The Electroretinogram in Albinos and Carriers of the Ocular Albino Trait

Krill, Alex E.; Lee, Gilbert B.

doi:10.1001/archopht.1963.00960040038008

Cited by 60 publications

(14 citation statements)

References 8 publications

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“…Our findings that some ERGs are larger from those with lighter eyes are in keeping with previous studies [2][3][4][5][6][7]. The ITs were earlier for the blue group compared with the brown group for most of ERG components, both dark-and lightadapted, which may suggest that a basic process, such as the phototransduction cascade, may be slightly accelerated in those with blue eyes.…”

Section: Discussionsupporting

confidence: 92%

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Light- and dark-adapted electroretinograms (ERGs) and ocular pigmentation: comparison of brown- and blue-eyed cohorts

et al. 2010

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This study characterizes differences in human ERGs based on ocular pigmentation. Light- and dark-adapted luminance-response (LR) series for a-, b- and i-waves and light-adapted oscillatory potentials (OPs) were recorded in 14 healthy volunteers (7 blue-eyed Caucasians; 7 brown-eyed Asians, aged 20-22 years). Amplitude interpolations were by logistic growth (Naka-Rushton), Gaussian or the combined 'photopic hill' functions. Implicit times (IT) for dark-adapted a- and b-waves, and for light-adapted a-, b- and i-waves were earlier in the blue-eyed group than in the brown-eyed group across all flash strengths (P < 0.05). For dark-adapted ERGs, saturated a-wave amplitude was larger for blue eyes (397 vs. 318 μV, P < 0.05) as was the a-wave to strong flash (10 cd·s/m(2); 357 vs. 293 μV, P < 0.05) and the b-wave to ISCEV standard 0.01 (354 vs. 238 μV, P < 0.05). Light-adapted b-waves for midrange flash stimuli were much larger for the blue-eyed group (photopic hill, Gaussian peak: 155 vs. 82 μV, P < 0.001) with no difference in saturated amplitudes. Similarly, interpolated i-wave amplitudes were larger (48 vs. 18 μV, P < 0.01). For a light-adapted 2.6 stimulus, a- and b-waves were larger for the blue-eyed group (52 vs. 39 μV; 209 vs. 133 μV, P < 0.01) as were OP4 and OP5 (37.2 vs. 15.6 μV; 47.5 vs. 22.2 μV, P < 0.01), but OP1-OP3 did not differ. ERGs have shorter ITs in people with blue irides than in those with dark pigmentation. Amplitude differences are highly non-linear and substantially larger from eyes with light pigmentation for components thought to be associated with the OFF retinal pathways.

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Section: Discussionsupporting

confidence: 92%

“…It has been reported that human eyes with lighter fundi demonstrate larger full-field ERGs under both light-and dark-adapted conditions with normal or supranormal amplitudes in those with albinism [2][3][4][5][6][7]. Fundus pigmentation corresponds closely to iris pigmentation, iris colour and skin pigmentation [8][9][10].…”

Section: Introductionmentioning

confidence: 99%

Light- and dark-adapted electroretinograms (ERGs) and ocular pigmentation: comparison of brown- and blue-eyed cohorts

et al. 2010

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“…Given the functional importance of normally melanized pigment epithelium in both the maintenance of photoreceptor metabolism and the protection against free radical formation, it seems likely that the hypomelanotic state is itself contributory to the visual impairment. Krill's ERG findings, in ocular and oculocutaneous albino s, of supernormal scotopic A & B waves, along with normal flicker fusions, have never been adequately explained (29).…”

Section: Discussionmentioning

confidence: 99%

Hermansky-Pudlak syndrome: albinism with lipofuscin storage

1981

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The Hermansky-Pudlak Syndrome, a "tyrosinase positive' form of oculocutaneous albinism, is a triad comprising albinism, a hemorrhagic diathesis and ceroid-lipofuscin storage. A pedigree is presented showing consanguinity with a pattern of pseudodominance. Electroretinography in two isolated Hermansky-Pudlak subjects was distinctly abnormal, showing decreased rod and cone responses (as well as abnormal flicker fusion responses) in one patient, and reduced photopic and scotopic responses in another. The decreased ERG responses are discussed with reference to the known retinal abnormalities in both generalized oculocutaneous albinism and Batten's disease, another ceroid-lipofuscin storage disorder.

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“…Other conditions that may feature supernormal Ganzfeld ERG are albinism [28], vascular disorders [10,15,21], cone dystrophy [9,22,34], and siderosis [24] but the pathogenesis remains obscure. Gouras et al [11] suggested an elevated level of cyclic guanosine monophosphate (cGMP), an internal messenger that is mostly lo- Fig.…”

Section: Discussionmentioning

confidence: 99%