432
Desferrioxamine toxicity, electro-oculogram in, 135
Detection threshold, 400
Deuteranopes (deutans)
early receptor potential in, 585, 589–591, 589f–590f
genetic carriers, ERG responses in, 587f, 588–589, 588f–589f
genetic development of, 600
rapid off-response in, 585–589, 586f–588f
suppressive rod-cone interaction in, 419
visual evoked potentials in, 604–607
Deuteranopia, 599. See also Deuteranopes
Deuteranopic color confusion line, 602
Diabetic retinopathy
color vision in, 603, 608, 608f
multifocal ERG in, 333–334
oscillatory potentials in, 569, 569f
S-cone response in, 603
Diagnostic flowcharts, 515–517, 516f
Diamox (acetazolamide) responses, 126, 128–130, 130f, 553, 554t, 555f, 562
Dichromatism. See Deuteranopes; Protanopes
Difference threshold, 400
Differential amplifiers, 238, 239f, 257, 275–276, 617–618, 617f
Digitalis toxicity, 657
Digital light projection (DLP) system, 271
Digoxin toxicity, 657
Direct current amplifiers, 238–239, 256–257, 559
Direct current component, of b-wave, 162–163, 164f
Direct current electroretinogram, 252, 557–563
clinical applications of, 560, 560f
equipment and procedure for, 557–560
Direct current potential, 11, 123–124, 126
Direction selectivity, 465, 466f
Disc membranes, of photoreceptors, 29
Disconnection(s)
visual-limbic, 117
visual-verbal, 116–117
visual-visual, 116
Distal retina, ERG components arising in, 143–149
Distortion, 442–444
linear, 442–444, 444f
nonlinear, 443
DNA, mitochondrial, 665. See also Mitochondrial disorders
Dog(s)
electroretinogram in, 911–916, 923–931
analysis of, 912
anesthetics for, 923–924, 925t, 927
bilateral, 931, 931f
components of, 912, 913f
correlation with clinical findings, 929–931
guidelines/protocols for, 927–929, 928f, 930f
long-flash, 912, 913f
maturation of, 912, 913f
methods of recording, 911–912, 927–929, 928f, 930f
in retinal dystrophies, examples of, 912–913
Leber congenital amaurosis in, 745, 747–748, 915–916
phenotype diversity in, 912
photoreceptor diseases in, 923, 924t, 929–931
progressive retinal atrophies in, 913–915, 914f–915f, 923, 924t
progressive rod-cone degeneration (prcd) in, 32, 914–915, 915f
retina of, versus human retina, 911
Domains, in Fourier analysis, 447–448
Dominant drusen, 717–724
clinical range of, 717, 719f
codon 172 RDS-related, 717, 720–721, 720f, 722f
definition of, 717
distribution of, 717, 718f
EFEMP1-related, 717, 721–722, 722f–723f
electro-oculogram findings with, 718
electroretinographic findings with, 718
functional changes with, 717–718
genetics of, 717–724
multifocal ERG findings with, 718
TIMP3-related, 717, 719–720
Dominant optic atrophy (DOA), pattern ERG in, 346–347, 348f
Dopamine, 88
in amacrine cells, 60, 61f, 63f, 88
drug/toxic effects on, 657–658, 657f
in Parkinson's disease, 868–873
and spatial processing, 869–870, 869f–871f
Dorsal pathway, 109–110, 110f
neuroanatomy and neurophysiology of, 113
syndromes of, 113–115
Double-blind study, 433
Double magnetic induction (DMI), of ocular motor assessment, 377
Double-opponent cells, 469–470
Double-sided (differential) amplifiers, 238, 239f, 257, 275–276, 617–618, 617f
Doyne's honeycomb dystrophy, 717, 719f, 721
Drug toxicities, 655–661. See also specific drugs
cell-specific functional alterations in, 655–660
central nervous system effects of, 659–660
color vision in, 609–610, 660–661
electroretinogram in, 819, 820f
ganglion cell changes in, 658–659
inner retina alterations in, 657–658
muscular effects of, 661
patient history in, 660
photoreceptor alterations in, 657
retinal pigment epithelium effects of, 655–657
special testing considerations in, 660–661
from symptoms to diagnosis of, 660–661
Drusen
basilar laminar
fluorescein angiography in, 536, 538f
hyperabnormal ERG responses in, 536, 538f
definition of, 717
dominant, 717–724
clinical range of, 717, 719f
codon 172 RDS-related, 720–721, 720f, 722f
definition of, 717
distribution of, 717, 718f
EFEMP1-related, 721–722, 722f–723f
electro-oculogram findings with, 718
electroretinographic findings with, 718
functional changes with, 717–718
genetics of, 717–724
multifocal ERG findings with, 718
TIMP3-related, 719–720
as hallmark of disease, 717
hard, 717
hereditary versus nonhereditary, 723
nature of, 717
soft, 717
DTL electrode, 248f, 250
DTL-Plus electrode, 250
Duchenne muscular dystrophy, electroretinogram in, 816
Dummy patient, 282, 282f, 618–619, 618f
Duplex retina, 48
Duplicity theory, 404–405
d-wave
current source density analysis of, 164–165
depth profile of, 164, 164f
of electroretinogram, 151
origins of, 164–165
of squirrel, 165, 165f
Dystonin, 103
Dystroglycan, 103–105
Dystrophins, 99–100, 103–105, 816
E
Early dark adaptation, 408
Early receptor potential (ERP), 549–551, 624–626
amplitude of, 549–550
chromatic recordings of, 585, 589–591, 589f–590f
clinical application of, 549–551
discovery of, 549, 550f
in ocular siderosis, 550, 550f
origins of, 549
polarity of, 549, 550f
R1 component of, 549, 550f
R2 component of, 549, 550f
rod versus cone contribution to, 549
spectral sensitivity of, 549
Early Treatment for Diabetic Retinopathy Study (ETDRS), 541
Eccentric fixation, in multifocal ERG, 315f, 317
EEG. See Electroencephalogram
EFEMP1 gene, and drusen, 717, 721–722, 722f–723f
Electrical noise, in multifocal ERG, 314f, 316
Electrode(s), 238, 245–254
for electro-oculogram, 252
for electroretinogram, 245–254
carbon fiber, 248f, 249–250
cleaning of, 291
contact lens, 6–7, 7f, 245–247, 246f, 252, 558–559, 559f
for dc recording, 252
development of, 5–8
in dogs, 911–912, 928–929, 928f
DTL fiber, 248f, 250
gold foil, 247–249, 248f
ground, 291
international use of, 251–252
ISCEV guidelines on, 291
in larger animals, 928–929, 928f
lid-hook, 247–250, 248f
in mice, 900
placement of, 143, 143f
polymethylmethacrylate, 252, 558
polyvinyl gel, 249
recording, 291
reference, 291
skin, 248f, 250–251
skin reference, 291
stability of, 291
for multifocal ERG, 197, 198f, 249, 310, 312, 319
for multifocal VEP, 199–200, 203f
for pattern ERG, 298, 341
10/20 system for, 227, 228f
for visual evoked potentials, 207, 226–229, 252–253, 304
ISCEV guidelines for, 304
number of, 227–229
positioning of, 207, 226–227, 228f, 304, 304f
problems with, 227
Electrode artifacts, 245, 278–279, 282, 618–619
Electrode impedance, and amplifiers, 277, 278f
Electrodiagnostic testing. See also Electroencephalogram; Electro-oculogram; Electroretinogram; Visual evoked potentials
commercially available systems for, 243–244, 243t
data acquisition systems for, 237–244
versus psychophysical approaches, 399
stimulators for, 265–275
Electroencephalogram (EEG)
alternatives to, 214–215
contrast and pattern reversal in, 210
dipole production in, 210
electrode positioning for, 226–227, 228f
electrode problems in, 227
evoked potentials in
cortical sources of, localization of, 211–213, 213f–215f
origins of and transmission to scalp, 210–214
pattern appearance, 211, 212f
visual, 207–210. See also Visual evoked potentials
flash response in, 208–210
history of, 208–210
in malingering and hysteria, 638
as mass phenomenon, 211, 211f
postsynaptic potentials in, 210–211, 212f
rhythms of, 208, 209f
signals and noise in, 255, 256t
spatial summation in, 210
temporal summation in, 210–211
Electronegative electroretinogram, 809–819
in acquired diseases of eye, 816–819
autosomal-dominant inheritance of phenotype for, 814
in clinical practice, 819
definition of, 809
disorders associated with, 809–819, 810t
in neurodegenerative disorders, 815–819
origins of, 809
Electronic noise, 255, 256t
Electronic visual acuity (EVA), 541
Electro-oculogram (EOG), 123–136, 623–624. See also specific disorders/findings
acetazolamide test in, 126, 128–130, 130f, 553, 554t, 555f, 562
alcohol test in, 130–133, 131f–135f
bicarbonate test in, 128–130, 553, 554t, 555f
classic, description of, 128
clinical findings of interest, in last 10 years, 135–136
clinical tests utilizing, 127–128
clinical utility of, 134–135
comparison and combination with other tests, 626–627
correlation with clinical findings, 628–629, 628t
current fields in, 123–124, 124f–125f
dark and light adaptation in, 128
electrodes of, 252
fast oscillation, description of, 128, 129f
history of, 11
hyperosmolarity test in, 126, 128–130, 130f, 553, 554t, 555f, 561–562
ISCEV standard for, 287–289
light rise in, 11, 124, 126, 130f
abnormal, in RPE dysfunction, 505–506
localization of lesions in, 505–506, 623–624, 624t–625t
membrane mechanisms of, 124–126
in mitochondrial disorders, 667
nonphotic responses in, 128–130
pharmacology of, 126–127
physiological characteristics of, 126–127
signals and noise in, 255, 256t
steady-state, 128
technical details of, 127
technical difficulties with, 127–128
variants of, 128
Electroretinogram (ERG), 140f. See also specific components, tests, and disorders
abnormal findings in
abnormal rod-specific b-wave, abnormal maximal ERG response a-wave, 506–507
abnormal rod-specific b-wave, (electro-) negative maximal ERG response, 507–510
normal maximal ERG response, abnormal cone ERG, 507
adaptation effects on, 593–596
Electroretinogram (ERG) (continued)
amplification for, 292
averaging in, 294
a-wave of, 4–5, 149–156, 557, 558f. See also a-wave
background illumination for, 292, 593–595, 594f, 595t
b-wave of, 156–163, 557, 558f. See also b-wave
chicken, 916–919
anesthetics for, 926t
delayed basal hyperpolarization in, 148, 148f
in disease, 917–918, 917f–919f
long-flash, 917, 917f
methods of recording, 916
normal, 916–917, 917f
chromatic recordings of, 585–591
chromatic (color) recordings of, 603–604
clinical, 6–8
comparison and combination with other tests, 626–627
components of, 4–5
distal, interaction of, 149
distal origins of, 143–149
modeling of, 143
correlation with clinical findings, 628–629, 628t
correlation with single-cell recordings, 142
c-wave of, 143–144, 144f, 557, 558f, 560–561. See also c-wave
dark-adapted (scotopic), 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 595–596, 596f
data display and averaging in, 292
diagnostic flowcharts for, 515–517, 516f
direct current (dc), 557–563
clinical applications of, 560, 560f
equipment and procedure for, 557–560
dog, 911–916, 923–931
analysis of, 912
anesthetics for, 923–924, 925t, 927
bilateral, 931, 931f
components of, 912, 913f
correlation with clinical findings, 929–931
guidelines/protocols for, 927–929, 928f, 930f
long-flash, 912, 913f
maturation of, 912, 913f
methods of recording, 927–929, 928f, 930f
in retinal dystrophies, examples of, 912–913
d-wave of, 164–165. See also d-wave
electrodes of, 245–254
carbon fiber, 248f, 249–250
cleaning of, 291
contact lens, 6–7, 7f, 245–247, 246f, 252, 558–559, 559f
for dc recording, 252
development of, 5–8
DTL fiber, 248f, 250
gold foil, 247–249, 248f
ground, 291
international use of, 251–252
ISCEV guidelines on, 291
lid-hook, 247–250, 248f
placement of, 143, 143f
polymethylmethacrylate, 252, 558
polyvinyl gel, 249
recording, 291
reference, 291
skin, 248f, 250–251
skin reference, 291
stability of, 291
electronegative, 809–819
in acquired diseases of eye, 816–819
autosomal-dominant inheritance of phenotype for, 814
in clinical practice, 819
definition of, 809
disorders associated with, 809–819, 810t
in neurodegenerative disorders, 815–819
origins of, 809
on electronic recording equipment, 292
e-wave of, 166
excitatory, 5, 6f
extracellular flow in, pattern of, 139
fast oscillation of, 557, 558f, 561–562, 562f
fast oscillation trough of, 143, 144f, 146–148, 147f
fixation in, 293
flicker, 581, 582f–583f
photopic fast, 165, 166f, 293–294
general principles of, 139
Granit's model of, 487, 488f
high-intensity, 293, 487–489, 488f–490f
need for, 487
protocols for, 487–489
history of, 3–8
early discoveries, 3
early recording, 3, 4f
first published, 3–4, 4f
hyperabnormal responses in, 533–540
classification of, 533–536, 537f
distribution by category, 536, 537t
fluorescein angiographic correlation of, 536, 538f, 538t, 539f
illustrative case reports of, 536–539
in macular degeneration, 536, 538, 538f–539f, 540
in optic nerve dysfunction, 536–538, 539f, 540
pathologic process suggested by, 539–540
in Stargardt's disease, 536, 538f
in infants, 745–746
inhibitory, 5, 5f
intraretinal depth recordings in, 142
ISCEV standard for, 287–288, 290–295
on basic technology, 291–293
on clinical protocol, 292–293, 784–791
on light adjustment and calibration, 292
on measurements and recordings, 294–295, 294f
i-wave of, 165
kernel analysis in, 483–484
larger animal, 923–931
anesthetics for, 923–924, 925t–926t, 927
bilateral, 931, 931f
correlation with clinical findings, 929–931
guidelines/protocols for, 927–929, 928f, 930f
specific procedures for, 924–927, 927f
latency of, 442
light-adapted (photopic), 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 593
light diffusion in, 291
light-evoked potassium changes in, 157–159, 160f–161f, 167, 168f
light peak of, 143, 144f, 557, 558f, 561–562, 562f
light sources in, 291–292, 559
local, 142
localization of lesions in, 505–511, 515–517, 624t–625t, 626–629
luminance versus pattern, 185–186, 187f
macaque monkey, 150, 150f
in mitochondrial disorders, 666–669, 666t, 668t–669t
modeling of cellular responses in, 143
mouse, 899–907, 900f
a-wave analyses in, 902–906, 902f
basic recording technique for, 900
b-wave analyses in, 900, 901f, 903, 904f, 906f
cone-mediated responses in, 899–900, 901f, 906, 906f
factors affecting, 900–901
general testing protocol for, 903
interstrain differences in, 903–907
Naka-Rushton analysis of, 903, 904f
oscillatory potentials in, 903, 905f
phototransduction in, 902–903
rod-mediated responses in, 899–900, 901f, 903, 904f–905f
rod- versus cone-mediated comparisons in, 906
specialized recording techniques for, 901
standard strains for, 903
Müller cell contribution in, 141, 141f, 144–146, 145f, 156–157, 160f
multifocal. See Multifocal electroretinogram
M-wave of, 166–168, 167f–168f
myopia and, 631–634, 632f
normal values in, 294, 533–536, 534t, 535f
optic nerve stimulation in, 176
orientation of cells in, 139
origins of, 139–177, 487, 899
approaches for determining, 141–143
oscillatory potentials of, 173–176, 565–578, 566f. See also Oscillatory potentials
paired-flash analysis in, 791, 791f
concept and methodology of, 519–520, 520f
of dark adaptation, 520–521, 521f
of light adaptation, 521–525, 523f–524f, 526f
of recovery following bright illumination, 525–530, 527f–530f
of rod phototransduction and adaptation, 519–530
patient isolation for, 292
patient preparation for, 292–293, 558
pattern. See Pattern electroretinogram
pharmacological dissection in, 142
photopic negative response in, 168–170, 169f
pigment epithelial component of, 146
in post-phototransduction dysfunction, 507–510
in pressure-induced retinopathy, 515–517, 517f
processes of (PI, PII, PII), 4–5, 5f, 142
proximal negative response in, 166, 167f–168f
P-wave of, 176
recalibration of, 292
relative magnitude of signals in, factors determining, 139–140
reporting results of, 294–295
in retinitis pigmentosa, 506–507, 506f, 508f
scotopic threshold response in, 170–173
signals and noise in, 255, 256t
single-flash cone, 293
site-specific lesions/pathology in, 142
slow oscillation of, 557, 558f, 561–562, 562f
slow PIII of, 143–146, 144f–145f
spatial buffering of [K+]o in, 140–141, 141f, 146
spreading depression in, 177
standard combined, 293
stimulus-response functions of, 473
stimulus strength-standard flash in, 292
stimulus wavelength in, 292
targeted mutations in, 142
waveform changes in disease, 626–628, 627t
EMMPIRN, 105
Emotion, and visual evoked potentials, 226
Enhanced S-cone syndrome (ESCS), 507, 508f–509f, 603–604
Environmental rotation, 115, 115f
EOG. See Electro-oculogram
Epinephrine, electro-oculogram effects of, 126
Epitaxial semiconductor junction diodes, 272
Equipment. See also specific types
amplifiers, 238, 255–260, 275–278
analog-to-digital converter, 240–241, 258–260
cathode-ray tubes, 267–269
commercially available systems, 243–244, 243t
control software, 242
data acquisition systems, 237–244
electrodes, 238, 245–254
filters, 239–240, 257–260, 271
light-emitting diodes, 271–275
liquid crystal displays, 269–271
plasma displays, 271
stimulators, 265–275
Equivalent background, 407
ERG. See Electroretinogram
ERG-Jet contact lens electrodes, 246f, 247
ERP. See Early receptor potential
Errors, Type I and Type II, 433–434
Essential nonlinearity, 479
Ethambutol toxicity, 659
Ethanol toxicity, 659
Ethical issues, in therapeutic trials, 544
Ethyl alcohol, electro-oculogram effects of, 126–127, 130–133, 131f–135f
EVA (electronic visual acuity), 541
Event-related potentials
in multiple sclerosis, 881–882
in Parkinson's disease, 878–881, 880f
Evoked potential(s)
alternatives to, 214–215
cortical sources of, localization of, 211–213, 213f–215f
definition of, 207
evaluation of
contrast and pattern reversal in, 210
flash response in, 208–210
origins of and transmission to scalp, 210–214
pattern appearance, 211, 212f
steady-state, 15
visual, 15–18, 207–232. See also Visual evoked potentials
visual subcortical, 16–17
e-wave, origins of, 166
Excitatory ERG (E-ERG), 5, 6f
Excitatory postsynaptic potentials, in EEG, 210–211, 212f
Expectancy, and visual evoked potentials, 224–226
Experimental design, 431–437
basic elements of, 432–433
Eye movement artifacts, 619
Eye position control, for VEP recording, 229
F
Familial optic atrophy, electroretinogram in, 814
Farnsworth-Munsell 100-hue test, 602, 607
Fast flicker ERG, photopic, 165, 166f, 293–294
Fast Fourier transform, in oscillatory potential analysis, 566, 567f, 575–576
Fast oscillation electro-oculogram, 128, 129f
Fast oscillation ERG, 557, 558f, 561–562, 562f
Fast oscillation trough (FOT), 143, 144f, 146–148, 147f
Fast retinal potential, 565. See also Oscillatory potentials
Fatty acid disorders, 889–895
Field potentials, 139
Figure-ground segregation, and visual evoked potentials, 219–220, 219f
Filter(s), 239–240, 257–260
absorption, 271
analog, 258, 258t
band-pass, 257–258, 257f
band-reject, 257–258, 257f
Filter(s) (continued)
digital, 240, 258–260, 258t
high-pass, 239, 257–258, 257f, 277, 277f
interference, 271
low-pass, 239, 240f, 257–258, 257f
for multifocal ERG, 311, 327–329, 330f
notch or line frequency, 240, 257–258, 257f
phase-free, 449, 450f
roll-off of, 239
types of, 239–240
Filtering, of visual evoked potentials, 229, 258, 259f, 260
First harmonic, 444
First-order kernels, 463, 464f, 468, 479–480
First-order receptive fields, 463
Fixation
eccentric, 315f, 317
in electroretinogram, 293
in multifocal ERG, 311, 313, 315f, 317
Flashed-on pattern, 17
Flash lamps, 265–266, 267f
Flash response, in EEG, 208–210
Flash stimulus
for high-intensity ERG, 487–489, 488f–490f
and oscillatory potentials, 573–575, 573f
and visual evoked potentials, 221, 222f, 232, 304, 306, 307f
Flat contact synapses, 96, 97f
Flicker ERG, 581, 582f–583f
chromatic recordings of, 585
fast photopic, 165, 166f, 293–294
Flicker stimulus, 413
for oscillatory potentials, 572–573, 573f
for suppressive rod-cone interaction, 417–418, 418f–419f
Floor effect, 543
Flowcharts, diagnostic, 515–517, 516f
Fluorescein angiography, 423–427
basic principles of, 423
of Bietti's crystalline retinal dystrophy, 423, 424f, 735, 738f–739f
of birdshot chorioretinitis, 685, 687f
of central serous chorioretinopathy, 683, 684f
of choroideremia, 423, 424f, 777, 778f
of cone dystrophy, 796f
correlation of hyperabnormal ERG responses in, 536, 538f, 538t, 539f
dark choroid effect in, 423, 426, 427f
of gyrate atrophy, 707, 711f
of hereditary retinal diseases, 423–427
of juvenile retinoschisis, 424, 425f, 823, 825f
of macular degeneration, 536, 538, 538f–539f, 540
of macular schisis versus edema, 423–425, 425f
of pattern dystrophies, 425, 757, 758f–759f
of quinine retinopathy, 841
of retinitis pigmentosa, 425, 426f, 427, 428f
of Stargardt disease, 426, 427f, 536, 538f
of temporal optic atrophy, 427, 427f
of Usher's syndrome, 424, 425f
Fluphenazine toxicity, 658
Forced-choice procedure, 401–402, 401f
Forsius-Eriksson ocular albinism, 810
Foster-Kennedy syndrome, 700–701, 701f
FOT (fast oscillation trough), 143, 144f, 146–148, 147f
Fourier, Joseph, 444, 444f
Fourier analysis, 241–242, 444–451
checkerboards and, 451, 452f
domains in, 447–448
linearity in, 449
of oscillatory potentials, 566, 567f, 575–576
periodic functions in, 444–447, 446f–447f, 449f
practical considerations on using, 448
receptive fields and, 451
spatial, aspects of, 449–451
spatial frequency in, 450–451
standard periodic signals in, 445, 445f
test signals in, 447
time versus space in, 449, 450f
of visual evoked potential, 230
Fourier integral, 445–446
Fourier series, 446
Fourier theory, 444
Fovea
development of, 25, 26f
ganglion cells of, 56, 57f
Foveal hypoplasia, in albinism, 369, 371f
Foveation period, in albinism, 377–382
Frame pulse artifact, 281, 281f
Frequency dependence, 440, 441f–442f
Frequency response function, of amplifier, 257–258, 257f
Frontal eye fields, 113
disorders of, 113–115
Frontal motor areas, 113
Frumkes effect, 798
“Functional disorders,” visual evoked potentials in, 231–232
Functional magnetic resonance imaging (fMRI), 214–215, 216f–217f
Fundamental harmonic, 444
Fundus albipunctatus, 409, 829, 834–836
associated with cone dystrophy, 835, 837f
dark adaptation in, 829, 831f, 834
electroretinogram in, 813, 835, 837f
fundus appearance of, 834–836
genetics of, 835–836
pathogenesis of, 836
typical, 835, 836f
Fundus photography (FP), 423
Fusion, in infant vision, 357–358, 357f–359f
G
Gamma aminobutyric acid (GABA), 86–88
and b-wave, 160–161, 163f
drug/toxic effects on, 657–658, 657f
and oscillatory potentials, 174–175
Gamma aminobutyric acid receptors, in amacrine cells, 58–60, 59f, 62f
Gamma-band activity, 881
Gamma rhythms, 881
Ganglion cell(s), 49, 50f–51f
alpha (cat), 52
beta (cat), 52
center surround organization of, 80
dopamine and, 869–870, 869f–871f
drug/toxic effects on, 658–659
dysfunction of
multifocal VEP in, 202–203
pattern ERG in, 189–196, 190f–192f, 345–347, 511–512, 514f
embryological origins of, 25
of fovea, 56, 57f
in glaucoma, 851–855
image properties of, 55–56
low-spatial-frequency attenuation of, 192–193
M cells, 52, 55–56, 57f
neurotransmitters of, 86–88
ON/OFF, 51–52, 55–58, 56f–57f, 86, 87f, 95
in oscillatory potential generation, 175
P (midget), 52, 55–56, 57f
postsynaptic responses of, 86, 87f
receptive field of, 55, 56f, 57–58
sustained versus transient, 86
synapses of, 51–52, 54f, 56–58, 57f, 59f, 86, 93, 94f
with amacrine cells, 58–60, 60f–61f, 63f
with bipolar cells, 51–52, 54f, 56, 57f, 93–95, 95f
Ganglion cell layer, 93
Ganzfield stimulator, 265–267, 266f, 559, 560f
Gaussian noise, 451–453
for kernel analysis, 482
Generalized object agnosia, 116
Giant cell arteritis, ischemic optic neuropathy with, 699
Gibbs’ phenomenon, 445
Glass contact lens electrodes, 246
Glaucoma
color vision in, 608, 609f, 854–855
contrast sensitivity in, 854–855
flash electroretinogram in, 851–852
mechanical theory of, 851
multifocal ERG in, 335
multifocal VEP in, 202, 335
pathogenesis of, 851
pattern ERG in, 851–855, 852t, 853f
primary open angle (POAG), 851
psychophysical studies of, 854–855
vascular theory of, 851
visual evoked potentials in, 851–852
visual field defect of, 851, 852f
Glaucomatous optic neuropathy (GOND), 875
Glial cells, drug/toxic effects on, 659
Glutamate, 85–86
a-wave effects of, 152–154
b-wave effects of, 157–158
drug/toxic effects on, 657, 657f
Glutamate receptor(s), 50–52, 53f–54f, 58, 59f, 80–82
ionotropic, 51, 53f–54f, 59f, 80–82
metabotropic, 51, 53f–54f, 59f
in Parkinson's disease, 872
Glutamate transporters, 79
Glycine, 86–88
drug/toxic effects on, 657, 657f
and oscillatory potentials, 174, 569–570, 570f
Glycine receptors, in amacrine cells, 58, 59f
Goethe's color theory, 597–598
Gold contact lens electrode, 245–247, 246f
Gold foil electrode, 247–249, 248f
Goldmann-Favre syndrome, 823
electroretinogram in, 507
fluorescein angiogram of, 424
Goldmann kinetic visual field testing, 541–542
Goldmann-Weekers Dark Adaptometer, 408
GOND (glaucomatous optic neuropathy), 875
G-protein-coupled receptors, in phototransduction, 67, 68f
Granit's model, of electroretinogram, 487, 488f
Granit's PII. See b-wave
Granit's PIII. See a-wave
Grating stimulus, 413
for visual evoked potentials, 215–216, 219
Green-sensitive cones, 48, 599, 599f
pathways/synapses of, 56–57, 57f
Ground loop, 616–617, 616f
Guanylate cyclase, in phototransduction, 71
Guanylate cyclase-activating proteins, 71–72
GUCY2D gene, and Leber congenital amaurosis, 748–749, 748f
Guinea pig, anesthetics for, 926t
Gyrate atrophy, 705–715
biochemistry of, 708–712, 712f
versus choroideremia, 712–713, 779
clinical description of, 707
dark adaptation in, 707, 710f
differential diagnosis of, 712–713
electro-oculogram in, 705, 708f
electroretinogram in, 705, 706f–707f, 710f
fluorescein angiography in, 707, 711f
histopathology of, 708, 711f
historical perspective on, 705
molecular genetics of, 712
muscle biopsy in, 707, 711f
natural history of, 707, 709f–711f
night blindness in, 707, 710f
OAT-deficient, 705, 708–713, 712f
physiology of, 708
pyridoxine-nonresponsive, 705, 706f–707f, 708–711, 709f–710f
pyridoxine-responsive, 705, 706f–707f, 708–711, 708f, 711f
relevant testing in, 712–713
treatment of, 711–712
visual field defect in, 707, 710f
H
Hagberg-Santavuori disease, 889
Haloperidol toxicity, 658
Haltia-Santavuori disease, 889
Harmonic(s)
first or fundamental, 444
in Fourier analysis, 444–445, 445f
higher, 444
Harmonic frequency, 440, 442f
Helmholtz's rule, 444
Hemianopia, in chiasmal dysfunction, 857
Hemispatial (hemifield) neglect, 114
Hemispheric asymmetry, in albinism, 382–395, 385f, 387f–394f
Henkes Lovac contact lens electrodes, 245–247, 246f
Henle's layer, 25, 26f
Higher cortical function, disorders of, 109
Higher harmonics, 444
High-pass resolution perimetry, in multiple sclerosis, 877
HK-loop electrode, 248f, 250
Horizontal cell(s), 49, 50f–51f
center surround organization by, 54–55, 55f, 80
embryological origins of, 25
feedback to, 54–55
image properties of, 52–55
light response of, 79–80
neurotransmitters of, 86–88
receptive field of, 52–54, 55f, 79
in suppressive rod-cone interaction, 417
synapses of, 52–55, 79–82
type A, 79
type B, 79
Horse(s)
electroretinogram in, 923–931
anesthetics for, 923–924, 925t–926t, 927
specific procedures for, 924–927
photoreceptor diseases in, 923, 924t
Humphrey Field Analyzer, 408, 542
Hum reduction, 617–618
“Hum tracer,” 615
Hutchinson-Tay choroiditis, drusen in, 717
Hyperabnormal ERG responses, 533–540
classification of, 533–536, 537f
Hyperabnormal ERG responses (continued)
distribution by category, 536, 537t
fluorescein angiographic correlation of, 536, 538f, 538t, 539f
illustrative case reports of, 536–539
in macular degeneration, 536, 538, 538f–539f
in optic nerve dysfunction, 536–538, 539f, 540
pathologic process suggested by, 539–540
in Stargardt's disease, 536, 538f
Hyperbolic function, 473
Hyperosmolarity responses, 126, 128–130, 130f, 553, 554t, 555f, 561–562
Hypertension. See also Glaucoma
pattern ERG in, 851–855, 854f
psychophysical studies of, 854–855
Hypoemotionality, visual, 117
Hypothesis testing, 432–433
Hysteria, electrodiagnostic testing in, 637–640, 638f–639f
I
Ideal observer, 402
Incandescent lamps, 266–267
Independent group design, 433
Independent variables, 436
Indomethacin toxicity, 657
Infant(s), 353–359
blind, management of, 751–752
electroretinogram in, 745–746
fusion and stereopsis in, 357–358, 357f–359f
preterm, vision in, 355–356, 356f
visual acuity testing in, 232, 353–359
visual evoked potentials in, 17, 232, 306, 353–359
multiple sweep paradigms for, 358
special considerations for, 358–359
sweep, reliability and validity of, 356–357, 357f
waveform development in, 361, 362f
visual maturation in, 353–355, 355f
Infantile Refsum's disease, electroretinogram in, 815–816, 817f
Inhibitory ERG (I-ERG), 5, 5f
Inhibitory postsynaptic potentials, in EEG, 210–211, 212f
Inner limiting membrane, 99, 100f
Inner nuclear layer, 93
Inner plexiform layer (IPL)
adhesion in, 98
drug/toxic effects on, 657–658
ON/OFF processing in, 95
organization of, 57–58
oscillatory potential generation in, 174–175
synaptic connections in, 57–62, 59f, 93–96, 94f–95f
Inner segment, of photoreceptors, 24f, 25, 29, 65, 66f
Input impedance, of amplifier, 257
Instrumentation amplifiers, 275–276
Integrin, in synaptic transmission, 98–99, 100f, 103
Interference filters, 271
Interhemispheric asymmetry, in albinism, 375–377, 376f, 382–395, 385f, 387f–394f
Internal limiting membrane, electrode positioning in, 5–6
International Society for Clinical Electrophysiology of Vision (ISCEV)
guidelines for multifocal ERG, 287–288, 309–317
standards of, 287–288
for electro-oculogram, 287–289
for electroretinogram, 287–288, 290–295, 784–791
for oscillatory potentials, 293
for pattern ERG, 287–288, 297–300, 341
for visual evoked potentials, 287–288, 303t
International Society for Veterinary Ophthalmology, 927
Interphotoreceptor matrix, 23, 24f
biochemistry of, 30–31
composition of, 23
embryological origins of, 23–25
function of, 30–31
insoluble components of, 30–31, 31f
pathologies affecting, 31–32
soluble components of, 30–31
structure of, 30–31
Interphotoreceptor space, development of, 23, 25–27
Interplexiform cell(s)
neurotransmitters of, 88
oscillatory potentials from, 568
Interval estimate, 432
Intracellular signaling, calcium in, 41f, 42
Intraflagellar transport, 66
Intraocular pressure, elevated, 851–855. See also Glaucoma
Intraretinal electroretinogram, 142
Inward rectifier ion channels, 37–38
Iodoacetic acid, and oscillatory potentials, 569–570, 570f
ION. See Ischemic optic neuropathy
Ion channel(s), 37–40
calcium-dependent, 38
chloride, 38–39
delayed rectifier, 37
in electro-oculogram, 124–126
inward rectifier, 37–38
M-type, 38
nonspecific, 38
potassium, 37–38
voltage-activated, 39
voltage-dependent, 38, 79
Ion transport
membrane proteins involved in, 37–40
by retinal pigment epithelium, 37–42, 41f
Ion transporters, 39–40
IPL. See Inner plexiform layer
ISCEV. See International Society for Clinical Electrophysiology of Vision
Ischemic central retinal vein occlusion, 817
Ischemic optic neuritis, 699
Ischemic optic neuropathy (ION), 699–701
arteritic (AAION), 699
clinical presentation of, 699
histology of, 699
treatment of, 699
conditions associated with, 699
nonarteritic (NAION), 699–701
clinical presentation of, 699
versus Foster-Kennedy syndrome, 700–701, 701f
pattern ERG in, 700
treatment of, 699
visual evoked potentials in, 699–701, 700f
Ishihara plates, 599
Isolation amplifiers, 255–256, 277–278
i-wave, 165
in dogs, 912, 915f
J
Jansky-Bielschowsky disease, 889
Juvenile macular degeneration. See Stargardt disease
Juvenile (X-linked) retinoschisis, 823–825
a-wave analysis in, 824–825, 826f
b-wave analysis in, 824–825, 825f–826f
differential diagnosis of, 823
electro-oculogram in, 823–824
electroretinogram in, 810–812, 811f, 823–825, 825f–826f
fluorescein angiogram of, 424, 425f, 823, 825f
fundus appearance of, 823, 824f
gene identification in, 823
photoreceptor and inner retinal responses in, 824–825, 826f
scotopic threshold response in, 824
suppressive rod-cone interaction in, 419
K
Kainate receptors, 51, 53f–54f
Kearns-Sayre syndrome (KSS), 665, 667–668
Kernel(s)
definition of, 479
first-order, 463, 464f, 479–480, 480f
pattern-reversal stimulus for, 483
second-order, 479–482, 480f
reverse correlation of, 468
zero-order, 479
Kernel analysis, 479–484
binary sequence control sequence for, 482, 482t
deconvolution errors in, avoidance of, 482
for multifocal techniques, 483–484
pseudorandom sequences for, 479, 482–483
stimuli for, 479, 480f, 482–484
Kinesin II, 65–66
Kjer-type dominant optic atrophy, pattern ERG in, 346–347
Kohlrausch knick, 406
Köllner's rule, 609–610
Kufs’ disease, 889
Kynurenic acid, a-wave effects of, 153
L
Lactic acid transport, 40, 41f, 42
Laminins, in synaptic transmission, 99–103, 100f–101f, 104f
Laplacian derivation, of visual evoked potentials, 230–231
Lapsing rate, 402
Larger animals
electroretinogram in, 923–931
anesthetics for, 923–924, 925t–926t, 927
bilateral, 931, 931f
correlation with clinical findings, 929–931
guidelines/protocols for, 927–929, 928f, 930f
specific procedures for, 924–927, 927f
photoreceptor variations in, 923
retina of, versus human retina, 923
Latency, 440–442, 464
Latency jitter, 456
Lateral geniculate nucleus (LGN), 109, 213
in albinism, 370, 372f
in monkey, 937, 937f
in mouse, 937–938
Lateral inhibition, and pattern ERG, 191–193
Late receptor potential, 151, 585
Layer of Chievitz, 25
LCA. See Leber congenital amaurosis
LCDs. See Liquid crystal displays
LCHAD. See Long-chain 2-hydroxyacyl CoA dehydrogenase
Lead intoxication, 658
Leber, Theodor, 845
Leber congenital amaurosis (LCA), 745–752
AIPL-1 gene and, 750, 751f
blind infant with, management of, 751–752
chicken model of, 749
clinical variability of, 745
CRB-1 gene and, 750
CRX gene and, 749–750
definition of, 745
dog model of, 745, 747–748, 915–916
electroretinogram in, 745–746, 748, 748f, 749–750, 751f
genetics of, 745–751, 746t
GUCY2D gene defects and, 748–749, 748f
light adaptation in, 594–595, 595t
molecular diagnosis of, 745
mouse model of, 747, 749, 900
prevalence of, 745
RDH12 gene and, 751
RPE65 gene defects and, 746–748
RPGRIP-1 gene and, 750–751
therapeutic studies of, 748
Leber's hereditary optic neuropathy (LHON), 845–847
clinical course of, 845
electro-oculogram in, 845
electroretinogram in, 845–846
fundus appearance of, 845
genetics of, 845
pathogenesis of, 845
pattern ERG in, 346–347
treatment of, 845
visual evoked potentials in, 845–847, 846f–848f
Leber's optic atrophy, color vision in, 610f
LEDs. See Light-emitting diodes
Lesion localization, 505–517, 623–629, 624t–625t
comparison and combination of tests for, 626–627, 628t
definitive tests for, 628–629, 628t
electro-oculogram for, 505–506, 623–624
electroretinogram for, 505–511, 515–517, 624t–625t, 626–629
pattern ERG for, 505–517, 624t–625t
test result-clinical finding correlation for, 628–629, 628t
visual evoked potentials for, 510–517, 624t–625t
Levodopa, visual effects of, 870–871, 872f, 879, 881
LGN. See Lateral geniculate nucleus
LHON. See Leber's hereditary optic neuropathy
Lid-hook electrodes, 247–250, 248f
Light adaptation
in bipolar cells, 84–85, 85f
of cones, 593
in electro-oculogram, 128
of rods, 521–525, 523f–524f, 526f
Light-adaptation effect, and oscillatory potentials, 572
Light-adapted a-wave, 150, 150f, 593
Light-adapted b-wave, 161–162, 163f, 593
Light-adapted electroretinogram, 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 593
fast flicker, 165, 166f, 293–294
Light-adapted oscillatory potentials, 173, 174f, 570–573, 571f–572f
Light-emitting diodes (LEDs), 265, 267, 271–275, 559–560
applications of, 275
applied current and light output of, relationship between, 273, 273f
arrays of, 274–275, 275f
in color vision testing, 602
defects affecting, 274
feedback loop of, 273, 274f
linear control in, 272–274
for multifocal ERG, 327, 329f
organic, 273
pulse density modulation of, 274
pulse width modulation of, 274
Light-emitting diodes (LEDs) (continued)
spatial output of, 272, 272f
spectral output of, 271–272, 273f
types of, 273
typical, construction of, 272, 272f
voltage drive circuit of, 273, 273f
white, 272–273, 273f
Light peak (EOG or ERG), 143, 144f, 148–149, 557, 558f, 561–562, 562f
Light peak/dark trough ratio (L/D), 553
Light rise, in electro-oculography, 11, 124, 126, 130f
abnormal, in RPE dysfunction, 505–506
Limits, method of, 400
Linear approximation, of nonlinear system, 479, 481f
Linear distortion, 442–444, 444f
Linearity, 439–440, 440f–441f
Linear regression analysis, 436
Linear systems
cardinal property of, 439–440
definition of, 439
pattern response of, 480, 481f
stimulus-response functions of, 473–476
Line frequency filter, 240
Line noise, 280
Liquid crystal displays (LCDs), 269–271, 269f
for multifocal ERG, 327, 328f
output spectra of, 270, 270f
problems with, 270
refresh rate of, 270
Local electroretinogram, 142
Localization of lesions, 505–517, 623–629, 624t–625t
comparison and combination of tests for, 626–627, 628t
definitive tests for, 628–629, 628t
electro-oculogram for, 505–506, 623–624
electroretinogram for, 505–511, 515–517, 624t–625t, 626–629
pattern ERG for, 505–517, 624t–625t
test result-clinical finding correlation for, 628–629, 628t
visual evoked potentials for, 510–517, 624t–625t
Lock-in amplifier, 241, 260–261
Long-chain 2-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency, 889, 893–895
electroretinogram in, 893–895, 895f
fundus appearance of, 893, 894f
Low-spatial-frequency attenuation (LSFA), of ganglion cells, and pattern ERG, 192–193
L-type calcium channels, 38, 42
Luminance, for VEP stimulation, 220
Luminance ERG, versus pattern ERG, 185–186, 187f
Luminance noise, 603
M
MacAdam ellipse, 602
Macaque monkey. See Monkey(s)
Macular degeneration (dystrophy)
age-related
alcohol electro-oculogram in, 135, 135f
color vision in, 608–610, 609f
drusen with, 717–718
multifocal ERG in, 334
Sorsby's fundus dystrophy and, 772
diagnostic flowcharts for, 515–517, 516f
electronegative electroretinogram in, 813
fluorescein angiography in, 536, 538, 538f–539f, 540
hyperabnormal ERG responses in, 536, 538, 538f–539f, 540
juvenile. See Stargardt disease
multifocal ERG in, 334, 505, 510–511
pattern ERG in, 342–345, 343f–346f, 505, 510–511, 512f–513f
visual evoked potentials in, 510–511, 513f
vitelliform. See also Best vitelliform macular dystrophy
differential diagnosis of, 765, 765t
in X-linked retinoschisis, 823, 824f
Macular schisis, fluorescein angiogram of, 423–425, 425f
Maculoscope, 727
Magnetic resonance imaging, functional, 214–215, 216f–217f
Magneto-encephalogram (MEG), 214–215
Magnitude estimation, 403–404
Magnitude production, 403–404
Mains interference, 615–617, 616f, 618
reduction of, 617–618, 617f
theories of, 616–617, 616f
Malattia Leventinese, 717, 718f–719f, 721
Malingering, electrodiagnostic testing in, 637–640, 638f–639f
Masking, in therapeutic trials, 544
Matched-subjects design, 433
Maternally inherited diabetes and deafness (MIDD), 668–669, 668t
M channels, 38
Mean, 431
standard error of, 432
Median, 431
Media opacities, visual evoked potentials with, 231
Melanoma-associated retinopathy (MAR), 691–697
clinical presentation of, 696
versus congenital stationary night blindness, 696–697, 697f
electro-oculogram in, 135
electroretinogram in, 510, 696, 697f, 818
synaptic transmission in, 88
MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes), 665, 667–669, 668t
MERRF (myoclonic epilepsy with ragged red fibers), 665–669
Mesopic (term), 404
Metarhodopsin, 68–69, 69f
Metathetic sensations, 399–400
Methanol toxicity, 659
mfERG. See Multifocal electroretinogram
mfVEP. See Multifocal visual evoked potential
Mice
electroretinogram in, 899–907, 900f
a-wave analyses in, 902–906, 902f
basic recording technique for, 900
b-wave analyses in, 900, 901f, 903, 904f, 906f
cone-mediated responses in, 899–900, 901f, 906, 906f
factors affecting, 900–901
general testing protocol for, 903
Naka-Rushton analysis of, 903, 904f
oscillatory potentials in, 903, 905f
phototransduction in, 902–903
rod-mediated responses in, 899–900, 901f, 903, 904f–905f, 906
rod- versus cone-mediated comparisons in, 906
specialized recording techniques for, 901
standard strains for, 903
lateral geniculate nucleus of, 937–938
Leber congenital amaurosis in, 747, 749, 900
Purkinje cell degenerative (pcd), 32
retinal-degenerative (rd), 32
retinal-degenerative slow (rds), 32
retina of, 936–937
retinitis pigmentosa in, 783
striate cortex of, 939
visual evoked potentials in, 935–939, 941–944
flash, 941–942, 942f
pattern, 942–943, 942f
sweep, 942f, 943
visual pathway anatomy of, 936–939
Microconductive fiber electrode, 248f
Microelectrodes. See Electrode(s)
Microfilaments, of photoreceptors, 65, 67f
Microtubules, of photoreceptors, 65, 67f
MIDD (maternally inherited diabetes and deafness), 668–669, 668t
Middle retina diseases, 675–681
Minimization, in therapeutic trials, 544
Minimum-phase rule, 440–442
Mitochondria, 65, 66f, 665
Mitochondrial disorders, 665–669
clinical manifestations of, 665
criteria for, 665
electro-oculogram in, 667
electrophysiology of, 665–669
in patients genetically defined, 667–669, 668t
in patients without genetic diagnosis, 666–667, 666t
full-field (flash) ERG in, 666–669, 666t, 668t
pattern ERG in, 667
rod response in, 666–667
visual evoked potentials in, 667
Mitochondrial myopathies, 665
Mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS), 665, 667–669, 668t
Mixed design, 433
Mizuo phenomenon, 823, 836–837
Mode, 431
Modified binary search (MOBS), 403
Modulation, definition of, 413
Monkey(s)
cortical visual areas of, 109
electroretinogram of, 150, 150f
lateral geniculate nucleus of, 937, 937f
retina of, 936–937, 936f
striate cortex of, 109, 938–939, 938f
visual evoked potentials in, 935–941, 943–944
flash, 939–940, 939f
pattern, 940, 940f
sweep, 940, 941f
Monochromatism (achromatopsia), 112–113, 795–796, 798t, 799–800
Motion perception, neurophysiology of, 113
Motion visual evoked potentials, 217–218, 224, 226f, 605
Motivation, and visual evoked potentials, 226
Mouse model. See Mice
Movement errors, in multifocal ERG, 317
M-sequence, 319–327, 464f, 465
binary, 320
for control of CRT stimulator, 321–322
in cross-correlation process, 322–324, 322f–324f
cycle contents of, 324
decimation of, 321, 322f
generator program for, 325–327, 335–338
period or length of, 324
photodiode test of, 325, 326f
poor selection of, danger of, 324–325
properties of, 324–325
shift and add property of, 324–325, 324f–325f
shift register generation of, 320–321, 321f
window property of, 324
Mucolipidosis IV, electroretinogram in, 815, 816f
Mucopolysaccharidosis IV, 32
Müller cell(s)
cytology of, 30
in electroretinogram
in b-wave generation, 156–157, 159f–160f
contribution of, 144–146, 145f
in scotopic threshold response generation, 172–173
in spatial buffering, 141, 141f, 146
embryological origins of, 25
function of, 30
Müller cell sheen retinal dystrophy, electroretinogram in, 813–814
Multifocal electroretinogram (mfERG), 197–199, 483–484, 483f
adaptation in, 313
amplifiers and filters for, 311, 327–329, 330f
animal testing of, 331–333
artifacts in
averaging and smoothing, 317
recognition of, 316–317
rejection of, 311
reporting and resolution of, 314
averaging in, 311, 313
blind spot in, 317
calibration of, 310–311
in central retinal vein occlusion, 677
in central serous chorioretinopathy, 685, 686f
clinical applications of, 333–335
contrast and background in, 311
contributing factors in response, dissection of, 330–331, 331f–332f
cross-correlation process in, 322–324, 322f–324f
CRT stimulation for, 321–322, 327
description of, 309, 310f
display options in, 311
eccentric fixation in, 315f, 317
electrical noise in, 314f, 316
electrodes for, 197, 198f, 249, 310, 312, 319
erroneous central peak in, 316f, 317
fast sequence of, 197, 200f
fixation monitoring in, 313
fixation targets in, 311
flicker sequence in, 311
frame frequency of, 310
versus full-field ERG, 197–198, 199f, 330
hardware for, 319, 320f
ISCEV guidelines for, 287–288, 309–317
on basic technology, 310–312
on clinical protocol, 312–314
kernel analysis in, 483–484
LCD stimulation for, 327, 328f
LED stimulation for, 327, 329f
luminance for, 310
measurements in, 313–314
monocular versus binocular recording in, 313
movement errors in, 317
m-sequence of, 319–327
binary, 320
for control of CRT stimulator, 321–322
cycle contents of, 324
decimation of, 321, 322f
generator program for, 325–327, 335–338
period or length of, 324
photodiode test of, 325, 326f
poor selection of, danger of, 324–325
properties of, 324–325
shift and add property of, 324–325, 324f–325f
shift register generation of, 320–321, 321f
window property of, 324
myopia and, 632–633, 633f
negative component of
initial (N1), 197, 199f, 313
Multifocal electroretinogram (mfERG) (continued)
second (N2), 197, 199f
nomenclature of peaks in, 310
normal values in, 314
orientation/shadowing error in, 315f, 317
origins of, 310, 329–330
outer retina activity in, 198
patient positioning for, 312
patient preparation for, 312
pharmacological studies of, 331–332
positive component of (P1), 197, 199f, 313
postoperative, 334–335
recording of, 197, 198f, 311–312
choices in, 313
duration of, 313
sequence of, 313
refraction in, 313
reporting of, 313–314
in retinal diseases, 198–199, 201f–202f
macular, 334, 505, 510–511
toxic, 335
vascular, 333–334
in retinitis pigmentosa, 199, 202f, 334, 508f
screen properties of, 310–311
signal analysis in, 311
slow sequence of, 197, 200f
software for, 319–320, 325–327
stimulation for, 310–311
stimulus delivery for, 327
stimulus parameters for, 311
stimulus pattern for, 311
stimulus size for, 311
stimulus source for, 310
sum of responses in, 197, 199f
topographic (3-D) response density plots in, 311–313, 314f
trace arrays in, 311, 312f, 313, 314f
waveforms of, 199, 310, 310f
preservation of shape, 327–329
wide-field, 335
Multifocal stimuli, 483
Multifocal techniques, 319–335, 483–484. See also Multifocal electroretinogram; Multifocal visual evoked potential
Multifocal visual evoked potential (mfVEP), 197, 199–203, 221
ganglion cell or optic nerve damage in, 202–203
in glaucoma, 202, 335
intersubject variability in, 200
intrasubject variability in, 200–202
kernel analysis in, 483–484
mean/averaged responses of, 200, 203f
in multiple sclerosis, 202–203, 204f
recording of, 199–200, 203f
Multiple evanescent white dot syndrome (MEWDS), 334, 685–687, 687f
Multiple sclerosis, 867–868, 873–878, 881–882
contrast perimetry in, 875–878
contrast sensitivity in, 875–878, 876f–877f
cortical pathology in, 874–875
electrophysiology and psychophysics of, 873–878, 881–882
event-related potentials in, 881–882
correlation with imaging results, 881–882
higher visuocognitive abnormalities in, 881–882
high-pass resolution perimetry in, 877
parallel pathways and, 875–878
pattern ERG in, 873, 875
stimulus specificity and diagnosis of, 873–874
visual acuity in, 875
visual evoked potentials in, 202–203, 204f, 513f, 514, 515f, 867, 873–878, 874f
delay/latency in, 874–875, 876f
pattern orientation in, 873–875, 876f
relationship with psychophysical measures, 875
visual field defects in, 875, 877f
Multiple system atrophy, versus Parkinson's disease, 879
Multivariate statistical analysis, 436–437
Muscarinic (M) channels, 38
Muscular dystrophy, electroretinogram in, 816
M-wave
barium effects on, 167, 168f
origins of, 166–168, 167f–168f
versus scotopic threshold response, 171–172
Mydriasis, drug-induced, 661
Mylar electrodes, 247
Myoclonic epilepsy with ragged red fibers (MERRF), 665–669
Myopia
and electroretinogram, 631–634, 632f
and multifocal ERG, 632–633, 633f
stretched retina hypothesis of, 634
Myosin VIIa, 66
N
Na+/Ca2+ exchanger, 39
Na+/HCO3- cotransporter, 39
Na+/H+ exchanger, 39
NAION. See Nonarteritic ischemic optic neuropathy
Naka-Rushton analysis, of mouse model, 903, 904f
Na+/K+-ATPase transporter, 39
Na+/K+/2 Cl<συπ>−<?συπ> cotransporter, 40
NARP (neurogenic muscle weakness, ataxia, and retinitis pigmentosa), 667–669, 669t, 670f–671f
N35 component, of PERG, 297, 298f, 341–342
N95 component, of PERG, 190–192, 192f, 297, 298f, 341–342, 505
in optic nerve dysfunction, 512–513, 514f
Nectins, 98
Negative electroretinogram, 809–819
in acquired diseases of eye, 816–819
autosomal-dominant inheritance of phenotype for, 814
in clinical practice, 819
definition of, 809
disorders associated with, 809–819, 810t
in neurodegenerative disorders, 815–819
origins of, 809
Neglect, hemispatial (hemifield), 114
Neonate(s). See Infant(s)
Neovascular inflammatory vitreoretinopathy, autosomal-dominant, electroretinogram in, 814–815
Neovascularization of iris (NVI), 675–681. See also Central retinal vein occlusion
Neural retina
composition of, 24f, 27, 28f
development of, 23–25
scleral surface of, 24f, 27, 28f
Neurodegenerative disorders. See also specific types
contrast sensitivity in, 867
electroretinogram in, 815–819
Neurogenic muscle weakness, ataxia, and retinitis pigmentosa (NARP), 667–669, 669t, 670f–671f
Neuromuscular junction, 98
Neuronal ceroid lipofuscinoses, 889–895
adult-onset (ANCL), 889
atypical forms of, 889–890
classical forms of, 889
clinical presentation of, 889
electroretinogram in, 815, 815f, 890–893, 891f–894f
Finnish variant (vLINCL) of, 889–890
incidence of, 889
infantile-onset (INCL), 889
juvenile-onset (JNCL), 889
late infantile-onset (LINCL), 889
night blindness in, 890
submicrovolt recordings in, 891–892
visual acuity in, 890
Neuronal response, reverse correlation of, 461–471
Neurotransmitters, 86–88, 655, 656t
drug toxicities and, 655, 657–658, 657f
release from bipolar cells, 85
release from photoreceptors, 79
relevance to clinical states, 88–91
Newton's color theory, 597–598
Nidogen, 99, 100f
Night blindness
in cone dystrophy, 795, 797–798
congenital stationary (CSNB), 829–837
color vision in, 834
complete, 510, 511f, 809, 811f, 829–834
dark adaptation in, 409, 409f, 829, 831f
electro-oculogram in, 832
electroretinogram in, 507–510, 511f, 809–810, 811f, 829–832, 831f–832f
intensity series in, 832, 833f
long-flash photopic, 832, 834f
genetics of, 809–810, 834, 835f
incomplete, 510, 511f, 809, 811f, 829–834
initial patient complaints in, 829, 830t
versus melanoma-associated retinopathy, 696–697, 697f
oscillatory potentials in, 570, 571f, 577, 577f
pathogenesis of, 834
refractive error in, 829, 830f
Schubert-Bornschein type of, 829
scotopic threshold response in, 832, 833f
visual acuity in, 829, 830f
in gyrate atrophy, 707, 710f
inherited, synaptic transmission in, 88
in neuronal ceroid lipofuscinoses, 890
suppressive rod-cone interaction in, 418–419
urban, 795
vitamin A deficiency and, 803–804
Nitric oxide, 88
in amacrine cells, 58, 60, 62f–63f
Nocturnal vision, rod specialization for, 47
Noise
electrical, in multifocal ERG, 314f, 316
electronic, 255, 256t
Gaussian, 452–453
for kernel analysis, 482
line, 280
luminance, 603
physiological, 255, 256t
white, for kernel analysis, 479, 480f, 482
Nonarteritic ischemic optic neuropathy (NAION), 699–701
clinical presentation of, 699
versus Foster-Kennedy syndrome, 700–701, 701f
pattern ERG in, 700
treatment of, 699
visual evoked potentials in, 699–701, 700f
Nonessential nonlinearity, 479
Nonlinear distortion, 443
Nonlinearity, 479, 480f
dynamic, 443
essential, 479
kernel analysis of, 479–484
linear approximation of, 479, 481f
nonessential, 479
static, 443
Nonorganic visual loss, 637
Non-parametric psychophysical techniques, 402
Non-parametric statistical testing, 435–436
Nonphotic standing potential responses, 553, 554t, 555f
Nonspecific cation channels, 38
Nonsteroidal anti-inflammatory drugs, electro-oculogram effects of, 127
Normal distribution, 431
Normal values
in electroretinogram, 294, 533–536, 534t, 535f
in multifocal electroretinogram, 314
in visual evoked potentials, 307
Normative data, 431–432
Notch filter, 240, 257–258, 257f
Nougaret, Jean, 781
NRL gene, and retinitis pigmentosa, 781
Nutritional deficiencies, 659
Nyctalopia, mild, cone dystrophy with
ERG findings in, 88–90, 89f
synaptic transmission in, 88–91
Nyctalopin, 834
Nystagmus
congenital, in albinism, 377–378, 378f–380f
latent, and VEPs in amblyopia, 645, 645f
NYX gene, and congenital stationary night blindness, 835
O
OAT. See Ornithine aminotransferase
Object agnosia, 116
Object anomia, 116–117
Occipitofugal pathways, 109–110, 110f
dorsal (where), 109–110, 110f
neuroanatomy and neurophysiology of, 113
syndromes of, 113–115
ventral (what), 109–110, 110f
lesions of, 116–117
neuroanatomy and neurophysiology of, 116
Ocular motor apraxia, 113–114
Ocular siderosis, early receptor potential in, 550, 550f
Ocular standing potential, 553
“Off c-wave,” 561, 562f
OFF pathways. See ON/OFF pathways
Oguchi's disease, 409, 603, 829, 836–837
dark adaptation in, 829, 831f, 836, 837f–838f
electro-oculogram in, 836
electroretinogram in, 834f, 836–837, 837f
fundus appearance of, 836, 838f
genetics of, 836–837
pathogenesis of, 837
OMPs (oscillatory membrane potentials), 176, 176f
ON/OFF pathways, 51–58, 53f
amacrine cell, 58, 60f
bipolar cell, 51–57, 53f–54f, 57f, 80–82, 80f–81f
ganglion cell, 51–52, 55–58, 56f–57f, 86, 87f, 95
in inner plexiform layer, 95
parallel processing in, 80, 80f–81f
ON pathways, 51–52, 53f
OPL. See Outer plexiform layer
OPs. See Oscillatory potentials
Opsin, 47, 599–600, 599f
Optic aphasia, 117
Optic ataxia, 113–114
Optic atrophy
color vision in, 609–610, 610f
dominant, pattern ERG in, 346–347, 348f
multifocal VEP in, 202
and pattern ERG, 189–192, 190f–192f
temporal
in cone dystrophy, 795, 798f, 799
fluorescein angiogram of, 427, 427f
Optic chiasm dysfunction. See Chiasmal dysfunction (lesions)
Optic nerve misrouting, in albinism, 369, 371f–372f, 514–515, 516f
Optic nerve stimulation, potentials evoked by, 176
Optic neuropathy
diagnostic flowcharts for, 515–517, 516f
drug-induced, 659
hyperabnormal ERG responses in, 536–538, 539f, 540
ischemic, 699–701
arteritic (AAION), 699
clinical presentation of, 699
histology of, 699
treatment of, 699
conditions associated with, 699
nonarteritic (NAION), 699–701
clinical presentation of, 699
versus Foster-Kennedy syndrome, 700–701, 701f
pattern ERG in, 700
treatment of, 699
visual evoked potentials in, 699–701, 700f
multifocal VEP in, 202–203
in multiple sclerosis, 867–868, 873–878, 881–882. See also Multiple sclerosis
nutritional, 659
in Parkinson's disease, 867–873, 878–882. See also Parkinson's disease
pattern ERG in, 345–347, 347f–348f, 512–513, 514f
traumatic, electrophysiology of, 515–517, 517f
visual evoked potentials in, 231, 505, 512–513, 514f
Optic primordium, 23
Optic stalk, 23
Optic sulcus, 23
Oregon eye disease, 816
Organic light-emitting diodes (O-LEDs), 273
Orientation error, in multifocal ERG, 315f, 317
Orientation selectivity
in multiple sclerosis, 873–875
in Parkinson's disease, 873
reverse correlation of, 469–470, 469f
Ornithine aminotransferase (OAT), 705
deficiency, in gyrate atrophy, 705, 708–713, 712f
Orthogonality, 453
Oscillatory membrane potentials (OMPs), 176, 176f
Oscillatory potentials (OPs), 565–578, 566f
analysis of response, methods of, 575–576
artificial variable sum amplitude of, 575
bandwidth filtered versus software-filtered, 566, 567f
bandwidth restriction of, 565, 566f, 575–576
b-wave relationship with, 569–570
chronological sequence of, 568
in cone dystrophy, 570, 571f, 576f, 577
in congenital stationary night blindness, 570, 571f, 577, 577f
depth profile of, 173–174, 174f
in diabetic retinopathy, 569, 569f
diagnostic use of, 576–578
discovery of, 8
early versus late, 568
extraction from raw ERG signal, 565–567, 566f–567f
fast Fourier transform analysis of, 566, 567f, 575–576
flash stimulus and, 573–574, 573f
flickering light stimulus and, 572–573, 573f
frequency of, 173, 565
generation of
cells involved in, 174–175
in intracellular responses from neurons, 175–176, 175f
mechanisms of, 175–176
neuronal interaction/feedback circuits in, 175
ISCEV standard for, 293–294
in mouse, 903, 905f
origins of, 173–176, 567–568
pharmacological manipulation of, 173–174, 569–570, 570f
photopic, 173, 174f, 570–573, 571f–572f
in rat, 927
retinal distribution of, 568
in retinitis pigmentosa, 576f, 577, 578f
scotopic, 573–575, 573f
shorter-latency versus longer-latency, 568
single-sweep versus averaged, 566–567, 567f
Oscilloscopes, 260
Outcome measures, 541
Outer limiting membrane, 24f, 29
Outer nuclear layer, 93
Outer plexiform layer (OPL), 93
development of, 25
drug/toxic effects on, 657–658
synaptic processing and organization in, 96–97
Outer retina disorders, 683–687
Outer segment, of photoreceptors, 24f, 25, 28–29, 28f, 47, 48f, 65, 66f
Output impedance, of amplifier, 257
P
Paired-flash ERG analysis, 791, 791f
concept and methodology of, 519–520, 520f
of dark adaptation, 520–521, 521f
of light adaptation, 521–525, 523f–524f, 526f
of recovery following bright illumination, 525–530, 527f–530f
of rod phototransduction and adaptation, 519–530
Parallel processing, 80, 80f–81f
Parameter(s). See also specific tests
pathology indications in, 432
population, estimation of, 432
Parameter estimation by sequential testing (PEST), 403
Parametric psychophysical techniques, 402
Parametric statistical testing, 435–436
Parastriate cortex, 109
damage to, syndromes caused by, 111
Parkinson's disease, 867–873, 878–882
color vision deficits in, 872–873
contrast sensitivity in, 867, 872
corticocortical interactions in, 881
differential diagnosis of, 879
dopamine deficiency in, 868–873
electrophysiology and psychophysics of, 868–873, 878–881
electroretinogram in, 873
event-related potentials in, 878–881, 880f
higher visuocognitive abnormalities in, 878–881
levodopa therapy for, visual effects of, 870–871, 872f, 879, 881
neuropharmacology of, 867, 879
non-dopaminergic visual effects in, 871–872
parallel pathways in, 870
pattern ERG in, 868–869, 869f, 871, 872f
P300 delays in, 878–879
P100 response in, 878–879, 880f
spatial and temporal frequency in, 868–869, 869f
spatial processing in, 869–870, 869f–871f
visual categorization impairment in, 879–880
visual cortical deficits in, 873
visual evoked potentials in, 868, 871–873
Parseval theorem, 453
Patching, and VEPs in amblyopia, 645, 645f
Patient compliance, and VEP recording, 232
Patient positioning
for data acquisition, 237–238
for multifocal ERG, 312
for pattern ERG, 341–342
Patient preparation
for electroretinogram, 292–293, 299–300, 558
for multifocal ERG, 312
for pattern ERG, 299
for visual evoked potentials, 305
Patient-related artifacts, 279, 619
Patient safety, amplifiers and, 255–256
Pattern dystrophies, 757–761
clinical findings in, 757
description of, 757
electro-oculogram in, 757–760, 760f
electroretinogram in, 757–760, 760f
fluorescein angiography in, 425, 757, 758f–759f
multifocal electroretinogram in, 757
physiological findings in, 757–760
vitelliform variant of, 765, 765t
Pattern electroretinogram (PERG), 185–194, 341–348, 342f. See also specific disorders/findings
amplification for, 299, 341
artifact rejection in, 299
averaging and signal analysis in, 299
background illumination for, 299
check size dependence (tuning) of, 191–193, 193f
clinical applications of, 342–347
color responses in, 604
comparison and combination with other tests, 626–627
components of, 341–342
contrast for, 298
contrast transfer function of, 194, 194f
correlation with clinical findings, 628–629, 628t
data display systems for, 299
diagnostic flowcharts for, 515–517, 516f
electrodes for, 298, 341
field and check size for, 298
frame rate of, 299
in ganglion cell dysfunction, 189–192, 190f–192f, 345–347, 511–512, 514f
generator of, pinpointing of, 186
ISCEV standard for, 287–288, 297–300, 341
on basic technology, 298–299
on clinical protocol, 299–300
on recording equipment, 299
on waveform nomenclature and measurement, 297–298
localization of lesions in, 505–517, 624t–625t
luminance for, 298
luminance versus pattern stimulation in, 185–186, 187f
in macular dysfunction, 342–345, 343f–346f, 505, 510–511, 512f–513f
in malingering and hysteria, 638–640, 638f–639f
in mitochondrial disorders, 667
monkey versus human, 190–192, 192f
monocular and binocular recording in, 299–300
N35 component of, 297, 298f, 341–342
N95 component of, 190–192, 192f, 297, 298f, 341–342, 505
normal, 341–342
in optic nerve dysfunction, 345–347, 347f–348f, 512–513, 514f
origins of, 341–342
patient positioning for, 341–342
patient preparation for, 299
P50 component of, 190–192, 192f, 297, 298f, 341–342, 505
photopic negative response and, 170
physical source localization in, 187–189, 189f
recalibration of, 299
recording of, 341–342
reporting results of, 300
in retinitis pigmentosa, 506–507, 508f
reversal rate of, 299
second harmonic in, 185–186
steady-state, 298–299
stimulus parameters for, 298–299
stimulus patterns for, proper versus improper, 185, 188f
transient
ISCEV guidelines on, 297, 298f, 299
peaks and components of, 190, 192f
Pattern-evoked potentials, aging and, 361–367
Pattern onset/offset VEP, 212f, 222, 223f, 302–303, 305–306, 306f
Pattern reversal
for evoked potential evaluation, 210, 222–224, 223f, 225f, 302, 305, 305f, 605
for kernel analysis, 483
P50 component, of PERG, 190–192, 192f, 297, 298f, 341–342, 505
in ganglion cell dysfunction, 511–512, 514f
in macular dysfunction, 510–511, 512f–513f
PDA (cis-2,3-piperidine dicarboxylic acid)
a-wave effects of, 153–155, 153f, 155f
b-wave effects of, 162, 163f
d-wave effects of, 165
and multifocal ERG, 332
PDE6A/PDE6A genes, and retinitis pigmentosa, 781
Peanut agglutinin (PNA)-binding glycoconjugates, in cone matrix sheaths, 27, 28f, 30, 31f
PERG. See Pattern electroretinogram
Periodic functions, in Fourier analysis, 444–447, 446f–447f, 449f
Peristriate cortex, 109
damage to, syndromes caused by, 111
pH, intracellular, regulation of, 42
Phagocytosis, by retinal pigment epithelium, 27
Pharmacological dissection, in ERG, 142
Phase characteristic, 440, 441f
Phase distortions, amplifiers and, 260
Phase-free filtering, 449, 450f
Phase shift, 440–442, 442f
Phenothiazine toxicity, 656–657
Phenytoin toxicity, 658
PhNR (photopic negative response), 168–170, 169f
Phosducin, 72
Phosphodiesterase
activation of, 70
inactivation of, 70
in phototransduction, 67, 68f, 70, 519
in retinitis pigmentosa, 783–784
Phosphodiesterase inhibitor toxicity, 657
Phosphors
for cathode-ray tube, 268, 268f
for light-emitting diodes, 273
Photic driving, 208, 221
Photochromatic interval, 406
Photocurrent saturation, 519
Photopic (term), 404
Photopic a-wave, 150, 150f, 593
Photopic b-wave, 161–162, 163f
Photopic ERG, 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 593
fast flicker, 165, 166f, 293–294
Photopic negative response (PhNR), 168–170, 169f
in humans and rodents, 170
relationship to pattern ERG, 170
wavelength of stimuli and, 170
Photopic oscillatory potentials, 173, 174f, 570–573, 571f–572f
Photoreceptor(s). See also Cone(s); Rod(s)
activity of, clinical assessment of, 497–500, 498f–499f
cell cytology of, 28–30, 49, 50f–51f
cilia of, 65–66, 66f–67f
currents of, and a-wave, 151–152, 151f–152f
dark adaptation of, 405–409, 406f
drug/toxic effects on, 657
duplicity theory of, 404–405
embryological development of, 25–27
function of, 28–30
health of
early receptor potential assessment of, 550–551
electroretinogram of, 505
measuring with a-wave leading edge, 487–500
hyperpolarizing response of, 49, 52f, 79
inner segment of, 24f, 25, 29, 65, 66f
interface with retinal pigmented epithelium, 23–32, 24f
metabolic machinery of, 65, 66f
microfilaments of, 65, 67f
microtubules of, 65, 67f
morphology of, 28–30, 28f
neurotransmitter release from, 79
outer segment of, 24f, 25, 28–29, 28f, 47, 48f, 65, 66f
parallel processing in, 80, 80f–81f
pathologies affecting, 31–32
physiology of, 65–75
protein migration/translocation, 72–75, 75f
retinal position of, 47
synapses of, 49–62, 53f–54f, 59f
synaptic transmission in, 79–91
relevance to clinical states, 88–91
types of, 49
Phototransduction, 66–75
activation cascade of, 67, 68f
cGMP-gated channels in, 67, 70, 71f
in cones, 72
in mouse, 902–903
phosphodiesterase in, 67, 68f, 70
relationship to electrical activity, 72, 73f–74f
in retinitis pigmentosa, 783–784
rhodopsin in, 67–69, 68f–69f
rod, paired-flash analysis of, 519–530
in rods, 66–72
transducin in, 67, 68f, 70
Photovoltaic effect, and artifact, 280
Physiological change, clinical assessment of, 434–435
Pigeons, anesthetics for, 926t
Pigmentary retinopathy, 665
Pigs, anesthetics for, 926t
PI process, 4–5, 5f, 142
PII process, 4–5, 5f, 142
PIII process, 4–5, 5f, 142. See also a-wave; b-wave
distal versus proximal, 146
slow, 143–146, 144f–145f
Pituitary tumors, 857. See also Chiasmal dysfunction
Plasma displays, 271
Plato's color theory, 597
PMMA electrode, 252, 558
PNR (proximal negative response), 166, 167f–168f
Point estimate, 432
Polymethylmethacrylate (PMMA) electrode, 252, 558
Polyvinyl (PVA) gel electrodes, 249
Population parameters, estimation of, 432
Positron emission tomography, 214–215
Posterior parietal cortex, 113
disorders of, 113–115
Postreceptoral response. See also Oscillatory potentials
a-wave, 152–155, 153f–155f
intrusion on leading edge, 491, 492f
b-wave, 475–476, 475f
derivation of, 475, 475f
Postsynaptic potentials, in EEG, 210–211, 212f
Potassium channel(s)
calcium-dependent, 38, 42
delayed rectifier, 37, 42
in electro-oculogram, 124–126
in electroretinogram
light-evoked changes in, 157–159, 160f–161f, 167, 168f, 172–173, 172f
in scotopic threshold response generation, 172–173, 172f
spatial buffering of, 140–141, 141f, 146
inward rectifier, 37–38
M-type, 38
Power
of electrical signal, 453–454
statistical, 434
Power density, 454
Power density spectrum, 454
PPRPE (preserved para-arteriolar retinal pigment epithelial retinitis pigmentosa), 425, 426f
Preamplifiers, 255–256
Preparation, and visual evoked potentials, 226
Preserved para-arteriolar retinal pigment epithelial retinitis pigmentosa (PPRPE), 425, 426f
P100 response, 17, 229, 305
aging and, 361–365, 363f–364f
in Parkinson's disease, 878–879, 880f
P300 response, in Parkinson's disease, 878–879, 880f
Pressure-induced retinopathy, electrophysiology of, 515–517, 517f
Preterm birth, and visual acuity, 355–356, 356f
Primary open angle glaucoma, 851. See also Glaucoma
Primary visual cortex
damage to, disorders associated with, 110–111
human, 109, 110f
monkey, 109, 938–939, 938f
mouse, 939
Principal component analysis, of VEPs in albinism, 373–374, 374f, 382–385
Processes of ERG (PI, PII, PIII), 4–5, 5f, 142
Progressive external ophthalmoplegia, 665
Progressive retinal atrophy, in dogs, 913–915, 914f–915f, 923, 924t
Progressive rod-cone degeneration (prcd), 32
in dogs, 914–915, 915f
Progressive rod-cone dysplasia, in dogs, 914–915, 915f
Proline supplementation, for gyrate atrophy, 711–712
Prosopagnosia, 116
Protanopes (protans)
early receptor potential in, 585, 589–591, 589f–590f
genetic carriers, ERG responses in, 587f, 588–589, 588f–589f
genetic development of, 600
rapid off-response in, 585–589, 586f–588f
suppressive rod-cone interaction in, 419
visual evoked potentials in, 604–607, 605f–607f
Protanopia, 599. See also Protanopes
Protein migration/translocation in photoreceptors, 72–75, 74f
Prothetic sensations, 399–400
Proximal negative response (PNR), 166, 167f–168f
Proximal PIII, 146
Pseudoisochromatic plates, 599
Pseudorandom sequence, for kernel analysis, 479, 482–483
Psychic paralysis of gaze, 113–114
Psychophysic(s)
definition of, 399
fundamental concepts of, 399–400
Psychophysical techniques, 399–409, 542
adaptive, 402–403
classical, 400
clinical applications of, 404
for color vision testing, 607–610
versus electrophysiological approaches, 399
in glaucoma/hypertension, 854–855
in multiple sclerosis, 875
non-parametric, 402
parametric, 402
suprathreshold, 403–404
Ptosis, drug-induced, 661
Pulse density modulation, of LEDs, 274
Pulse width modulation, of LEDs, 274
Pupillary size, aging and, 365
Pure alexia, 116–117, 117f
Purkinje cell degenerative (pcd) mice, 32
PVA gel electrodes, 249
p-value, 433–434
P-wave, 176
Pyridoxine-nonresponsive gyrate atrophy, 705, 706f–707f, 708–711, 709f–710f
Pyridoxine-responsive gyrate atrophy, 705, 706f–710f, 708–712, 708f, 711f
Q
Quality of life, 542–543
Questionnaires, 542–543
QUEST method, 403
Quinine retinopathy, 841–842
clinical course of, 841
electroretinogram in, 819, 820f, 841–842, 842f
fluorescein angiography in, 841
pattern ERG in, 842f
visual evoked potentials in, 841–842
R
Rab escort protein-1 (REP-1), in choroideremia, 779
Randomized controlled trial, 541
Range, statistical, 431
Raster, 268, 322
Rat(s)
anesthetics for, 926t
oscillatory potentials in, 927
RCS, 32
RDH5 gene, and fundus albipunctatus, 835–836
RDH12 gene, and Leber congenital amaurosis, 751
RDH5 gene mutations, 784
RDS/peripherin gene
and drusen, 717, 720–721, 720f, 722f
and retinitis pigmentosa, 783
Rebound, 470
Receiver operating characteristic (ROC) curve, 401, 434–435
Receptive field(s)
in alert animal, 465–466, 466f–467f
of cones, 49, 52f
dopamine and, 869–870, 869f–871f
first-order, 463, 464f
and Fourier analysis, 451
of ganglion cells, 55, 56f, 57–58
of horizontal cells, 52–54, 55f, 79
mapping spatial structure of, 463–465, 464f
reverse correlation of, 461–471
Recovery following bright illumination, 525–530, 527f–530f
Rectification, 443, 443f
Red-green color deficiency, 599
classification of, 588
ERG recordings in, 585–591
Red-sensitive cones, 48, 599, 599f
pathways/synapses of, 56–57, 57f
Refractive error
in congenital stationary night blindness, 829, 830f
and electroretinogram, 631–634, 632f
and multifocal ERG, 632–633, 633f
in Oguchi's disease, 836
Refsum's disease, infantile, electroretinogram in, 815–816, 817f
Regression analyses, 436
Repeated measurements design, 433
Reporting, ISCEV standards for, 294–295, 300, 307–308
Response latency, of visual evoked potentials, 208
Response maps, 465
Retina. See also specific entries
cells of, 49, 50f–51f
development of, 23–25
embryological origins of, 23–25
functional organization of, 47–62, 48f–51f
neural. See Neural retina
Retinal, 47
Retinal artery occlusion
branch, 334
central, 681
c-wave in, 561, 561f
electroretinogram in, 507, 681, 816–817
Retinal pigment epithelium (RPE)
black layer of, 47
cell cytology of, 27–28
development of, 25
disorders/dysfunction of, 683–687. See also specific disorders
electro-oculogram of, 505–506, 623–624
drug/toxic effects on, 655–657
electrode positioning in, 5–6
embryological origins of, 23–25
fast capacitative compensation by, 40
functions of, 27–28, 37–42
interface with photoreceptors, 23–32, 24f
intracellular pH regulation by, 42
intracellular signaling in, 42
light peak/dark trough ratio in, 553
membrane mechanisms of, 37–42, 124–126
morphology of, 27–28
pathologies affecting, 31–32
pattern dystrophies of, 425, 757–761
phagocytosis by, 27
responses related to, 553, 554t, 555f
slow responses of, 557
synthesis and secretion by, 27–28
transepithelial potential of, 146
Retinal pigment epithelium (RPE) (continued)
transport by, 27, 37–42
ion, 37–42, 41f
lactic acid, 40, 41f, 42
voltage difference across, 11, 123–126, 124f–125f. See also Electro-oculogram
Retinal vein occlusion
branch, 681
central, 675–681
electroretinogram in, 675–681, 676f–680f, 816–818, 818f
amplitudes in, 675–676, 676f–677f
intensity-response analysis in, 677–678, 680f
temporal factors in, 676–677, 678f–679f
ischemic, 817, 818f
multifocal ERG in, 677
nonischemic, 817
photoreceptor function in, 679–680, 680f
Retinitis pigmentosa, 781–792
autoimmune retinopathy with, 691–697
versus choroideremia, 777
clinical features of, 781
clinical-test result correlation in, 628–629, 628t
Coats’ reaction in, 427, 428f
conditions associated with, 781
electro-oculogram in, 624
electroretinogram in, 506–507, 506f, 508f, 623, 784–791, 812–813, 812f–813f
amplitude-retinal illuminance functions in, 787–791, 789f–790f
a-wave analysis in, 506–507, 790f, 791
c-wave in, 560f, 561
extensions of protocol for, 787–791
full-field, sensitivity of, 786–787, 787f
paired-flash method in, 791, 791f
protocol for, 784–786, 785f–787f
flicker ERG in, 581, 582f–583f
fluorescein angiogram of, 425, 426f, 427, 428f
fundus appearance of, 781, 782f
genetic analysis of, 781–784, 782t
light adaptation in, 594–595, 595t
mouse model of, 783
multifocal electroretinogram in, 199, 202f, 334, 508f
oscillatory potentials in, 576f, 577, 578f
pattern ERG in, 506–507, 508f
photoreceptor responses in, 496f–498f, 497, 506–507, 506f, 508f, 783–784
phototransduction cascade in, 783–784
preserved para-arteriolar retinal pigment epithelial, 425, 426f
prevalence of, 781
psychophysical approaches in, 399
suppressive rod-cone interaction in, 418–419
visual cycle in, 784
X-linked, 781
alcohol electro-oculogram in, 134–135, 134f
oscillatory potentials in, 576f, 577
Retinol, transport of, 27
Retinoschisis, juvenile (X-linked), 823–825
a-wave analysis in, 824–825, 826f
b-wave analysis in, 824–825, 825f–826f
differential diagnosis of, 823
electro-oculogram in, 823–824
electroretinogram in, 810–812, 811f, 823–825, 825f–826f
fluorescein angiogram of, 424, 425f, 823, 825f
fundus appearance of, 823, 824f
gene identification in, 823
photoreceptor and inner retinal responses in, 824–825, 826f
scotopic threshold response in, 824
suppressive rod-cone interaction in, 419
Retinovascular disorders. See also specific types
electroretinogram in, 816–818
Retrochiasmal dysfunction (lesions), 665, 860–863
bilateral, 861–863
cortical blindness in, 861–863
unilateral, 860–861
visual evoked potentials in, 231, 307, 515, 860–863, 861f–862f
Reverse correlation, 461–471
in alert animal, 465–466, 466f–467f
basics of, 462–463, 462f
of color selectivity, 469–470, 469f
of depth selectivity, 469–470
early technical limitations of, 465
of orientation selectivity, 469–470, 469f
potential pitfalls in, 470–471
of second-order kernels, 467f, 468
of simple cell receptive fields, 463–465, 464f
space-time maps in, 465, 466f
suppression versus no response in, 470
Rhodopsin, 28–29, 47, 48f, 65
activation of, 67–68, 68f
cycle of, 69f
and early receptor potential, 549–551
inactivation of, 68–69, 69f
in retinitis pigmentosa, 783–784
vitamin A deficiency and, 803, 805f
Rhodopsin kinase, in Oguchi's disease, 836–837
Riddoch phenomenon, 111
“Rim” protein, 29
RLBP1 gene, and retinitis pigmentosa, 781
ROC (receiver operating characteristic) curve, 401, 434–435
Rod(s)
activity of, clinical assessment of, 497–500
biochemistry of, 29–30
cell cytology of, 28–30
cilia of, 65–66, 66f–67f
currents of, and a-wave, 151–152, 151f–152f
definition of, 47
disc membranes of, 29
in duplicity theory, 404–405
embryological development of, 25–27
functional organization of, 47–48
health of, measuring with a-wave leading edge, 487–500
image properties of, 49–50
inner segments of, 24f, 25, 29, 65, 66f
microfilaments of, 65, 67f
microtubules of, 65, 67f
morphology of, 28–30
neurotransmitter release from, 79
in nocturnal vision, 47
outer segments of, 24f, 25, 28–29, 28f, 47, 48f, 65, 66f
phototransduction in, 66–72
paired-flash ERG analysis of, 519–530
physiology of, 65–75
protein migration/translocation in, 72–75, 74f
recovery following bright illumination, 525–530, 527f–530f
responses of. See Rod response
spatial density of, 405
suppressive interaction with cones, 417–420. See also Suppressive rod-cone interaction
synaptic transmission in, 79–91
Rod-cone break, 406, 406f, 407, 407f
Rod-cone dysplasia, in dogs, 913–915, 914f–915f
Rod monochromatism, 795–796, 798t
Rod response, 72, 73f–74f, 405. See also specific disorders/findings
a-wave, 154–155, 154f–155f, 157f, 487–500, 488f, 809
age and, 493, 494f
alternative models of, 493–495, 495f
high-intensity stimulation of, 487–489, 488f–490f
model of, 489–491, 490f–492f
in retinal disease, 495–497, 496f
S and Rmax parameters of, 495–497, 496f
b-wave, 473–476, 474f, 632–633, 635f
clinical assessment of, 497–500, 498f–499f
dark-adapted, 405–409, 406f, 520–521, 521f, 595–596, 596f
in early receptor potential, 549–551
in ERG, ISCEV standard for, 293
e-wave, 166
hyperpolarizing, 49, 79
light-adapted, 521–525, 523f–524f, 526f
in mitochondrial disorders, 666–667
in mouse, 899–900, 901f, 903, 904f–905f, 906
paired-flash ERG analysis of, 519–530
RP. See Retinitis pigmentosa
RPE. See Retinal pigment epithelium
RPE65 gene
and Leber congenital amaurosis, 746–748
and retinitis pigmentosa, 784
RPGR gene, and retinitis pigmentosa, 781
RPGRIP-1 gene, and Leber congenital amaurosis, 750–751
S
Sample and hold amplifier, 241, 242f
Sampling, of visual evoked potentials, 229
Schopenhauer's color theory, 597–599
Scleral search coil method, of ocular motor assessment, 377
Scotoma(s)
in Bietti's crystalline dystrophy, 735
in chiasmal dysfunction, 857
in Sorsby's fundus dystrophy, 771
Scotopic (term), 404
Scotopic a-wave, 150, 150f
Scotopic b-wave, 160–161, 163f
stimulus-response functions for, 473–476, 474f, 476f
Scotopic (dark-adapted) ERG, 7–8, 8f, 139–140, 140f, 150, 150f, 292–293, 595–596, 596f
Scotopic oscillatory potentials, 573–575, 573f
Scotopic threshold response (STR), 150–151, 168
in congenital stationary night blindness, 832, 833f
depth distribution of, 171–172, 171f
in dogs, 912
K+-Müller cell mechanism for, 172–173, 172f
versus M-wave, 171–172
negative, 170–171, 171f
neuronal origins of, 173
origins of, 170–173
positive, 170–171, 171f
sensitivity of, 172, 172f
as separate response from proximal retina, 171–173
in X-linked retinoschisis, 824
Second c-wave, 149
Second harmonic, in pattern ERG, 185–186
Second messenger(s)
calcium as, 41f, 42
in electro-oculogram, 126
and light peak, 149, 149f
Second-order kernels, 479–482, 480f
reverse correlation of, 468
Seebeck, August, 599
Sensory scaling, 403–404
Serotonin, 88
in Parkinson's disease, 872
SFD. See Sorsby's fundus dystrophy
Shadowing error, in multifocal ERG, 315f, 317
Shift and add property, of m-sequences, 324–325, 324f–325f
Shift register sequence generation, 320–321, 321f
Short-wavelength automated perimetry (SWAP), 607
Shutter systems, 266–267
Sidekicks, 98, 99f
Siderosis, ocular, early receptor potential in, 550, 550f
Signal analysis, basic concepts of, 439–444
Signal averaging, 242
Signal detection theory, 400–402
Signal extraction, 241–242
Signal-to-noise ratio
improving, with averaging, 455
in stimulus-response functions, 475
Significance level, 433
Sildenafil toxicity, 657
Silent substitution, in color vision testing, 602
Simple cell receptive fields
in alert animal, 465–466, 466f–467f
mapping spatial structure of, 463–465, 464f
Simultanagnosia, 113–114
Single-cell recordings, ERG correlation with, 142
Single-flash cone ERG, 293
Single photon emission computed tomography (SPECT), 214
Skewed distribution, 431
Skin electrodes, 248f, 250–251
Slow oscillation ERG, 557, 558f, 561–562, 562f
Slow PIII, of ERG, 143–146, 144f–145f
Smoothing artifacts, 317
Snellen visual acuity, 541
Sodium/calcium exchanger, 39
Sodium/HCO3- cotransporter, 39
Sodium/hydrogen exchanger, 39
Sodium/potassium-ATPase transporter, 39
Sodium/potassium/chloride cotransporter, 40
Software
control, 242
for multifocal ERG, 319, 325–327
Sorsby's fundus dystrophy (SFD), 769–772
and age-related macular degeneration, 772
clinical features of, 769
color vision in, 769
dark adaptation defect in, 409, 769–771
drusen in, 717, 719f, 720, 723–724
electro-oculogram in, 769
electroretinogram in, 769, 770f–771f
fundus findings in, 769, 770f
genetics of, 720, 771
histopathology of, 769
pattern ERG in, 769, 772f
psychophysics of, 769–771
treatment of, 771–772
visual field defects in, 771
Source-sink analysis, 142
Spatial buffer currents, in ERG, 140–141, 141f, 146
Spatial contrast, 414–415, 414f
loss of, 414, 415f
testing of, 415–416
Spatial Fourier analysis, 449–451
Spatial frequency, in Fourier analysis, 450–451
Spatial summation, in EEG, 210
Spielmeyer-Sjögren syndrome, 889
Spielmeyer-Vogt disease, 889
S potentials, 49
Spreading depression (SD), 177
Squirrel
anesthetics for, 926t
d-wave of, 165, 165f
SRCI. See Suppressive rod-cone interaction
SST-1 Scotopic Sensitivity Tester, 408
Staircase method, 402–403, 403f
Standard deviation, 431–432
Standard error of mean, 432
Standing potential
definition of, 557
nonphotic stimuli/responses of, 553, 554t, 555f
Starburst amacrine cells, 61f, 86
Stargardt disease, 727–732
clinical presentation of, 727
color vision in, 609, 610f
versus cone-rod dystrophy, 727–732
electro-oculogram in, 624
electroretinogram in, 727, 728f
fluorescein angiography in, 426, 427f, 536, 538f
genetics of, 727–732, 732f
hyperabnormal ERG responses in, 536, 538f
incidence of, 727
multifocal ERG in, 334, 727, 730f–731f
pattern ERG in, 343–344, 511, 512f
Stargardt-fundus flavimaculatus, 727. See also Stargardt disease
Static, 443
Statistics, 431–437
Steady-state electro-oculogram, 128
Steady-state evoked potential, 15
Steady-state stimulus, for visual evoked potentials, 221
Stereopsis, in infants, 357–358, 357f–359f
Stiles two-color technique, 602
Stimulators, 265–275
Stimulus. See also specific types and tests
spatially structured, 265
unstructured, 265
Stimulus-response functions, 465
parameters for, 476, 476f
for scotopic b-wave, 473, 476f
signal-to-noise ratio in, 475
stimulus intensity for, 473–474, 474f
STR. See Scotopic threshold response
Stretched retina hypothesis, 634
Striate cortex
damage to, disorders associated with, 110–111
human, 109, 110f
monkey, 109, 938–939, 938f
mouse, 939
Stroboscope artifacts, 619
Subretinal space, 23
changes in, fast capacitative compensation for, 40
ERG electrodes in, 143, 143f
Substance P, 88
Sulpiride toxicity, 658
Superposition principle, 439
Suppressive rod-cone interaction (SRCI), 417–420
background of, 417–418
clinical perspective on, 418
in color vision disorders, 419
electroretinogram of, 417
flicker stimulus for, 417–418, 418f–419f
newer developments in, 420
in night blindness, 418–419
parameters for, 417
pharmacological studies of, 417
in X-linked conditions, 419–420
Supranormal ERG responses. See Hyperabnormal ERG responses
Synapse(s), 49–62, 53f–54f, 59f, 93–105
development of, 97–105, 99f
flat contact, 96, 97f
in inner plexiform layer, 57–62, 59f, 93–96, 94f–95f
invaginating, 96, 97f
molecular organization of, 96–97, 98f
in outer plexiform layer, 96–97, 97f
retinal layers of, basic organization of, 93–96
stabilization of, 97–105
Synaptic gain, 82
Synaptic ribbon, 96, 97f–98f
Synaptic transmission, 79–91
adhesion in, 97–105, 99f–100f
postsynaptic elements of, 96–97, 97f–98f
presynaptic elements of, 96, 97f–98f
relevance to clinical states, 88–91
Synchronous amplification (detection), 456–457, 457f–458f
System analysis, 454–455
T
Tamoxifen toxicity, 659
Tapetum cellulosum, 923
Tapetum lucidum, 923
Teller Acuity Cards, 353, 357–358
Temporal contrast, 413–414, 414f, 415
Temporal optic atrophy
in cone dystrophy, 799
fluorescein angiogram of, 427, 427f
Temporal summation, in EEG, 210–211
10/20 system, of electrode placement, 227, 228f
TEP (transepithelial potential), 146, 553
Test(s), 623–629, 624t–625t. See also specific tests and disorders
comparison and combination of, 626–627, 628t
correlation with clinical findings, 628–629, 628t
definitive, 628–629, 628t
Test signals, in Fourier analysis, 447
Tetrodotoxin (TTX)
in ERG studies, 142
in multifocal ERG, 332–333
in pattern ERG, 190–192, 192f
Thalamus, as pacemaker for cortical activation, 213–214
Théorie Analytique de la Chaleur (Fourier), 444
Therapeutic trials
case selection for, 544
ceiling and floor effects in, 543
electrophysiology in, 542
endpoints of, continuous versus categorical variables as, 543
ethical issues in, 544
evaluating patients for, technical issues in, 541–544
interventions in, 544
masking in, 544
outcome measures in, 541
psychophysical tests in, 542
questionnaires in, 542–543
small sample sizes in, strategies to overcome, 543–544
statistical considerations in, 543
study design of, 541
visual acuity measures in, 541
visual field measures in, 541–542
Threshold
classes of, 400
definition of, 400
detection, 400
difference, 400
psychophysical measurement of, 400
Tissue inhibitor of metalloproteinase 3 gene (TIMP3)
and dominant drusen, 717, 719–720
and Sorsby's fundus dystrophy, 720, 771
Toxicology, 655–661. See also specific drugs
Tracking procedure, 400
Transducin, 67, 68f
activation of, 70
inactivation of, 70
migration/translocation of, 72–75, 74f
Transepithelial potential (TEP), 146, 553
Transfer function, 440
Transport
by ciliary cytoskeleton, 65–66
intraflagellar, 66
of lactic acid, 40, 41f, 42
membrane proteins involved in, 37–39
by retinal pigment epithelium, 37–42, 41f
Transporters, 39–40
Treatment condition, 432–433
Trichromacity. See also Color vision
history of, 599–600
Tritan color confusion line, 602
Tritanopia, 599
visual evoked potentials in, 604–607, 606f–607f
t-test, 436
TULP1 gene, and retinitis pigmentosa, 781
U
Undersampling, of visual evoked potentials, 229
United Parkinson's Disease Rating Scale, 872
Unit step function, 447
Univariate statistical analysis, 436–437
Urban night blindness, 795, 797–798
Usherin, 100f, 105
Usher syndrome, 781
versus choroideremia, 779
fluorescein angiogram of, 424–425, 425f
light adaptation in, 594–595, 595t
V
Variance, statistical, 431
VECPs. See Visual evoked potentials
Venous occlusions, 675–681. See also Branch retinal vein occlusion (BRVO); Central retinal vein occlusion (CRVO)
Ventral pathway, 109–110, 110f
lesions of, 116–117
neuroanatomy and neurophysiology of, 116
VEPs. See Visual evoked potentials
Vernier acuity, and VEPs in amblyopia, 646
Veterinary guidelines, 927–929, 928f, 930f. See also Animal testing; specific animals
Viagra toxicity, 657
Vigabatrin retinopathy, 658
electro-oculogram in, 135
multifocal ERG in, 335
Visual acuity
in congenital stationary night blindness, 829, 830f
electronic (EVA), 541
in multiple sclerosis, 875
in neuronal ceroid lipofuscinoses, 890
normal maturation of, 354–355, 355f
preterm birth and, 355–356, 356f
testing of
in drug toxicities, 660
in infants, 232, 353–359
measures for, 541
potential pitfalls in, 353–354, 354f
visual evoked potentials for, 231–232, 232f, 353–359
Visual allesthesia, 114–115, 115f
Visual amnesia, 117
Visual cortex, 109
dorsal (where) pathway of, 109–110, 110f
neuroanatomy and neurophysiology of, 113
syndromes of, 113–115
human, 109, 110f
monkey, 109, 938–939, 938f
mouse, 939
primary (V1), 109, 110f
damage to, disorders associated with, 110–111
V2 (parastriate), 109, 110f
damage to, syndromes caused by, 111
V3 (peristriate), 109, 110f
damage to, syndromes caused by, 111
V4 (human color center), 109, 110f, 112f, 600
damage to, syndromes caused by, 112–113
V5 (area MT), 109, 110f
damage to, syndromes caused by, 113
ventral (what) pathway of, 109–110, 110f
lesions of, 116–117
neuroanatomy and neurophysiology of, 116
Visual evoked cortical potential (VECP). See Visual evoked potentials
Visual evoked potentials (VEPs), 15–18, 207–232, 867, 868f. See also specific disorders/findings
aging and, 361–367
accommodation changes with, 366, 366f–367f
amplitude changes with, 361, 363f
contrast threshold changes with, 362–364, 365f
gender differences in, 362, 364f
general changes with, 361–362
luminance threshold changes with, 364, 365f
peak latency changes with, 361–362, 364f
pupillary size and, 365
temporal frequency changes with, 362, 363f
in albinism, 369, 373–395, 373f
age and, 386–396, 390f–394f
averaging of, 380–382, 383f
checkerboard stimulus for, 378–382
data analysis of, 375, 376f
genetic differences in, 386, 387f–389f
hemispheric asymmetry in, 375–377, 376f, 382–395, 385f, 387f–394f
latency of, 382, 384f
methodology for, 375–377
misrouting test of, 369, 375–377, 514–515, 516f
pattern onset/offset, 378–380, 381f–384f
pattern reversal, 378–380, 381f
principal component analysis of, 373–374, 374f, 382–385
stimulus for, 375
topography of, versus normal controls, 382–395
alternatives to, 214–215
in amblyopia, 644–647
binocular, 646–647
binocular summation and facilitation of, 646
dichoptic stimulation of, 646–647
interocular differences in, 644
latent nystagmus and, 645, 645f
Visual evoked potentials (VEPs) (continued)
patching and, 645, 645f
pattern assessment of, 644–645
threshold measurements of, 646
Vernier acuity and, 646
amplifier characteristics for, 229, 304–305
amplitude of, 230, 305, 440, 441f
analysis techniques for, 229–231
animal, 935–944
appearance/disappearance, 222, 223f
applications of, 231–232
arousal and, 224–226
attention and, 224–226
averaging of, 207–208, 304–305
in chiasmal and retrochiasmal dysfunction, 231, 307, 505, 514–515, 516f, 857–860, 858f–860f
to chromatic stimuli, 224
cognitive set and, 226
color, 218, 604–607, 605f–607f
coma or reduced level of consciousness and, 232
comparison and combination with other tests, 626–627
components (waves) of, 15–16, 16f, 211–213
contrast transfer function of, 194, 194f
correlation with clinical findings, 628–629, 628t
in cortical blindness, 651–652
works reporting abnormal, 652
works reporting normal, 651–652
works reporting recovery of, 652
cortical sources of, localization of, 211–213, 213f–215f
definition of, 207
diagnostic flowcharts for, 515–517, 516f
different stimuli for and different types elicited, 207
electrodes for, 226–229, 252–253, 304
ISCEV guidelines for, 304
number of, 227–229
positioning of, 207, 226–227, 228f, 304, 304f
problems with, 227
electroencephalogram of, 207–210
emotion and, 226
expectancy and, 224–226
extrinsic and intrinsic activation of, 207
eye position control for, 229
figure-ground segregation and, 219–220, 219f
filtering of, 229, 258, 259f, 260
form of, 222f–223f, 225f–226f, 230, 230f
Fourier analysis of, 230
frequency contents of, 230
in “functional disorders,” 231–232
in ganglion cell dysfunction, 511–512, 514f
history of, 15–18, 208–210
in infants, 17, 232, 306, 353–359
for fusion and stereopsis testing, 357–358, 357f–359f
multiple sweep paradigms for, 358
of normal maturation, 353–354, 354f
preterm birth and, 355–356, 356f
special considerations for, 358–359
sweep, reliability and validity of, 356–357, 357f
waveform development in, 361, 362f
interpretation of, 308
ISCEV standard for, 287–288, 301–308, 303t
on basic technology, 302–305, 303t
on clinical protocol, 305–308
on measurement and reporting, 307–308
Laplacian derivation of, 230–231
latency of, 224, 229, 305, 442
lesions affecting, 17–18
during life span, 17
localization of lesions with, 510–517, 624t–625t
in macular dysfunction, 510–511, 513f
in malingering and hysteria, 637–640, 638f–639f
mirroring of visual system by, 208
in mitochondrial disorders, 667
monkey, 935–941, 943–944
flash, 939–940, 939f
pattern, 940, 940f
sweep, 940, 941f
motion, 217–218, 224, 226f, 605
motivation and, 226
mouse, 935–939, 941–944
flash, 941–942, 942f
pattern, 942–943, 942f
sweep, 942f, 943
multifocal, 197, 199–203, 221
ganglion cell or optic nerve damage in, 202–203
in glaucoma, 202, 335
intersubject variability in, 200
intrasubject variability in, 200–202
kernel analysis in, 483–484
mean/averaged responses of, 200, 203f
in multiple sclerosis, 202–203, 204f
recording of, 199–200, 203f
normal flash, 221, 222f, 232, 304, 306, 307f
normal values in, 307
in optic nerve dysfunction, 231, 505, 512–513, 514f
patient preparation for, 305
pattern onset/offset, 212f, 222, 223f, 302–303, 305–306, 306f
pattern reversal stimulus for, 222–224, 223f, 225f, 302, 305, 305f, 605
pattern stimulation of, 17
pediatric, 306–307
phase characteristic of, 440, 441f
poor patient compliance and, 232
preparation and, 226
in pressure-induced retinopathy, 515–517, 517f
recording of, 207, 226–229
multi-channel, 307
parameters for, 304
rectification of, 443, 443f
response latency of, 208
sampling and undersampling of, 229
signals and noise in, 255, 256t
standard transient responses in, description of, 305–306
stimulation of
check size for, 220
contrast for, 220
field size for, 220
luminance for, 220
modulation in color space, 218
modulation in depth, 218–219
modulation in space, 215–216
modulation in time, 216–217
modulation in time and space, 217–218
modulation of spatial frequency, 219, 231–232
parameters for, 220–221, 302, 303t
physiological targeting of and responses to, 221–226
steady-state, 221
techniques for, 216–221
temporal frequency for, 220–221
synchronization of stimulus monitor for, 229
for visual acuity testing
in infants, 232, 353–359
media opacities and retinal dysfunctions in, 231
objective assessment in, 231–232, 232f
potential pitfalls in, 353–354, 354f
Visual evoked subcortical potential (VESP), 16–17
Visual field defects
in chiasmal dysfunction, 857
in drug toxicities, 660
in glaucoma, 851, 852f
in gyrate atrophy, 707, 710f
in multiple sclerosis, 875, 877f
in Sorsby's fundus dystrophy, 771
Visual field measures, 541–542
Visual hypoemotionality, 117
Visual latency, 440–442, 464
Visual-limbic disconnection, 117
Visual-verbal disconnection, 116–117
Visual-visual disconnection, 116
Visuospatial processing
disorders of, 113–115
dorsal pathway in, 109–110, 110f, 113
Vitamin A, 47
and dark adaptation, 409, 803, 805f
deficiency of, 803–804
conditions associated with, 803–804
electroretinogram in, 803, 804f–805f
fundus appearance of, 803, 804f
metabolism of, 803
supplementation, 409, 803
Vitelliform (term), 763
Vitelliform macular lesions, differential diagnosis of, 765, 765t
Vitreous humor, 93
Voltage-activated chloride channels, 39
Voltage-controlled oscillator (VCO), 274
Voltage-dependent calcium channels, 38, 79
von Kries, Johannes, 599
W
Wagner disease, 823
Wavelets, ERG. See Oscillatory potentials
Weak signal artifact, 316f, 317
Werblin-Westheimer procedure, 420
“What” (ventral) pathway, 109–110, 110f
lesions of, 116–117
neuroanatomy and neurophysiology of, 116
“Where” (dorsal) pathway, 109–110, 110f
neuroanatomy and neurophysiology of, 113
syndromes of, 113–115
White light-emitting diode, 272–273, 273f
White noise, for kernel analysis, 479, 480f, 482
Wide-field multifocal ERG (WF-mfERG), 335
Window property, of m-sequence, 324
X
Xenon flash lamps, 265–266, 267f
X-linked albinism, 373, 386, 387f–389f
X-linked cone dystrophy, 795, 796f, 800
X-linked retinitis pigmentosa, 781
alcohol electro-oculogram in, 134–135, 134f
oscillatory potentials in, 576f, 577
suppressive rod-cone interaction in, 419
X-linked retinoschisis, 823–825
a-wave analysis in, 824–825, 826f
b-wave analysis in, 824–825, 825f–826f
differential diagnosis of, 823
electro-oculogram in, 823–824
electroretinogram in, 810–812, 811f, 823–825, 825f–826f
fluorescein angiogram of, 424, 425f, 823, 825f
fundus appearance of, 823, 824f
gene identification in, 823
photoreceptor and inner retinal responses in, 824–825, 826f
scotopic threshold response in, 824
suppressive rod-cone interaction in, 419
x-wave, of electroretinogram, in dogs, 7–8, 912
Z
Zero-order kernels, 479