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[Disorders of color vision].

This review gives a summary of all colour vision disorders (dyschromatopsias) and diagnostic methods and tests. Colour vision is inadequately treated in current literature with regard to the choice of diagnostic methods and the interpretation of results for a single disorder, which contributes to wrong dyschromatopsia diagnosing seen every day in specialist practice. Examination for colour disorders is usually outpatient and is carried out by ophthalmology or neurology departments or occupational health services under the supervision of an ophthalmologist to prevent misinterpretation of results and wrong occupational choices. The problem is very serious, and proper education should be able to provide guidelines for correct and early diagnosis of dyschromatopsia. As the examination is not well defined, it is very important to set unique criteria in diagnosing any single colour vision disorder.

Color Perception Tests↗

The multifocal pattern electroretinogram in chloroquine retinopathy.

PURPOSE: Optimal screening for ocular toxicity caused by chloroquine and hydroxychloroquine is still controversial. With the multifocal pattern electroretinogram (mfPERG), a new electrophysiological technique has recently become available to detect early changes of ganglion cells. In this study this new technique is applied to a series of 10 patients seen consecutively receiving long-term chloroquine medication. METHODS: In 10 patients receiving chloroquine medication, clinical examination, Amsler visual field testing and computerized color vision testing were performed. If toxicity was suspected, automated perimetry was carried out. In addition, in all patients conventional pattern electroretinogram (PERG) and mfPERG testing were performed. RESULTS: On clinical examination 8 patients showed no chloroquine-associated maculopathy, while 2 patients did. Of these 2, only 1 reported abnormalities when viewing the Amsler chart, while automated perimetry showed typical, ring-like paracentral scotomas in both affected patients and color vision was significantly abnormal. In the normal patients, 4 of 8 had a mild color vision disturbance, which correlated to age-related macular changes. The amplitudes of the PERG and the central (approximately 10 degrees ) responses of the mfPERG were markedly reduced in chloroquine maculopathy, while the latencies were unchanged. The peripheral rings of mfPERG (ranging to 48 degrees ) were not affected by chloroquine toxicity. Both PERG and mfPERG were less affected by age-related macular changes. CONCLUSIONS: The reduction of PERG and central mfPERG responses in chloroquine maculopathy may help with the early detection of toxicity.

Adult↗

The use of colour vision measurement in the diagnosis of digoxin toxicity.

Colour vision has been measured, using the Farnsworth-Munsell 100-Hue test, in control subjects and in patients taking digoxin. In 10 patients with digitalis toxicity, of whom only two described symptomatic abnormalities, colour vision was impaired compared with that of both control subjects and non-toxic patients who had been taking digoxin for more than two months. Withdrawal of digoxin from toxic patients led to improved colour vision. Colour vision scores correlated well with (a) log plasma digoxin concentrations, and (b) various measures of the pharmacodynamic effects of cardiac glycosides on cation transport in the patients' own erythrocytes. These results suggest that colour vision assessment may be of use in screening for digitalis toxicity at the bedside and in assessing the degree of digitalis intoxication in an individual patient as an aid to clinical research.

Adult↗

Vision defects in albinism.

We have examined the possible presence of color vision anomalies in 9 individuals (17 eyes, 1 blind) with fundus findings suggesting ocular albinism using the Ishihara plates, the 28-hue Roth test, and the Davico anomaloscope. Results indicate that four of these individuals show no sign of the anomalies expected in an albino in either of the two eyes. Of the remaining cases, two are simple deuteranomals in both eyes, according to Pickford's classification criteria. The rest have protanomaly; however, in these the deviation toward red appears in both eyes in only one subject, whereas in the other two subjects it appears in only one eye, their binocular color vision being basically normal. Our study shows that a large proportion of these albinos have photophobia, pendular nystagmus, strabismus, noticeable refractive errors (astigmatism and high myopia), and poor visual acuity [usually less than 6/30 (20/100) with correction]. The measurement of contrast sensitivity function (CSF) indicates that the frequency of 12 cpd cannot be perceived, even in binocular vision.

Adolescent↗

The use of the Lanthony New Color Test in determining the effects of aging on color vision.

The primary purpose of this study was to collect data on the loss of color vision as a function of age. The Lanthony New Color Test (NCT), which measures acquired losses of color vision in the dimensions of hue, saturation, and brightness, was used to compile data on 68 subjects. The minimum number of subjects were 10 per decade from age 30 to 90 years. An age gradient of selective loss of discrimination of saturation beginning at age 50 was demonstrated, with rapid change noted after age 60. Similar findings were seen for hue but were not evident for brightness. By age 70, a neutral zone emerged at blue/purple, Munsell chroma level 2. The instrument was shown to be reliable and valid in comparison to the Farnsworth Dichotomous Panel D.15. It is seen that this information will provide a basis for planning safer, more functional environments for elderly people.

Adult↗

Mutation in the gene GUCA1A, encoding guanylate cyclase-activating protein 1, causes cone, cone-rod, and macular dystrophy.

PURPOSE: To determine the underlying molecular genetic basis of a retinal dystrophy identified in a 4-generation family and to examine the phenotype and the degree of intrafamilial variability. DESIGN: Prospective case series. PARTICIPANTS: Six affected individuals from a nonconsanguineous British family. METHODS: Detailed ophthalmologic examination, color fundus photography, autofluorescence imaging, and electrophysiologic assessment were performed. Blood samples were taken for DNA extraction, and mutation screening of GUCA1A, the gene encoding guanylate cyclase-activating protein 1 (GCAP1), was undertaken. RESULTS: All affected subjects complained of mild photophobia and reduced central and color vision. Onset was between the third and fifth decade, with subsequent gradual deterioration of visual acuity and color vision. Visual acuity ranged between 6/9 and counting fingers. Color vision was either absent or markedly reduced along all 3 color axes. A range of macular appearances was seen, varying from mild retinal pigment epithelial disturbance to extensive atrophy. Electrophysiologic testing revealed a range of electrophysiologic abnormalities: isolated cone electroretinography abnormalities, reduced cone and rod responses (with cone loss greater than rod), and isolated macular dysfunction. The 4 coding exons of GUCA1A were screened for mutations in affected and unaffected family members. A single transition, A319G, causing a nonconservative missense substitution, Tyr99Cys, segregated uniquely in all affected subjects. CONCLUSIONS: The Tyr99Cys GUCA1A mutation has been previously shown to cause autosomal dominant progressive cone dystrophy. This is the first report of this mutation also causing both cone-rod dystrophy and isolated macular dysfunction. The phenotypic variation described here exemplifies the intrafamilial heterogeneity of retinal dysfunction that can be observed in persons harboring the same mutation and chromosomal segment.

Adult↗

Rayleigh match ranges of red/green color-deficient observers: psychophysical and molecular studies.

Large-field Rayleigh match ranges were measured in 27 red/green color-deficient male observers, using bright, temporally alternating, 3-9 deg annular test fields. The observers' X-linked opsin gene arrays were characterized by molecular genetic techniques, and used to infer the absorption maxima of each observer's L and/or M cone photopigment(s). Measured match ranges decreased rapidly as the inferred separation in pigment absorption maxima increased from 0 to 2-3 nm, and varied irregularly thereafter. Following He & Shevell [(1995) Vision Research, 35, 2579-2588] predicted match ranges were calculated for various pigment separations and assumed values of pigment optical density. The predicted variations in match range encompassed the measured match ranges of most (but not all) of the color-deficient observers. The calculations also showed that differences in pigment optical density, in two cone types containing the same pigment, are sufficient to allow a moderate degree of chromatic discrimination. Such models thus provide a possible account of the fact that some color-deficient observers, with only a single X-linked opsin gene, can make red/green chromatic discriminations.

Adolescent↗

[Evaluation of desaturated Panel D-15. III. Evaluation of the validity of the desaturated Panel D-15].

The estimation of the validity of a test refers to whether this test measures what it claims to measure. The validity of the desaturated panel D-15 was estimated in 248 subjects with congenital hereditary dyschromatopsia. The method used was based on a qualitative and quantitative study of the so-called lines of confusion joining the positions of the colored caps on the classical Farnsworth's diagram. The qualitative estimation was evaluated according to the prevailing number of lines of confusion which were parallel to a given line of reference of the diagram; it was related to the axis of the dyschromatopsia. The comparison of the results with results of Nagel's anomaloscope used as a reference test, demonstrated an overvaluation of the number of the protan subjects (anomaloscope: 77; desaturated D-15: 107) and an undervaluation of the number of deutan subjects (anomaloscope: 169; desaturated D-15: 70). This kind of discrepancy was not apparent with the standard Panel D-15. The quantitative estimation was evaluated according to the whole number of lines of confusion whatever their directions; it was related to the severity of the dyschromatopsia. The comparison of the results with the results of Nagel's anomaloscope demonstrated that the dichromatic and extreme anomalous trichromatic subjects nearly always (40 subjects out of 42) had 7 lines of confusion, or more; the simple anomalous trichromatic subjects had a variable number of lines of confusion, from 1 to 8, without any prevalence in the great majority of cases (193 subjects out of 204).(ABSTRACT TRUNCATED AT 250 WORDS)

Color Perception Tests↗

Clinical experience with the Lovibond Colour Vision Analyser. Results from the examination of normal and congenital colour-deficient subjects.

The Colour Vision Analyser was used for testing 98 persons of both sexes, aged from 10 to 70 years, and recognized as normal by means of pseudo-isochromatic plates and an anomaloscope. A drop of the saturation thresholds from yellow to green and from blue to purple was observed from the age of 40 years. The saturation thresholds from yellow to green was found lower in every age group than that from blue to purple. Congenital colour defects could be completely distinguished from normal subjects. As for the classification in types, those diagnosed as deutan by means of the anomaloscope were also diagnosed as deutan by the Analyser, however, there were, among those diagnosed as protan by the anomaloscope, some subjects who were diagnosed as deutan by the Analyser. Nearly all cases could be classified as anopia or anomaly.

Adolescent↗

Clinical analysis of colour vision deficiencies with The City University test.

The City University colour vision test (CUCVT) was used for the examination of 158 subjects suffering from congenital colour vision defects (36 protanopes, 122 deutanopes) and its results were compared with that of an anomaloscope and of the panel D-15. 23% of the subjects classified as protanopes and 98% of the subjects classified as deuteranopes by means of the anomaloscope were also classified as such by means of the CUCVT, while 93% of the subjects classified as protanomalous and 90% of the subjects classified as deuteranomalous by means of the anomaloscope gave normal answers at the CUCVT. The results of the CUCVT were almost the same as with panel D-15 except protanopia. The colour spots of each plate of the CUCVT were plotted on a CIE chromaticity diagram and the results of this study are also reported.

Adolescent↗

Wavelength discrimination deteriorates with illumination in blue cone monochromats.

Two types of incomplete congenital achromats were studied: one type (blue cone monochromats) has a conspicuous short wavelength cone mechanism, and the other type (deutan incomplete achromats) has a conspicuous long wavelength cone mechanism. The photoreceptor mechanisms were inferred from color matches and from test action spectra measured on rod-saturating backgrounds of different wavelengths. Interestingly, the illumination-dependency of color discrimination (for 5 degrees bipartite fields that were centrally fixated) differed between the two patient types, even though rhodopsin photoreceptors were common to both. As illumination level increased, the ability to discriminate wavelength differences deteriorated for the blue cone monochromats, whereas, for the deutan achromats, wavelength discrimination remained relatively constant even near 100,000 scotopic trolands. The performance decrement in the blue cone monochromats was probably not associated with rod saturation, as the field action spectrum to cause a just-noticeable-difference (jnd) decrement in discrimination was poorly fitted by a rhodopsin action spectrum. In addition, the blue cone monochromats had rhodopsin photoreceptors that did not saturate in bright illuminations. The authors hypothesize that the deterioration of wavelength discrimination at high illuminations is not an abnormality of blue cone monochromacy. Rather, it may be a property of the normal color mechanism through which signals from the short wavelength cones pass.

Adolescent↗

Large-field color naming of dichromats with rods bleached.

A color-naming method was used to examine the large-field red/green discriminations of dichromats screened with standard tests. The stimulus was a 12 degrees annular field with the central 4 degrees removed, flashed for 300 ms. Four wavelengths were equated in brightness for each observer at two retinal illuminance levels, approximately 10 and 100 trolands. The stimuli were then presented in random order and the observer was asked to name each, using one of four color terms. The entire experiment was done with the observers dark-adapted and also with the rods bleached. For all four deuteranopes and two of four protanopes, color names were very significantly related to both illuminance and wavelength in both adaptation conditions. The relationship between name and wavelength was similar to that of a normal trichromat, but the performance of a dichromat was very poor by comparison. Performance was generally somewhat better in the dark than with the rods bleached. However, the result in the bleached condition is consistent with recent evidence that at least some observers who are classified as dichromats with standard small-field screening procedures actually have a weak residual third cone mechanism.

Adaptation, Ocular↗

Color vision sensitivity in normally dichromatic species and humans.

Spectral-sensitivity functions for large, long-duration increments presented on a photopic white background indicate that wavelength-opponent mechanisms mediate detection in both normal and dichromatic humans. Normal humans exhibit high color-vision sensitivity as they discriminate the color of spectral flashes at detection-threshold intensities. However, dichromatic humans require stimuli up to about 0.4 log units above detection intensity to see certain colors. This low color-vision sensitivity in human dichromats may be an abnormal condition involving a defect in postreceptoral color processing. To test this hypothesis, we determined color-discrimination thresholds in normally dichromatic species: chipmunk, 13-lined ground squirrel, and tree shrew. For comparison, we also tested humans with normal and abnormal (deutan) color vision with the same apparatus and methods. Animals were trained to perform spatial two-choice discrimination tasks for food reward. Detection thresholds were determined for increments of white, 460 nm, 540 nm, 560 nm, 580 nm, 500 nm/long-pass, and 500 nm/short-pass on white backgrounds of 1.25 cd/m2, 46 cd/m2, and 130 cd/m2. Animals were also trained to respond to the colored increments when paired with the white increment when both were at equally detectable intensities. Color-discrimination thresholds were determined by dimming stimulus pairs (colored vs. white) until the subjects could no longer make the discriminations. Results indicated that the normally dichromatic species could discriminate colored stimuli from white at a mean intensity of 0.1 (+/-0.1) log units above detection threshold. The ability of normally dichromatic species to discriminate color near detection-threshold intensity is consistent with increment spectral-sensitivity functions that indicate detection by wavelength-opponent mechanisms. In keeping with previous studies, normal human trichromats discriminated color near detection-threshold intensities but humans with deutan color vision required suprathreshold intensities to discriminate the color of middle and long wavelengths. This high color-vision sensitivity of normally dichromatic species suggest that the low color-vision sensitivity in dichromatic humans is an abnormal condition and indicates a possible defect in their postreceptoral color-vision processing.

Animals↗

Color vision measured with pseudoisochromatic plates at five-and-a-half years in eyes of children from the CRYO-ROP study.

PURPOSE: To investigate the prevalence of color deficits at age 5 1/2 years in preterm children with birth weights of less than 1251 g who participated in the multicenter Cryotherapy for Retinopathy of Prematurity (CRYO-ROP) study. METHODS: Two cohorts of CRYO-ROP participants served as subjects: 1055 children who participated in a long-term study of the natural history of ROP at 5 of the 23 CRYO-ROP centers, and 187 children (from all 23 study centers) who had threshold ROP in both eyes and who were randomized to receive cryotherapy in 1 eye. Monocular color vision was tested at age 5 1/2 years, using the Standard Pseudoisochromatic Plates, part 2 (SPP2) for acquired color vision defects. RESULTS: In the Natural History cohort, prevalence of red-green (R-G) color deficits was 6.6% for males and 1.0% for females, similar to that of the general adult population. Prevalence of blue-yellow (B-Y) color deficits was 2.8% for males and 2.2% for females, more than 200 times that in the general adult population. Prevalence of B-Y deficits was not related to birth weight, gestational age, acute-phase ROP, optic atrophy, or retinal residua of ROP, but was related to visual acuity. In the Threshold ROP cohort, color vision deficits were no more likely in eyes that had received cryotherapy than in control eyes. CONCLUSIONS: The results confirm an increased prevalence of B-Y deficits in children born before term, and provide evidence that the increased prevalence is not related to birth weight, gestational age, or severity of ROP within this group of preterm children. No evidence was found to indicate that cryotherapy increased the rate of color vision deficits in eyes with threshold ROP.

Birth Weight↗

Psychophysical flicker thresholds and ERG flicker responses in congenital and acquired vision deficiencies.

Psychophysical flicker thresholds and ERG flicker responses were compared with Farnsworth's 100-hue colour test data in cases of congenital and acquired vision deficiences. The results, evaluated in term of sensitivity and specificity lead to some practical and theoretical suggestions. In most cases, flicker responses and colour tests results were better than other classical tests for evaluating the first signs or gravity importance of a macular disturbance. Relatively minor differences between the 3 tests may be related to the observed or supposed extension of lesion in the macular area, but in some cases the observed functional changes can be understood only by admitting some lateral effect from one area to another. Generally acquired colour differences are associated with flicker response changes. In congenital red-green blindness of different gravity, moderate abnormal white light flicker responses could be observed. But they do not seem to be associated clearly with a type and gravity of the deficiency. With coloured stimuli, the difference between normal and red-green colour-blindness is more clear-cut, and red-blind can be discriminated from green-blind. Subclinical retinal pathology as well as specific stimulus conditions in normal and red-green colour-blind subjects reveal some non-linear processes in the low-frequency range of the temporal attenuation characteristics.

Adolescent↗

An acquired color defect of the opponent-color system.

An acquired unilateral color defect in a 22-year-old man has been investigated with standard clinical tests and by using techniques which, it is thought, test specifically for the sensitivity of the luminance and opponent-color systems. The spectral sensitivity of the defective left eye, using 1 degree 200 ms. test flashes on a white background, has a single broad peak at about 550 nm. and resembles the photopic luminosity curve; in contrast, the normal curve, measured in the same conditions, has three peaks at about 440, 520, and 600 nm. However, the subject's spectral sensitivity curve for detecting 20 Hz. flicker is quite normal and is similar to his curve for 200 ms. flashes. It has recently been proposed that the three peaks of the normal curve for 200 ms. flashes reflect the activity of the opponent-color system, whereas the single peak for flicker detection is related to the luminance system. The preceding observations may thus be interpreted in terms of a specific loss of the subject's opponent-color system and this would explain his poor color discrimination. His luminance system appears to be normal, and evidence is presented for the maintained function of red- and green-sensitive (but not blue-sensitive) cones. The spectral sensitivity of the subject's right eye is nearly normal, suggesting a precortical origin of the defect; however, there seems to be some abnormality in this eye, indicating a less developed form of the same defect.

Adult↗

Impact of congenital colour vision defects on occupation.

AIMS: To investigate whether there is an association between congenital colour vision defects (CVD) and occupational choice and employment history, in order to inform the debate about the value of universal childhood screening for these disorders. METHODS: Participants were 6422 males and 6112 females from the 1958 British birth cohort, followed from birth to 33 years, whose colour vision was assessed (Ishihara test) at 11 years. RESULTS: A total of 431 males (6.7%) had CVD. Men with CVD had pursued some careers for which normal colour vision is currently regarded as essential; for example, eight men (3.1%) with CVD were in the police, armed forces, or fire-fighting service at 33 years compared to 141 men (3.8%) with normal colour vision. They were, however, under-represented compared to those with normal colour vision, in other occupations; for example, no men with CVD were employed in electrical and electronic engineering at 33 years compared to 15 men (0.4%) with normal colour vision. CONCLUSIONS: The findings of this study suggest there is little to be gained by continuing with existing school screening programmes for CVD, whose primary purpose is to advise affected children against certain careers. Other ways of informing young people about potential occupational difficulties and pathways for referral for specialist assessment are likely to be more useful.

Career Choice↗

The dyschromatopsia of optic neuritis is determined in part by the foveal/perifoveal distribution of visual field damage.

Most hypotheses of acquired dyschromatopsia invoke the mechanism of selective damage to specific components of the afferent visual system to explain the predominance of red-green and blue-yellow hue-discrimination defects found in neural and retinal disorders, respectively. However, this pattern of hue-discrimination disturbance in ocular disease may vary. There are frequent exceptions which are inadequately explained by existing hypotheses. In an effort to explain the pattern and pathogenesis of acquired dyschromatopsias better, the authors examined patients with nonproliferative diabetic retinopathy (DR) and late-stage retrobulbar neuritis (RBN) using age-corrected Farnsworth-Munsell 100-hue testing and threshold static perimetry. As expected, most DR eyes showed some degree of relative blue-yellow dyschromatopsia (89%) with few showing a greater weighting towards red-green dyschromatopsia (11%). However, an approximately equal number of RBN eyes had a relative blue-yellow (48%) versus red-green dyschromatopsia (52%). For RBN, the authors found a strong association between the spatial distribution of field defect and the type of relative hue-discrimination disturbance. Eyes with greater field depression at the fovea relative to the perifovea showed a relative preponderance of red-green dyschromatopsia (68%) as opposed to blue-yellow dyschromatopsia (32%), whereas eyes with greater relative perifoveal impairment showed a relative preponderance of blue-yellow dyschromatopsia (100%). This relationship between the relative spatial distribution of visual field damage and the relative hue-discrimination deficit in RBN was statistically significant (P = 0.002). Such an association was not found for DR.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗