ereditary Fundus ystrophies @SPECIAL INVESTIGATIONS 488 © CHOROIDAL DYSTROPHIES 505 Electroretinography 488, Choroideremia 505 Electro-oculography 489 Gyrate atrophy 506 Dark adaptometry 489 Central areolar choroidal dystrophy 508 Colour vision 490 Difuse choroidal atrophy 508 Helicoidal parapapillary chorioretinal @RETINAL DYSTROPHIES 491 degeneration 508 Retinitis pigmentosa 491 Pigmented paravenous retinochoroidal atrophy 508 Progressive cone dystrophy 494 Stargardt disease 496 @ VITREORETINOPATHIES 508 Fundus flavimaculatus 496 Congenital retinoschisis 508 Jwenile Best disease 497 Stickler syndrome 510 ‘Adult vitellform foveomacular dystrophy 499 Favre-Goldmann syndrome S114 Multifocal Best disease 499. Familial exudative vitreoretinopathy 511 Familial drusen 499 Erosive vitreoretinopathy 512 Sorsby pseudo-inflammatory macular dystrophy 500 Dominant neovascular inflammatory North Carolina macular dystrophy 501 vitreoretinopathy 513 Butterfy macular dystrophy S01 Dominant vitreoretinochoroidopathy 513 Dominant cystoid macular oedema 502 Bieti crystalline dystrophy 502 @ ALBINISM 513 Alport syndrome 502 Oculocutaneous albinism 513 Benign familial fleck retina 503 Ocular albinism — 514 Leber congenital amaurosis 503 Congenital stationary night blindness 504 © CHERRY-RED SPOTAT MACULA 515 Congenital monochromatism 505@ Special investigations Electroretinography General principles The clectroretinogram (ERG) is the record of an action potential produced by the retina when itis stimulated by light, of adequate intensity. The recording is made between an active electrode embedded in a contact lens placed on the patient's cornea (or a gold foil electrode placed on the e) and a reference electrode on the patient's forehead. The potential between the two electrodes is then amplified and displayed (Fig. 15.1). The ERG is elicited both in the light- adapted (photopic) and dark-adapted (scotopic) states. The normal ERG is biphasic (Fig. 15.2). 1. The a-wave is an initial negative deflection which arises from the photoreceptors. 2. The b-wave is a positive deflection, which although generated by Miller cells, represents electrical activity in the bipolar cell region. The amplitude of the b-waveis measured from the trough of the a-wave to the peak of the b-wave, ‘and increases with both dark adaptation and increased light stimulus. The b-wave consists of bl and b2 subcomponents. ‘The former probably represents both rod and cone activity and the latter mainly cone activity, Itis possible to single out rod and cone responses with special techniques. Normal ERG This consists of five recordings (Fig. 15.3). The first three are elicited afler 30 minutes of dark adaptation (scotopic), and Reference electrode Receptors Bipolar cols + O—<>—_O—< Mille calls Fig. 15.2 2 ‘Components and origins of the electroretinogram_ Smsiaiv Fig. 153 Normal electroretinographic recordings the last two afler 10 minutes of adaptation to bright diffuse illumination (photopic).1. Scotopic ERG 4, Rod responses are elicited with a very dim flash of white light or a blue light, resulting in a large b-wave and a small or non-recordable a-wave. b. Combined rod and cone responses are elicited with a very bright white flash resulting in a prominent a-wave anda b-wave. ¢, Oscillatory potentials are elicited by using a bright flash and changing the recording parameters. The oscillatory wavelets occur on the ascending limb of the bewave and are generated by cells in the inner retina, Photopic ERG Cone responses are elicited with a single bright flash resulting in an a-wave and a b-wave with small oscillations. b, Cone flicker is used to isolate cones by using a flickering light stimulus at a frequency of 30 Hizto which rods can- not respond. It provides a measure of the amplitude and implicit time of the cone b-wave. Cone responses can be dicted in normal eyes up to 50 Ha, after which point individual responses are no longer recordable (critical flicker fusion). -oculography lectro-oculogram (HOG) measures the standing potential the electrically positive cornea and the electrically ineback of the eye (Fig, 15.4). Itreflects the activity of the fand the photoreceptors. This means that an eye blinded by proximal to the photoreceptors will have a normal EOG, al diffuse or widespread discase of the RPE isneeded to Dark trough Light peak Light pos arrogr” * = 1% alfect the BOG response significantly: The test is performed in both light- and dark-adapted states as follows: 1. The electrodes are attached to the skin near the medial and lateral canth. 2. The patient is asked to look rhythmically from side to side, making excursions of constant amplitude. Each time the eye moves the cornea makes the nearest electrode positive with respect to the other. 3. The potential difference between the two electrodes is amplified and recorded, ‘As there is much variation in BOG amplitude in normal subjects, the result is calculated by dividing the maximal height of the potential inthe light (light peak) by the minimal height of the potential in the dark (dark trough). This is ‘expressed as a ratio (Arden ratio) or as a percentage, The normal value is over 1.85 or 185%. Dark adaptometry Dark adaptation (DA) is the phenomenon by which the visual system (pupil, retina and occipital cortex) adapts to decreased illumination. Dark adaptometry is particularly useful in patients complaining of night-blindness (nyetalopia). 1. Goldmann-Weekes adaptometry is performed as follows: a, The subject is frst exposed to an intense light that will totally bleach the photoreceptors. b. After so being rendered fully light-adapted, the subject is then suddenly placed in scotopic conditions. . A series of flashes of light of gradually increasing intensity, localized 11° below fixation. is then presented. 4. The threshold at which the subject just perceives the light is plotted. e, The flashes are repeated at regular intervals: sensitivity of the eye to light gradually increas The sensitivity curve is bipartite (Fig. 15. © The initial rapid segment represents cone function and the second, slower segment rod function, the Recovery of cone sensitivity photopic vision| Rod-cone recovery time Rocovery of rod sensitivity Mesopic vision ‘Threshold luminence (log units), Fig. 15.5 Dark adaptation curve