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Evolution of benign concentric annular macular dystrophy.

In 1974, Deutman described a family with an autosomal dominantly inherited macular dystrophy that he termed "benign concentric annular macular (bull's-eye) dystrophy." Ten years later, we performed a follow-up examination. Some patients complained of deterioration of visual acuity, night vision, and color vision. The macular dystrophy had progressed. The fundus periphery was more involved and in two patients there were bone corpuscle-like pigmentations. Electrophysiologic examination showed increased photoreceptor dysfunction with equal involvement of the rod and cone system. The patients had an acquired type III blue-yellow color vision defect with pseudoprotanomaly.

Adult↗

[Acute retinal epithelitis: uncommon symptoms in a clinical case].

CASE REPORT: We describe an acute retinal pigment epithelitis (A.R.P.E) case in a young woman who noticed an acute decreased vision and color vision defects. The patient was treated with systemic nonsteroidal anti-inflammatory drugs (NSAIDs) with recovery of visual acuity to normal in three weeks. DISCUSSION: We highlight the debut with important decrease of visual acuity at levels that are not common in this illness. The disturbance in color vision is a rare symptom. The visual acuity recovery in a short period of time is what usually happens, and in this case it is just three weeks, not knowing how it relates to NSAID.

Acute Disease↗

Parallel pathways for spectral coding in primate retina.

The primate retina is an exciting focus in neuroscience, where recent data from molecular genetics, adaptive optics, anatomy, and physiology, together with measures of human visual performance, are converging to provide new insights into the retinal origins of color vision. Trichromatic color vision begins when the image is sampled by short- (S), middle- (M) and long- (L) wavelength-sensitive cone photoreceptors. Diverse retinal cell types combine the cone signals to create separate luminance, red-green, and blue-yellow pathways. Each pathway is associated with distinctive retinal architectures. Thus a blue-yellow pathway originates in a bistratified ganglion cell type and associated interneurons that combine excitation from S cones and inhibition from L and M cones. By contrast, a red-green pathway, in which signals from L and M cones are opposed, is associated with the specialized anatomy of the primate fovea, in which the "midget" ganglion cells receive dominant excitatory input from a single L or M cone.

Animals↗

Positional adaptation reveals multiple chromatic mechanisms in human vision.

Precortical color vision is mediated by three independent opponent or cardinal mechanisms that linearly combine receptoral outputs to form L/M, S/(L+M), and L+M channels. However, data from a variety of psychophysical and physiological experiments indicate that chromatic processing undergoes a reorganization away from the basic opponent model. Frequently, this post-opponent reorganization is viewed in terms of the generation of multiple "higher order" chromatic mechanisms, tuned to a wide variety of axes in color space. Moreover, adaptation experiments have revealed that the synthesis of these mechanisms occurs at a level in the cortex following the binocular integration of the inputs from each eye. Here we report results from an experiment in which the influence of chromatic adaptation on the perceived visual location of a test stimulus was explored using a Vernier alignment task. The results indicate that not only is positional information processed independently within the L/M, S/(L+M), and L+M channels, but that when adapting and test stimuli are extended to non-cardinal axes, the existence of multiple chromatically tuned mechanisms is revealed. Most importantly, the effects of chromatic adaptation on this task exhibit little interocular transfer and have rapid decay rates, consistent with chromatic as opposed to contrast adaptation. These findings suggest that the reorganization of chromatic processing may take place earlier in the visual pathway than previously thought.

Adaptation, Ocular↗

Eye disease and color defects.

The groundwork for understanding color defects in eye disease was established by the end of the nineteenth century. Thereafter the field was neglected as scientists concentrated on studies of normal color vision and congenital color vision defects. Spurred by the development of the Farnsworth 100 hue-test, interest was renewed in the 1950s. The past 25 years have seen an explosion of interest in color defects in eye disease. The International Research Group on Color Vision Deficiencies has played an important role in this activity. The development of new clinical tests and instruments as well as refinement of laboratory techniques are among the important developments.

Color Perception Tests↗

The X-Chrom lens. On seeing red.

A broadband red filter placed over one eye will have the effect of improving the ability of certain color vision defectives to name colors correctly. A red-tinted contact lens will have the same effect; such a device is marketed under the name, the X-Chrom lens. In this article, the author examines the basic properties of color vision defects, explains the optical effect of the red filter, and reviews several studies which evaluate the effect of the X-Chrom lens. He concludes that while the lens may allow the wearer to achieve a better score on certain color vision tests, it does not actually correct color vision in the natural environment. Deleterious effects of wearing a red filter are also discussed. It is advised that patients wearing the X-Chrom lens be fully apprised of its limitations and of dangers that may be created by the associated visual distortions.

Color Perception Tests↗

Early detection of inborn dyschromatopsias in preschoolers and young schoolchildren.

Color vision is one of the most important factors of human vision. Early detection of color vision malfunction in children is of utmost importance, allowing parents and teachers to take appropriate and timely measures for children's future professional orientation. A cohort of 300 children aged 4-6 years (49-86 months) were examined by color vision testing and divided into three age groups of 4-5, 5-6 and > 6 years. Their ability to name the fundamental spectral colors and read Ishihara's pseudoisochromatic plates were recorded. The results obtained are presented in tables and figures. Data analysis showed the youngest group of children to be unsuitable for such testing of color vision. Numerical data revealed considerable percentage deviations as compared to the older groups. In addition, a great difference between the girls and boys indicated psychomotor development to be much faster in girls of that age. Generally, data obtained by the distribution of the results for the two older age groups were consistent with those found in the literature; thus, the authors propose an early examination for the detection of inborn chromatopsia to be performed in preschool children, but not before the age of five. Ishihara's pseudoisochromatic test can be useful in differentiating between dyschromatic and other children, but it is very difficult to implement for singling out dyschromates according to the protan or deutan type.

Age Factors↗

Ability of the D-15 panel tests and HRR pseudoisochromatic plates to predict performance in naming VDT colors.

Color codes in VDT displays often contain sets of colors that are confusing to individuals with color-vision deficiencies. The purpose of this study is to determine whether individuals with color-vision deficiencies (color defectives) can perform as well as individuals without color-vision deficiencies (color normals) on a colored VDT display used in the railway industry and to determine whether clinical color-vision tests can predict their performance. Of the 52 color defectives, 58% failed the VDT test. The kappa coefficients of agreement for the Farnsworth D-15, Adams desaturated D-15, and Richmond 3rd Edition HRR PIC diagnostic plates were significantly greater than chance. In particular, the D-15 tests have a high probability of predicting who fails the practical test. However, all three tests had an unacceptably high false-negative rate (9.5-35%); so that a practical test is still needed.

Color Perception↗

Farnsworth-Munsell 100-hue test for patients with diabetes mellitus.

We evaluated 164 eyes of 87 patients with diabetes mellitus compared with 50 eyes from 25 healthy subjects as the control group. We compared 87 patients with diabetes mellitus (164 eyes) in relation to their duration of diabetes, fundus findings, visual acuity, and color vision defects. In all patients, color vision defects were determined using the Farnsworth-Munsell 100-hue test, and the total error score was established on the basis of age norms from subjects without diabetes. No color vision defect was detected in the control group. In the diabetic group, fundus degeneration and color vision defects were observed and correlated with the duration of diabetes. The dominant color defect was of the blue-yellow type.

Adolescent↗

[Cerebral achromatopsia (symptoms, course, differential diagnosis and strategy of the study). I].

To the patient, the sudden onset of cerebral achromatopsia is like switching to black and white on a color TV. As a rule, the defect arises due to bilateral ischemic infarction in the inferior occipitotemporal region. Bilateral upper homonymous quadrantanopsias usually leave the macula more or less unimpaired, so that visual acuity is largely preserved. Prosopagnosia and loss of topographic memory are often associated with central achromatopsia. Investigations of color vision must include color-naming procedures and largefield tests in addition to the conventional methods. Color-naming tasks are indispensable in differentiating cerebral achromatopsia from the aphasic and disconnective types of color anomia. The authors' recommended strategy for investigating color vision relies on records of a case of cerebral achromatopsia obtained six months and two years, respectively, after the onset of symptoms. In addition to the above-mentioned procedures, spectral increment thresholds on white and colored backgrounds were determined. For the first time in cerebral achromatopsia, examinations with large-field spectral matches were performed using the projection anomaloscope. Large-field tests are indispensable for monitoring recovery in cases of central achromatopsia. In the author's patient, recovery of blue-green discrimination was far more complete than that of red-yellow-green discrimination, and for both conditions large-field color vision was far superior to small-field.

Aged↗

[Cerebral achromatopsia (symptoms, course, differential diagnosis and examination strategy). II].

To the patient, the sudden onset of cerebral achromatopsia is like switching to black and white on a color TV. As a rule, the defect arises due to bilateral ischemic infarction in the inferior occipitotemporal region. Bilateral upper homonymous quadrantanopsias usually leave the macula more or less unimpaired, so that visual acuity is largely preserved. Prosopagnosia and loss of topographic memory are often associated with central achromatopsia. Investigations of color vision must include color-naming procedures and large-field tests in addition to the conventional methods. Color-naming tasks are indispensable in differentiating cerebral achromatopsia from the aphasic and disconnective types of color anomia. The authors' recommended strategy for investigating color vision relies on records of a case of cerebral achromatopsia obtained six months and two years, respectively, after the onset of symptoms. In addition to the above-mentioned procedures, spectral increment thresholds on white and colored backgrounds were determined. For the first time in cerebral achromatopsia, examinations with large-field spectral matches were performed using the projection anomaloscope. Large-field tests are indispensable for monitoring recovery in cases of central achromatopsia. In the author's patient, recovery of blue-green discrimination was far more complete than that of red-yellow-green discrimination, and for both conditions large-field color vision was far superior to small-field.

Anomia↗

[Revisions in the assessment of fitness for aircraft flying].

So far medical requirements for aviators have been defined by national licensing authorities. For a long while ophthalmological problems, insufficiencies in vision, refraction or color vision, have been responsible for approximately 50% of rejection of applicants for medical reasons. New international regulations proposed as well by ICAO as JAA try to lead to a new balance in these requirements. In general, ophthalmological requirements now allow for lower standards, specifically in refraction, but also in color vision. Also new examination methods are admitted. German and French authorities have announced their opposition being concerned about flight safety for the future.

Aerospace Medicine↗

The red-green visual pigment gene region in adrenoleukodystrophy.

Although recent data established that a specific very-long-chain fatty acyl-CoA synthetase is defective in X-linked adrenoleukodystrophy (ALD), the ALD gene is still unidentified. The ALD locus has been mapped to Xq28, like the red and green color pigment genes. Abnormal color vision has been observed in 12 of 27 patients with adrenomyeloneuropathy (AMN), a milder form of ALD. Furthermore, rearrangements of the color vision gene cluster were found in four of eight ALD kindreds. This led us to propose that a single DNA rearrangement could underlie both ALD and abnormal color vision in these patients. Study of 34 French ALD patients failed to reveal a higher than expected frequency of green/red visual pigment rearrangements 3' to the red/green color vision gene complex. The previous report of such rearrangements was based on small numbers and lack of knowledge that the frequency of "abnormal" color vision arrays on molecular analysis was twice as high as expected on the basis of the frequency of phenotypic color vision defects. The red/green color pigment (R/GCP) region was studied by pulsed-field gel electrophoresis in 14 of these patients, and we did not find any fragment size difference between the patients and normal individuals who have the same number of pigment genes. The R/GCP region was also analyzed in 29 French and seven North American ALD patients by using six genomic DNA probes, isolated from a cosmid walk, that flank the color vision genes. No deletions were found with probes that lie 3' of the green pigment genes. One of the eight previously reported ALD individuals has a long deletion 5' of the red pigment gene, a deletion causing blue cone monochromacy. This finding and the previous findings of a 45% frequency of phenotypic color vision defects in patients with AMN may suggest that the ALD/AMN gene lies 5' to the red pigment gene and that the frequent phenotypic color vision anomalies owe their origin to deleted DNA that includes regulatory genes for color vision. It is possible, however, that phenotypic color vision anomalies in AMN may be phenocopies secondary to retinal or neural involvement by the disease. The single case of blue cone monochromacy may therefore be a fortuitous coincidence of two diseases.

Adrenoleukodystrophy↗

X chromosome and color blindness.

A red-green color blindness was studied in four families. All the carriers of the gene (mothers and sisters of the color blind individuals) had normal color vision. The color blind individuals were protanomalous or deuteranomalous. No protanopia or deuteranopia was found in the subjects who have been studied.

Color Vision Defects↗