Liebreich's sign for defective colour vision among artists.
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A red contact lens (X-Chrom lens) worn on the nondominant eye by 12 color-defective subjects caused significant improvements on the Dvorine, Ishihara, and Hardy-Rand-Rittler pseudo-isochromatic color plate tests. Color vision scores on the Farnsworth Lantern, Color Threshold Tester, and Aviation Signal Light Gun were not improved. Minimal changes were found on the Farnsworth D-15 test, aeronautical chart color identification task, visual acuity, phoria, and stereoscopic depth perception. Control and color-defective subjects perceived a change in the path of a pendulum (Pulfrich test) when viewing through the X-Chrom lens or a monocular red filter. The X-Chrom lens may require extended wearing before its optimum effect becomes apparent.
Two cases, those of a man and a woman, developed retinal periphlebitis after hormonal treatment for infertility and contraceptives, respectively. Both had a unilateral severe decrease in visual acuity, and a central scotoma and defect in color vision were also present. The fundus had retinal edema, especially of the macula, a hyperemic disc with blurred margins, normal arteries, congested veins, and marked sheathing along the main veins, with retinal hemorrhages in the inferior half of the retina. Fluorescein angiography showed a delayed filling of the veins in the affected retina and late staining of these veins. Since there was severe visual impairment in each case, systemic adrenocorticosteroids were administered, and a rapid improvement in visual acuity occurred. In one patient, a trace Marcus-Gunn sign, a few small paracentral scotomas, and a defect in color vision were permanent sequelae.
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The minimum requirements for a reliable study of (acquired) defects of colour vision have been formulated by Verriest. Taking these minimum requirements as a guide, about 200 patients were selected. For the differential diagnosis of disorders of the retina and the optic nerve the determination of the neutral zone and examination with the anomaloscope are important. This is true for both red-green and blue-yellow defects.
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A genealogical link was established six generations back between a family living in England and Australia, and one of the families reported originally by Sorsby et al (1949) as suffering from autosomal dominant inflammatory macular dystrophy (fundus dystrophy). The onset--in the fifth decade of life--and the progress of the condition, which usually ends in blindness, has been observed in a number of patients and the prodromal development of a colour vision deficiency in some of them confirmed. This defect is fundamentally different from the X-linked colour vision defects and merits further investigation.
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