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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



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