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Presumed vitelliform dystrophy with perimacular flecks and retinal detachment.

Seven of eight siblings of asymptomatic non-consanguineous parents were investigated. Two of them had atrophic cystoid macular degeneration and flat or subnormal electro-oculograms suggesting the diagnosis of vitelliform dystrophy. In one eye the central cystoid lesion was surrounded by atypical small whitish hyperfluorescent flecks resembling fundus flavimaculatus. In the other eye of this patient cystoid macular degeneration progressed to shallow non-rhegmatogenous detachment of the retina. One of the asymptopmatic siblings had a mild colour vision defect of tritan-type and some fleckish hyperfluorescence around the macula and another sister showed abnormal EOG responses. These patients are probably carriers of the pathological gene responsible for the disease.

Adult

The ability of protan color defectives to perform color-dependent air traffic control tasks.

Air traffic controllers perform a variety of tasks which require them to identify, discriminate and name colors. Qualification standards for this occupation require applicants to have normal color vision. Although the validity of this standard has been questioned, Adams and Tague recently presented evidence in this Journal (1985;62:744-50) that protanopes cannot perform color-dependent air traffic control tasks reliably. In our study, the results of 7 severe and 2 moderate protans are compared to those of 78 normals on a set of tasks which simulated critical tasks performed daily by air traffic controllers. The four tasks included discriminating red from black pencil marks on flight progress strips, color-naming of 1(0) and 0.1(0) discs, and identification of colored line segments embedded in a multi-colored background. The severe protans we tested performed none of the tasks as well as normals. While the performance of the moderate protans was better, statistical conclusions could not be drawn. Our set of tasks bears many similarities to the set used by Adams and Tague and it appears we were trying to answer the same questions. The results of the two studies are similar and the conclusions are the same: severe protans cannot perform color-dependent air traffic control tasks reliably.

Accidents, Aviation

Colour vision tests and colour naming by thirteen incomplete achromats in Bishnupur.

As an exploratory study six colour vision tests were given to nine male and two female achromats from the Shankhabanik community in Bishnupur, and to two additional similar males. All thirteen subjects had severe photophobia, fixation nystagmus, extreme weakness of vision (4/24 to 3/60) and the red end of the spectrum was much shortened. This research indicates that they had a form of incomplete achromatopsia, varying from an almost complete to a very severe partial loss of colour vision. The condition is inherited as an autosomal recessive. The most likely interpretation of these cases is that they are incomplete rod achromats. Their performance on the colour vision tests is tabulated, and shows complete inability to do the Ishihara test; nearly complete inability on the HRR test, with a possible slight tendency to do better in the yellow-blue than the red-green sub-tests; on Sloan's test they show approximate accordance with her results for achromats; they have severe difficulty with the dichotomous and 100-hue tests, with a possible slight tendency to make fewer errors on the G/B sections. The anomaloscope shows little abnormality of mid-matching points, but great increases in average matching ranges above the normal, although not absolute loss of colour sense, but with extreme darkening or shortening of the red end of the spectrum. Their colour naming was carefully recorded, and was fairly good occasionally, sometimes erroneous without being wildly at fault, and most often completely wrong. The records of colour naming were made, not, of course, as a form of colour vision test, but simply to illustrate the ways in which such defectives make an effort to use colour names in general use among their friends and relatives.

Adult

Scotopization and pseudoprotanomaly in blue-yellow/colour vision defects.

With a routine clinical colour vision test battery we found scotopization in 32% of retinal diseases presenting with pseudoprotanomaly as sign of an acquired type III blue-yellow colour vision defect. In blue-yellow colour vision defects of retinal origin scotopization is a transient phenomenon, present in early stages of the disease, but it is not an obligatory finding. There is no evident relationship between visual acuity and scotopization.

Color Perception Tests

Polymorphisms of red-green vision in some populations of Southern Africa.

Some 5,000 schoolboys of the Khoikhoi, Negro, "Coloured," and Malay populations were screened with the Ishihara plates, and those with defective red-green vision were diagnosed with an anomaloscope. The findings are presented in terms of the six protan and deutan mutant alleles, a few large population-samples (e.g., Nama and Zulu) being characterized by absence of the allele for protanopia. The overall frequencies of mutants range from less than 1% to over 4%. No correspondence was found between these data and linguistic affinities of eight Bantu-speaking groups, nor between the frequencies of colorblindness and previously estimated proportions of San genes in these eight populations; on the other hand, a north-south cline of increasing frequences of mutants and of dichromacies among the Bantu-speakers was noted. The overall frequency of defective red-green vision among Cape Coloureds, 3.3%, is compatible with previously estimated racial composition of this population. The Malay sample is characterized by the highest frequency of protan mutants (2%), a 1:1 protan-deutan ratio, and an overall frequency of 4% of red-green defects. The study illustrates the potential value of anomaloscopic characterization of colorblindness in attempts to evaluate human evolutionary processes.

Adolescent

Visual mechanisms for the analysis of spatial pattern.

After a brief outline of the structure and electrophysiology of the normal visual pathways, the responses, as revealed by psychophysical studies, of the visual system to spatially and temporally varying stimuli are reviewed. An appropriate network model, involving two sequentially organized classes of visual channel, is presented. Examples are given in which the psychophysical methods developed to analyse the normal visual pathways are applied to cases in which these pathways are defective (amblyopia, albinism, hemianopia, parietal cortical lesion, inhibitory central colour vision defect). These cases not only illustrate the value of psychophysical techniques in analysing disturbances of visual function but are also suggestive of the mechanisms involved in higher processing of the retinal image.

Adolescent

Spectrally selective flash early receptor potential (ERP) in dichromats.

The human spectrally selective flash early receptor potential (ERP) was studied in 12 dichromats: 6 protanopes (12 eyes) and 6 deuteranopes (12 eyes). Color filters used were Kodak Wratten filters No.23A, No.57, and No.47 for the red, green, and blue flash ERPs, respectively. The ERP amplitude was measured between the summits of R1 and R2. Mean amplitudes of the red flash ERP and green flash ERP were highly significantly decreased in the protanopes (p less than 0.001) and deuteranopes (p less than 0.01) as compared with the corresponding data in 10 normal subjects (20 eyes). The mean amplitude of the blue flash ERP was significantly lower than normal (p less than 0.001) in the deuteranopes. The mean ratio of the blue flash ERP amplitude to the red flash ERP amplitude showed a highly significant increase in the protanopes (p less than 0.001) and a highly significant decrease in the deutoranopes (p less than 0.001) compared with the mean ratio in the normal subjects, indicating a new, useful index for the objective clinical detection of congenital color defects.

Adolescent

[Multiple evanescent white dot syndrome].

A 38-year-old male patient experienced a unilateral visual acuity decrease to 20/60 and showed white dots at the level of the retinal pigment epithelial interface characteristic of multiple evanescent white dot-syndrome. Fluorescein angiography demonstrated early hyperfluorescent defects and some late staining. In spite of improvement of the visual acuity and the alterations of the fundus, an enlargement of the blind spot and some sharply demarcated depigmentations of the retinal pigment epithelium remain. This case shows, that already at the beginning of symptoms the characteristic white dots may be present. Enlargement of the blind spot and depigmentations of the retinal pigment epithelium may remain as defects after multiple evanescent white dot-syndrome.

Adult

An analysis of colour vision in 10,000 patients.

Examination of a great number of patients resulted in the depth localisation theory. The combination of this theory with the fixation-eccentrisation theory is clinically useful: acquired colour vision defects can be subdivided by the fixation mode and by signs of receptor damage. There are indications that in multiple sclerosis the slowly progressive cases present with more receptor damage than the acute cases.

Adolescent

Color matching and Stiles-Crawford effect in central serous choroidopathy.

Color matching and Stiles-Crawford effect measurement were performed in 3 patients with central serous choroidopathy, 2 normal and 1 deuteranomalous trichromats. The color matches in the affected eye of each patient were displaced to red and could be explained by the hypothesis that the visual photopigments are in reduced optical density due to receptor disorientation caused by serous elevation of the sensory retina. The Stiles-Crawford effects of the affected eyes was abnormal confirming the hypothesis of receptor disorientation. The type III color defect accompanied by pseudo-protanomaly ascribable to receptor disorientation as occurs in central serous choroidopathy may be differentiated from the type III defect without pseudo-protanomaly.

Absorption