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PubMed1991

High myopia with cone dysfunction.

All 3 children, 2 boys and 1 girl (the probands), in a family had high myopia and subnormal visual acuities. The boys had high myopia in both eyes, the girl had high myopia in 1 eye and low myopia in the other eye. Both of the boys had a protanomalous colour vision defect. The colour vision testing of the high myopic eye of the girl was not successful, the other eye had normal colour vision. In the electroretinogram examination, both cone and rod responses were decreased in 2 of the children. In the family study, results of an eye examination of 30 relatives were available. No other cases of high myopia or subnormal visual acuities were found. The father of the children, 1 of the paternal relatives, and 5 of the maternal relatives had low myopia. One maternal male cousin of the probands had a protanomalous colour vision defect. In the genealogical study, no relationship was found between the families of the father and the mother of the probands going back to the fifth generation. The heredity of this disorder is difficult to define. It could be autosomal dominant or recessive if the myopia only are taken into consideration. If the high myopias and cone dysfunction are considered to be parts of the same syndrome, the heredity could be x-chromosomal recessive or autosomal recessive.

Adolescent↗

PubMed1982

Crossed-quadrant homonymous hemianopsia. The "checkerboard" field defect.

A 70-year-old man with a history of hypertension and coronary artery disease suffered an abrupt loss of vision in June 1980. Neuro-ophthalmologic examination in August 1981 revealed 20/20+ acuity in both eyes, but quantitative perimetry disclosed a classic crossed-quadrant homonymous hemianopsia. This is known as the "checkerboard" visual field defect; a right upper quadrantanopsia as well as a left lower quadrantanopsia. A review of the eight previously reported cases is presented. A trial with "checkerboard" Fresnel prisms gave only a slight improvement in ambient field in this patient. The significance of that point is discussed. To our knowledge, this is the first patient with a "checkerboard" occipital lobe infarction pattern documented by computed tomography.

Aged↗

PubMed1993

Colour blindness and natural selection: studies in four nomadic tribal groups from Andhra Pradesh, India.

438 males and 369 females from four endogamous nomadic groups of the Yerukala viz. Suvvi, Badda, Uppu and Kunchapuri of Andhra Pradesh (India) were examined for red-green colour blindness using the 15th edition of Isihara's colour blindness chart. The absence of defective colour vision in the Badda Yerukala and very low frequencies among the other groups agree not only with the hypotheses of Post (1962) and Pickford (1963) concerning the operation of natural selection, but corroborates, too, the opinion of Malhotra (1978) that nomads by virtue of their life-style demand good colour vision.

Color Vision Defects↗

PubMed1983

Pseudoisochromatic plate design--Macbeth or tungsten illumination?

Three sets of pseudoisochromatic plates were evaluated by photometry and colorimetry. The luminance contrast between the figure and background was measured and compared with a contrast detection threshold. The chromaticity coordinates of the figure and background were evaluated on the basis of how closely they approached a dichromatic line of confusion. The separation of the coordinates of the figure and background are a measure of the severity of the defect for which the plate tests. The plates were evaluated under both Macbeth (C) and tungsten (A) illuminants; two sets of plates were found to be better designed for tungsten illumination.

Color Perception Tests↗

PubMed2006

Choroideremia: variability of clinical and electrophysiological characteristics and first report of a negative electroretinogram.

PURPOSE: To analyze the variability of clinical and electrophysiological characteristics in X-linked choroideremia and provide the first report of a negative electroretinogram in choroideremia. DESIGN: Retrospective study. PARTICIPANTS: The records of 18 male patients with choroideremia and 8 female carriers were evaluated. METHODS: The data were reviewed regarding visual acuity (VA), color vision, perimetry, fundus autofluorescence, and full-field electroretinography (according to standards of the International Society for Clinical Electrophysiology of Vision). MAIN OUTCOME MEASURES: Morphological and functional phenotype characteristics, fundus autofluorescence, electroretinography, and Rab escort protein 1 (REP-1) mutations. RESULTS: Four unrelated families with choroideremia (9 affected males, 7 carriers) and 10 unrelated individuals (9 affected males, 1 carrier) were included. Mutational analysis, performed in 2 families and 3 individual males, revealed REP-1 mutations in all except 1 male. The age of the males ranged from 5.9 to 63.0 years (mean, 33.9), and VA ranged from hand movements to 1.0 (median, 0.7). Fundus autofluorescence (n = 7) showed defects in the retinal pigment epithelium in all males. Electroretinography (n = 13) was almost undetectable in 6 males and reduced in 6, indicating a rod-cone dystrophy. A further male showed a negative electroretinogram, with a b:a wave ratio of 0.5. Visual acuity of the 8 carriers (age, 4.8-56.8 years [mean, 24.0]) ranged from light perception to 1.2 (median, 1.0). Light perception was present in 1 carrier manifesting choroideremia with distinct chorioretinal atrophy. Pigmentary stippling, seen in the other carriers, was seen in fundus autofluorescence (n = 1) with a distinct speckled pattern. Electroretinograms were normal in 6 of 7 and reduced in the manifesting carrier. Defects in color vision and visual field were found in affected males and in the female carriers. CONCLUSIONS: The phenotype of choroideremia presents with high variability. In addition to the previously reported findings, we observed a negative electroretinogram, indicating a postreceptoral retinal dysfunction, in 1 affected male; severe course of choroideremia with early blindness in 1 manifesting carrier; color vision deficits in the majority of affected males and carriers; and characteristic alterations in fundus autofluorescence.

Adaptor Proteins, Signal Transducing↗

PubMedDate not supplied

Progressive human cone-rod dysfunction (dystrophy).

The author has classified progressive human cone-rod dysfunction into primary and secondary types. The primary type, identified by early ERG cone and usually also rod abnormalities, was further subdivided into types 1 and 2 based on, amoung other distinguishing characteristics, the extent of associated retinal pigment epithelial defects. Secondary cone-rod dysfunction apparently results from disease initially affecting the retinal pigment epithelium. Initially normal ERG findings and the presence of flecks characterize this (type 3) progressive cone-rod dysfunction.

Adolescent↗

PubMed1993

The Farnsworth-Munsell 100 hue test in the first episode of demyelinating optic neuritis.

The Farnsworth-Munsell 100 hue test (F-M 100) was used to examine 30 patients with their first episode of unilateral demyelinating optic neuritis (DON) at presentation, after 6 weeks and after 6 months. Twelve patients satisfactorily completed the test with the affected eye at presentation. This number had increased to 23 by 6 weeks and to 27 by 6 months. No patient with a visual acuity of LogMAR 0.86 (Snellen equivalent approx 6/43) or worse, could complete the test. The mean total error score of affected eyes showed significant improvement at each subsequent examination but was always worse than the non-affected eyes. There was a significant correlation between total error scores and visual acuities of affected eyes at presentation and after 6 months. Fourteen patients recovered a visual acuity of LogMAR 0.0 (Snellen equivalent 6/6) or better but the total error scores of the affected eyes were significantly worse than the non-affected eyes (p = 0.017), indicating that defective colour vision is an indicator of a previous episode of DON despite the recovery of normal visual acuity. DON is reported to produce a red-green (Type II) axis of colour defect but individual F-M 100 polar diagrams were usually generally abnormal and did not show any predominance of recognisable axis of colour defect at any examination. Group averaging of the F-M 100 data from such a well-defined group of patients with acute DON revealed a significant bipolar abnormality in the tritan (blue-yellow) axis at presentation which was not demonstrated at the subsequent examinations or at any examination of the non-affected eyes.

Adult↗

PubMed1993

Colour vision in retinitis pigmentosa. Influence of cystoid macular edema.

In retinitis pigmentosa patients the effect of cystoid macular edema on colour vision was studied. The occurrence of cystoid macular edema decreases with increasing colour vision defect. The mutual proportion of the main types of colour vision defects remains stable until visual acuity has dropped to 0.5; at lower VA levels the number of red-green defects increases. Neither the finding of a blue-yellow colour vision defect in FM100 Hue testing nor the appearance of anomaloscopic pseudoprotanomaly is influenced by cystoid macular edema. The authors conclude that cystoid macular edema in retinitis pigmentosa patients mainly affects visual acuity and not colour vision. They also noted a familial occurrence of cystoid macular edema.

Color Perception↗

PubMed1997

Spectral sensitivity in patients with dysthyroid eye disease.

The majority of patients with dysthyroid eye disease have an acquired colour vision defect. However, no psychophysical investigation of selective damage to colour or flicker pathways has been carried out. In order to clarify the nature of the visual pathology, we have used a psychophysical technique (spectral sensitivity) to selectively stimulate the chromatic and achromatic mechanisms. Spectral spots of size 1 degree presented at a rate of 1 Hz on a bright 1000 td white background are detected by the chromatic mechanism but a rate of 25 Hz reveals the achromatic mechanism. Fifteen patients (28 eyes) between the ages of 50-70 years were tested. The study showed that all patients had reduced spectral sensitivity, either 1 Hz, 25 Hz or both. The patients with reduced 1 Hz or 25 Hz spectral sensitivity only had a shorter systemic and ocular duration of the condition, had no proptosis, normal intraocular pressures in primary gaze, slightly higher intraocular pressures on upgaze, normal visual field plots and FM 100-Hue error scores higher than the normal age-matched values. The patients with reduced both 1 Hz and 25 Hz spectral sensitivities had a longer systemic and ocular duration of the condition, had proptosis, normal intraocular pressures in primary position, higher intraocular pressures on upgaze and higher FM 100-Hue error scores than the age-matched normals and those in Groups 1 and 2. A total of 50% of patients in Group 3 had defective visual field plots. These data suggest that there is a damage of the large achromatic fibres and small chromatic fibres in dysthyroid eye disease. The mechanism of the damage could be one of ischaemic or mechanical or both.

Aged↗

PubMed1986

City tests and information theory.

The responses of 455 male subjects on both editions of the City University Colour Vision Tests (City 1 and City 2) were recorded after the establishment of each subject's criterial status using the Nagel anomaloscope. Based on the Information Theory, each plate was assigned a contributory weighted score to the total test score. Using informational analysis, a cut-off point in test score separating normals and defectives was also determined for each test. This scoring system is an improvement on the existing (or manufacturer's) system. The analysis has also shown that the second edition (City 2) is a considerable improvement on the first (City 1). Despite this, even the improved City 2, like its origin, the D-15, is shown to be poorer than most of the commonly used PIC tests.

Adolescent↗

PubMed1977

The locus of unique green in deuteranomalous trichromats.

A method of color naming was used to determine the spectral locus for unique green (UG) as it was perceived by 20 color normals and 24 deuteranomalous trichromats. The loci for the normal group were distributed bimodally, as earlier investigators had reported, and a bimodal distribution was also found for the deuteranomalous group. In the latter group, UG was located at long wavelengths only by those who had been classified as mild deuteranomals according to several clinical criteria. Those who located UG at shorter wavelengths included individuals whose defects ranged from mild to severe. This result is discussed in the context of theories of deuteranomaly and is presented as further evidence for the existence of 2 types of deuteranomaly.

Adolescent↗

PubMed1987

Spectral characteristics of electroretinography in congenital red-green color blindness.

There are few conclusive electroretinography (ERG) studies comparing the spectral characteristics in deutans and normals in contrast to protans and normals. The difficulties of research on deutans were thought to be due to problems in detecting the very slight differences in the spectral characteristics between deutans and normal subjects. To record monochromatic ERG responses accurately in deutans, our time-locked scanning method was improved as follows: We used 12 interference filters for stimulus lights with narrow half widths (4-6 nm) and wavelengths of peak transmission arranged at intervals of 10 nm between 520 nm and 600 nm. Each stimulus light was strictly adjusted to an equal energy and checked simultaneously with ERG recordings. Contact lens electrodes were reformed for comfortable fitting to subjects' corneas. The time interval between each stimulation was set at 300 msec and one scanning of all stimulations took only 3.9 sec. ERG bp-waves were recorded in congenital color blindness by scanning monochromatic light stimuli, and spectral responses obtained could be evaluated as a spectral pattern. Different spectral patterns of responses from those of normal subjects and shift of the peak in the spectral response curves were obtained for congenital color blind subjects. The maximal responses were recorded at around 540 nm in protans and at 570-580 nm in deutans under white adaptation. Differences in the response curves were not found between dichromats and anomalous trichromats. Moreover, selective chromatic adaptation disclosed the separate responses of green cone and red cone systems. In normal subjects the peak of the spectral response curves was shifted to around 540 nm by red adaptation and to around 580 nm by blue adaptation. The spectral patterns changed so that they looked like the patterns under white adaptation of protans and deutans, respectively. But in protans and deutans the same spectral response patterns and almost the same wavelengths of the peak in the spectral response curves as those obtained under white adaptation were recorded under chromatic adaptation. This method provides the possibility of differentiating between red and green color blind subjects and normal subjects by the ERG. Defects or marked abnormality in the red cone system in protans and the green cone system in deutans can also be detected. Monochromatic ERGs of deutans were recorded under more intense red adaptation.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

PubMed1998

Solar retinopathy. A study from Nepal and from Germany.

319 patients with a solar retinopathy were seen in an eye clinic in Nepal within 20 months. All patients had either a positive history of sun-gazing or typical circumscribed scars in the foveal area. In more than 80% of the patients the visual acuity was 6/12 or better and did not deteriorate over time. 126 (40%) patients had a history of gazing at the sun during an eclipse, 33 (10%) were sun worshipers and 4 (1%) were in both categories. Three years later 29 patients were re-examined in a follow-up study. Only 16 had had visual disturbances directly after they had gazed into the sun. No colour vision defects were seen in any of the 44 affected eyes, when tested with Panel D 15, while four patients (6 eyes) had some uncertainty with the tritan plates of the Ishihara test charts. Metamorphopsia were recorded in 11 eyes. Five German patients with solar retinopathy were examined in more detail. Colour contrast sensitivity (CCS) was tested for the central and the peripheral visual field. CCS for tritan axis was raised in all patients for the central visual field, while it was normal for the peripheral visual field.

Adolescent↗

PubMed2006

The new Richmond HRR pseudoisochromatic test for colour vision is better than the Ishihara test.

AIM: The Hardy-Rand-Rittler (HRR) pseudoisochromatic test for colour vision is highly regarded but has long been out of print. Richmond Products produced a new edition in 2002 that has been re-engineered to rectify shortcomings of the original test. This study is a validation trial of the new test using a larger sample and different criteria of evaluation from those of the previously reported validation study. METHODS: The Richmond HRR test was given to 100 consecutively presenting patients with abnormal colour vision and 50 patients with normal colour vision. Colour vision was diagnosed using the Ishihara test, the Farnsworth D15 test, the Medmont C-100 test and the Type 1 Nagel anomaloscope. RESULTS: The Richmond HRR test has a sensitivity of 1.00 and a specificity of 0.975 when the criterion for failing is two or more errors with the screening plates. Sensitivity and specificity become 0.98 and 1.0, respectively, when the fail criterion is three or more errors. Those with red-green colour vision deficiency were correctly classified as protan or deutan on 86 per cent of occasions, with 11 per cent unclassified and three per cent incorrectly classified. All those graded as having a 'mild' defect by the Richmond HRR test passed the Farnsworth D15 test and had an anomaloscope range of 30 or less. Not all dichromats were classified as 'strong', which was one of the goals of the re-engineering and those graded as 'medium' and 'strong' included dichromats and those who have a mild colour vision deficiency based on the results of the Farnsworth D15 test and the anomaloscope range. CONCLUSIONS: The test is as good as the Ishihara test for detection of the red-green colour vision deficiencies but unlike the Ishihara, also has plates for the detection of the tritan defects. Its classification of protans and deutans is useful but the Medmont C-100 test is better. Those graded as 'mild' by the Richmond HRR test can be regarded as having a mild colour vision defect but a 'medium' or 'strong' grading needs to be interpreted in conjunction with other tests such as the Farnsworth D15 and the anomaloscope. The Richmond HRR test could be the test of choice for clinicians who wish to use a single test for colour vision.

Adolescent↗

PubMed1998

Can clinical colour vision tests be used to predict the results of the Farnsworth lantern test?

Clinicians usually do not have access to a lantern test when making an occupational assessment of the ability of a person with defective colour vision to recognise signal light colours: they must rely on the results of ordinary clinical tests. While all colour vision defectives fail the Holmes Wright Type B lantern test and most fail the Holmes Wright Type A lantern, 35% of colour vision defectives pass the Farnsworth lantern. Can clinical tests predict who will pass and fail the Farnsworth lantern? We find that a pass (less than two or more diametrical crossings) at the Farnsworth Panel D 15 Dichotomous test has a sensitivity of 0.67 and specificity of 0.94 in predicting a pass or fail at the Farnsworth lantern test: a Nagel range of > 10 has a sensitivity of 0.87 and a specificity of 0.57. We conclude that neither the D 15 nor the Nagel Anomaloscope matching range are satisfactory predictors of performance on the Farnsworth Lantern.

Color Perception Tests↗

PubMed2002

An artist with extreme deuteranomaly.

BACKGROUND: There has been speculation about the colour vision of some artists of earlier generations based on the uncertain evidence of how they used colour, but it seems that no major artist has been shown to have a colour vision defect. A few lesser artists are known to have abnormal colour vision and its influence on their painting has been reported in the literature. However, there has been only one report of a deuteranomalous artist and no detailed report of one with extreme deuteranomaly. METHODS: An amateur artist was diagnosed as having extreme deuteranomaly using standard clinical tests. He was interviewed about his difficulty with colour when painting and the strategies he used to counter these problems. His work was studied to determine the colour palette he used and he was set the task of copying another painting to determine the nature of any errors he might make. RESULTS: The subject limits his palette to short-wave blues and blue-greens and long-wave yellow, orange and red. He avoids use of yellow-greens of which he is uncertain. He has adopted a few strategies that help him avoid mistakes in manipulating colour. Despite these difficulties, he is able to create attractive paintings. His early work tended toward monochrome but in his later work he has been able to create warm colourful effects with a limited palette. CONCLUSION: Defective colour vision is a handicap in those artistic activities using colour but it is not an insurmountable barrier. Optometrists should counsel patients with a colour deficiency who are considering a career in the graphic arts about the difficulties they will encounter and the strategies they can use to help minimise those problems.

Aged↗

PubMed1988

Sorsby's pseudoinflammatory macula dystrophy--Sorsby's fundus dystrophies.

The findings are presented on the updated Kempster pedigree with Sorsby's fundus dystrophy. The study confirms the features described in other families: autosomal dominant inheritance with complete penetrance, loss of central vision due to subfoveal ingrowth of new vessels, and progressive peripheral chorioretinal atrophy. By contrast to other reports the family in the current study have peripheral retinal dysfunction, a deposit of a subretinal yellow material throughout the fundus and a tritan colour defect, all prior to the loss of central vision; in some patients there was loss of central vision from atrophic disease, rather than from ingrowth of subretinal new vessels; and, there was a different temporal progression of the central subretinal neovascular complex. These features suggest the possibility of genetic heterogeneity.

Adult↗

PubMed1996

Cavernous hemangioma with cone dysfunction.

We report a 13-year-old male who complained of strabismus and low visual acuity in the right eye. Saccular aneurysms filled with dark-colored blood were noted in the upper nasal quadrant of the right fundus. Ishihara color plates and Lanthony's new color test revealed a red-green color defect. Cone response and 30-Hz flicker responses were nearly absent. Cavernous hemangioma of the retina is a rare vascular hamartoma; the associated findings show cone dysfunction concomitant with this peripheral lesion.

Adolescent↗