The luminance fall in anomaloscope examination: clinical examples

PURPOSE: The evaluation of the anomaloscope slope quotient in patients with acquired colour vision deficiency. METHODS: Two patients with Stargardt's disease in combination with protanomaly and deuteranomaly, respectively, were selected and also 3 patients with a presumed dominant optic atrophy of the protan type. The anomaloscope examination was performed according to the Linksz procedure. The luminance fall was calculated as the slope quotient SQ:Y units luminance fall per X units width of the matching range. RESULTS: The SQ of the 2 Stargardt patients was steeper than the SQ of congenital colour vision defectives, especially at the red end of the anomaloscope green-red mixture scale, indicating pathologic scotopization superimposed on the congenital deficiency. In optic atrophy of the protan type the SQ was flatter than in congenital deficiency, indicating that this deficiency has nothing to do with congenital protan deficiency. CONCLUSION: Calculation of the slope quotient SQ is helpful for the diagnosis of acquired colour vision deficiency, especially when the subject also has a congenital colour vision deficiency or is supposed to have such a deficiency.     

Pathologic scotopization: A shortened Nagel-II anomaloscopic micro-screw method

The Nagel-II micro-screw method uses eleven colour equations between 620 and 560 nm. The luminance settings are given and are based on the data of colour normal individuals. In the shortened version, intended to detect pathologic scotopization, it is ascertained at which position of the micro-screw the patient's colour adjustments drop beneath the level of 60 scale Units. A total of 64 patients was examined, 29 congenital colour defectives and 35 acquired colour defectives. With the shortened micro-screw method the patients can be divided into four groups: (1) a group without pathologic scotopization, which includes congenital protan defectives; (2) a group in which pathologic scotopization starts; (3) a group with evident pathologic scotopization, due to Stargardt's disease and other cone dystrophies; and (4) a group with complete pathologic scotopization, which includes the congenital achromats and the end-stages of the cone dystrophies.     

Vision evaluation in people with Down's syndrome

We tested the colour vision of 72 people with Down's syndrome using the Ishihara test and an anomaloscope. We found that 13 of the subjects, 6 males and 7 females, had defective colour vision according to Pickford's classification. In monocular vision 10 eyes were protan (five simple, three extreme and two deviant), one eye was simple deuteranomalous and the remaining eyes were normal: in binocular vision four of the subjects were protan (two simple and two deviant), two subjects were deutan (one simple and one deviant) and the rest were normal. Many of our subjects had lens opacities, strabismus, nystagmus, hypermetropia, high myopia and astigmatism, confirming literature reports. The contrast sensitivity function measured with the VCTS test showed a considerable toss of low-frequency sensitivity in our subjects compared to a normal population, which was more marked in the more severely impaired subjects.     

Color vision

Many visual disorders produce acquired color vision defects. Color vision theory emphasizes several stages of visual processing: prereceptoral filters (lens, macular pigment, pupil), cone photopigments (L-, M-, and S-cones), and postreceptoral processes (red-green, S-cone, and luminance channels). Congenital color defects, which affect 8% to 10% of males and 0.4% to 0.5% of females, result from alterations in the photopigment absorption spectra or the absence of one or more photopigments. The most common defects are color vision deficiencies (protan and deutan defects), which are milder than the rarer achromatopsias (complete loss of color vision). Acquired color vision defects can be attributed to a number of different causes: alteration of prereceptoral filters, reduced cone photopigment optical density, greater loss of one cone type than the others, and disruption of postreceptoral processes. Acquired color vision defects have been divided into three classes: type 1, red-green defect with scotopization; type 2, red-green defect without scotopization; and type 3, blue defects (with or without pseudoprotanomaly). Blue defects are usually type 3 acquired defects because congenital tritan defects have an incidence of one in several tens of thousands. Red-green defects can be acquired or congenital, and ruling out acquired defects can require a battery of tests (plates and arrangement tests, anomaloscopy, perhaps genetic analysis). Color vision tests must be administered carefully (with a standard illuminant and protocol), and pupillary miosis or high lens density should be noted and their possible effects considered when interpreting test results. Plate tests provide a simple screening method but do not provide a diagnosis. Arrangement tests and anomaloscope testing take more time and make greater demands on the tester, but they provide a more thorough evaluation. When standard protocols are followed and results are interpreted in terms of prereceptoral filters, photopigment optical density, cone loss, and disruption of postreceptoral processes, a battery of color vision tests can be useful in the differential diagnosis, after progression of the disease, and for evaluating the effectiveness of treatment.     

Influence of pathologic scotopization on the extended Rayleigh Match

Pathologic scotopization, an important symptom of retinal disease, can be studied by means of the Nagel II anomaloscope. This method is called the micro-screw method. The micro-screw method was performed in 14 congenital and 13 acquired colour vision defective individuals. The method proves to be useful in detecting symptoms of rod intrusion in colour vision under photopic conditions.     

Repeatability of the C‐100 colour vision test

Background: The C‐100 colour vision test has been shown to have a high validity for diagnosing the type of red‐green colour vision defect, however, there is little information on the repeatability of the test. This study examines the repeatability of the C‐100 in classifying the colour vision defect as either protan or deutan. Methods: The C‐100 was administered on two occasions to 58 subjects with congenital red‐green colour vision defects: The sessions were separated by a minimum period of 10 days. Results: The repeatability of the C‐100 was high with a kappa coefficient of agreement for diagnosis of 0.96. The few discrepancies were misclassifying protans as deutans. Conclusion: The C‐100 is a highly repeatable test in terms of separating protans from deutans. However, if a discrepancy occurs, it is more likely to be a protan misclassified as a deutan rather than vice versa.     

Visibility of protective helmets worn by forestry workers

Background : High visibility helmets must be worn by Forestry workers in New Zealand for protection and as conspicuous ‘clothing’ to alert workers to the presence and location of other workers. The colours yellow-green (fluorescent yellow-green) and ‘water melon’ (fluorescent pink) are used and both appear to be conspicuous. To solve controversy, we investigated which helmet colour is more visible for use in a forest setting for workers having normal or defective colour vision. Method : We obtained threshold angular sizes for two-millimetre square samples met material presented against a textured background containing colours representative of those found in the foliage and bark of the most common forest type (Pinus Radiata). Observers with normal colour vision (n = 22) and with deutan (n = 8 and protan (n = 6) defects participated. Subjects with mild colour vision defects were excluded. Results : The yellow-green colour was significantly more visible than the pink for the normal (p < 0.001) and protan (p < 0.05) observers. For the deutan observers the pink helmet colour was significantly more visible (p < 0.01). The median equivalent out-door detection distances were for normal observers 400 m (pink) and 500 m yellow-green); for protan observers 185 m (pink) and 500 m (yellow-green); and for deutan observers 550 m (pink) and 450 m (yellow-green). Conclusions : The yellow-green helmet can be detected at large distances by all observers. The yellow-green helmet has greater reflectance and therefore greater luminance contrast. The pink helmet colour can be confused with green forest background colours by observers with protan defects. For some observers with a protan colour vision defect, detection distances for the pink helmet colour are less than half of normal detection distances.     

Impairment of colour contrast sensitivity and neuroretinal dysfunction in patients with symptomatic HIV infection or AIDS.

Ophthalmic and neurological complications are frequent findings in patients with AIDS. Little is known about neuroretinal dysfunction in patients with HIV infection. The purpose of this study was to measure and evaluate colour vision in patients with HIV infection or AIDS. Colour contrast sensitivity tests were performed on 75 patients (150 eyes) in different stages of HIV infection. A highly sensitive computer graphics system was used to measure tritan, deutan, and protan colour contrast thresholds. Patients were classified into three clinical groups: (a) asymptomatic HIV infection, (b) lymphadenopathy syndrome or AIDS-related complex, and (c) AIDS. Overall, tritan (p < 0.0001), deutan (p = 0.003), and protan (p = 0.009) colour contrast sensitivities were significantly impaired in patients with HIV infection compared with normal controls. Colour thresholds in patients with asymptomatic HIV infection (mean tritan threshold: 4.33; deutan: 4.41; protan: 3.97) were not impaired compared with normal controls. Colour vision was slightly impaired in patients with lymphadenopathy syndrome or AIDS-related complex (tritan: 6.25 (p < 0.0001); deutan: 4.99 (p = 0.02); protan: 4.45 (p = 0.05)). In patients with AIDS the impairment was even more marked (tritan: 7.66 (p < 0.0001); deutan: 5.15 (p < 0.0009); protan: 4.63 (p = 0.004)). Analysis of covariance controlling for age demonstrated a close association between impairment of tritan colour contrast sensitivity and progression of HIV disease (p < 0.0001). Following Köllner''s rule, our study suggests that neuroretinal dysfunction occurs in patients with symptomatic HIV infection or AIDS. This is emphasised by the finding that the relative impairment in tritan vision compared with deutan/protan vision might reflect the difference in the number of cones or receptive fields. Measurement of tritan colour contrast sensitivity appears to be an appropriate and easily applicable method to detect early neuroretinal dysfunction in patients with HIV disease.     

Evaluation of an updated HRR color vision test

The HRR pseudoisochromatic plate (pip) test was originally designed as a screening and diagnostic test for color vision deficiencies. The original HRR test is now long out of print. We evaluate here the new 4th edition of the HRR test, produced in 2002 by Richmond Products. The 2002 edition was compared to the original 1955 edition for a group of subjects with normal color vision and a group who had been previously diagnosed as having color vision deficiencies. The color deficient subjects spanned the range of severity among people with red-green deficiencies except for one individual who had a mild congenital tritan deficiency. The new test compared favorably with the original and in at least two areas, outperformed it. Among subjects with deutan defects the classification of severity correlated better with the anomaloscope results than the original; all the subjects who were classified as dichromats on the anomaloscope were rated as "severe" on the new HRR, while those diagnosed as anomalous trichromats were rated as mild or medium on the new test. Among those with moderate and severe defects the new test was highly accurate in correctly categorizing subjects as protan or deutan. In addition, a mild tritan subject made a tritan error on the new test whereas he was misdiagnosed as normal on the original.     

Evaluation of congenital colour vision deficiencies

Three hundred patients who have congenital colour vision deficiencies were examined at the author's eye clinic for 3 years (1987-1990) using 5 types of colour vision tests: Hahn's, TMC's, Okuma's (new), H-R-R's colour vision tests and Double 15 Hue Test (Hahn). The results obtained from each test were quite different in type and grade, and the summarized results were considered to be the best: Type: protan 23.3%, deutan 76.0%, unclassified 0.7% Grade: mild 20.3%, medium 25.3%, strong 54.4% The frequency of coincidence both in type and grade between the summarized results and those of each test were compared, and the highest was 62.3% in Double 15 Hue Test. The efficiency of the author's colour vision test and Double 15 Hue Test were evaluated with the data in this clinical trial, and they were found to be useful for classifying the type and estimating the grade of the congenital and also acquired colour vision deficiencies.     

Clinical use of the City University Test (2nd Edition)

The City University test (TCU test) aims to identify people with significant colour deficiency and to classify the type of defect. 222 people with congenital red-green colour deficiency, diagnosed with the Nagel anomaloscope, were examined with the TCU lest (2nd Edition), All deuteranopes and 44% of deuteranomalous trichromats failed the TCU test. Deutans who failed could be subdivided into two categories of severity depending on whether errors were made on five or more plates. 96% of protanopes and 26% of protanomalous trichromats failed. Protans made fewer errors than deutans and subcategories of severity could not be distinguished according to the number of errors made. The Farnsworth D15 test was found to be more effective than the TCU test in identifying significant protan colour deficiency. Detection and classification rates varied on all the plates of the TCU test. Mixed protan and deutan classification errors were made by 61% of subjects with the majority result correct in 80%. The most efficient plates are identified and recommendations are made for the optimum use of the TCU test in clinical practice.     

Scotopization and the Nagel-II anomaloscope

The term scotopization refers to the intrusion of rod activity in colour vision when assessed under photopic observation conditions. Scotopization is an important symptom of cone dystrophies. The detection of scotopization is not easy. With the Nagel-II anomaloscope scotopization can be detected in two ways. One method is new and this method is described in the present paper.     

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.     

New investigations concerning the relationships between congenital colour vision defects and road traffic security

New extentive experiments demonstrated that: (a) protan observers are more deficient than deutan ones with regard to perception distances of some traffic panels, of vehicle red stop lights, of vehicle red rear-position lights and of white, yellow and red reflectors. Contrarily, deutan observers are more deficient than protan ones for the distinction of differently coloured traffic lights and vehicle rear lights; (b) protan and deutan drivers are nevertheless not responsible for more traffic accidents than drivers with normal colour vision; (c) this apparent contradiction is due to psychological compensation mechanisms. The practical conclusions are: (a) that persons with defective colour vision need not to be excluded from non professional road traffic; (b) that it is nevertheless useful that they should be aware of their handicap; (c) that the red traffic signal has to be larger than the other ones; and (d) that the stop and red position lights of vehicles must be sufficiently intense and that the filters transmitting only pure red should be avoided in them.     

The Nagel anomaloscope in the diagnosis of eye diseases]

The Nagel anomaloscope can be incorporated in the diagnosis of eye diseases. Three parameters are relevant: 1. The measure of the absolute matching range (scale units) 2. The preferred direction of the widened matching range (to red or to green) 3. The luminance matches with the yellow (decreasing matches indicating a pathologic scotopisation). - Six pathologic anomaloscope findings can be differentiated: 1. Pseudoprotanomaly (retinal diseases; type III acquired blue-yellow defects) 2. Symmetrically widened absolute matching range (reduced hue discrimination without reference to its etiopathology) 3. Absolute matching range asymmetrically widened to red with scotopisation (retinal diseases; type III acquired blue-yellow defects or type I acquired red-green defects) or without scotopisation (retinal diseases or optic nerve diseases; type III acquired blue-yellow defects) 4. Absolute matching range asymmetrically widened to green (mostly optic nerve diseases; type II acquired red-green defects) 5. Acceptance of both end matches ("0" up to "73") with scotopisation (retinal diseases) or without scotopisation (optic nerve diseases) 6. Achromatic matches (selective cone diseases, such as Stargardt's dystrophy or progressive cone dystrophy). Indications for anomaloscope examinations and clinical application of the method are discussed in ten cases. The utility of the Rayleigh-equation consists in diagnosing pathologic scotopisation (differential diagnosis between retinal diseases and optic nerve diseases) and in making a quantitative evaluation of the acquired color vision defect (follow-up examination).     

Colour vision defects in asymptomatic carriers of the Leber's hereditary optic neuropathy (LHON) mtDNA 11778 mutation from a large Brazilian LHON pedigree: a case-control study

AIMS: To determine if asymptomatic carriers from a previously identified large pedigree of the Leber's hereditary optic neuropathy (LHON) 11778 mtDNA mutation have colour vision deficits. METHODS: As part of a comprehensive analysis of over 200 members of a large Brazilian LHON pedigree spanning seven generations, colour vision tests were obtained from 91 members. Colour vision was tested one eye at a time using the Farnsworth-Munsell 100 (FM-100) hue colour vision test. The test was administered under uniform conditions, taking into account: ambient light levels, daylight colour temperature of 6700 kelvin, and neutral uniform background. Tests were scored using the FM-100 MS-Excel computer scoring program. Defects were determined and categorised as tritan, deutan, or protan. Categorisation of each dyschromatopsia was based on review of demonstrated axis computer generated plots and age adjusted error scores which coincided with Verriest 95% confidence intervals. Only the axis with the greatest magnitude error score was used to classify the defect. 55 of the 91 test subjects were LHON mtDNA 11778 J haplotype mutation carriers, proved by mtDNA analysis. The remaining 36 subjects were age matched non-blood relatives (off pedigree), who served as controls. RESULTS: 27 of 55 carriers (49.10%) were shown to have colour vision defects in one or both eyes. 13 of the 27 (48%) abnormal tests in the carrier group were tritan defects and the remaining 14 (52%) were deutan defects. Nine of the 27 (33%) abnormals in the carrier group were identified as having bilateral defects. Six of these were deutan, and the remaining three were tritan dyschromatopsias. Only six of the 36 (16.66%) age matched controls were found to have any type of dyschromatopsia. Five (83.3%) of these were deutan defects. The remaining one was a tritan defect. The difference between the two groups using a chi(2) test with one degree of freedom was statistically significant with a p value less that 0.001. CONCLUSIONS: Until now, LHON has always been characterised by a sudden, devastating vision loss. Asymptomatic carriers, those without vision loss, were considered unaffected by the disease. It now appears that asymptomatic carriers of the LHON mutation are affected by colour vision defects and may manifest other subtle, yet chronic, changes.     

Red-green mixture thresholds in congenital and acquired color defects

A color television display was used to measure thresholds for mixtures of red and green on a white background; red and green components could be either incremental, decremental or zero. Ellipses are fitted to a plot of green contrast as a function of red contrast, and it is argued that the length of the ellipse is a measure of red-green color discrimination and the width of the ellipse is a measure of luminance discrimination. It is shown that the technique reliably distinguishes normals from congenital color defectives and also protan from deutan subjects. For some cases of acquired color defects (e.g. optic neuritis), there is a roughly equal loss of color and luminance discrimination whereas, in other cases (e.g., hereditary optic atrophies), the loss of color discrimination is much greater than the loss of luminance discrimination.     

Anomaloscope examination in macular gliosis, macular holes and central serous choroidopathy

· Background: Surgery for macular gliosis and macular holes has become increasingly successful with regard to anatomical outcome. Assessment of the damage to the receptors by these processes is still difficult, but is important in predicting functional outcome. · Methods: Examination with the Nagel II or the Neitz OT anomaloscope was performed in 36 patients with macular gliosis, 23 patients with full-thickness macular holes and 47 patients with central serous choroidopathy. The anomaloscope matches were expressed as the quotient of anomaly. · Results: In macular gliosis the mid-matching point is usually 1.0; there is no pseudoprotanomaly. In macular holes the mid-matching point is 1.0 when visual acuity is 0.3 or greater; in eyes with lower visual acuity there may be signs of diminished red sensitivity, but anomaloscope examination becomes difficult. In central serous choroidopathy the mid-matching point is shifted towards red, and pseudoprotanomaly is present, even when visual acuity is normal. · Conclusions: Diseases of the inner retina, in early stages, do not alter colour vision substantially, whereas diseases of the outer retina give rise to early colour vision deficiency. In macular gliosis and macular holes, anomaloscope examination enables estimation of macular receptor misalignment. Received: 16 December 1996 Revised version received: 1 April 1997 Accepted: 1 October 1997     

Orienteers with poor colour vision require more than cunning running

Background: Highly detailed colour coded maps are used in the sport of orienteering to enable competitors to navigate from one check point to another and to provide guidance on the nature of the terrain to be traversed. The colours are defined by the International Orienteering Foundation (IOF) and are said to have been chosen so they will not be confused by competitors who have abnormal colour vision. However, there are anecdotal reports that individuals with colour vision defects do have problems with the colour coding. Method: A Minolta Spectrophotometer CM‐503i was used to measure the CIE x,y chromaticity co‐ordinates and the reflectances of the standard colours recommended by the IOF for the colour coding of orienteering maps, as well as the colours on two maps used in orienteering events. Results: Four pairs of IOF standard colours are likely to be confused by protan observers and four pairs by deutan observers. There were three pairs of colours likely to be confused by both deutan and protan observers on one of the competition maps and one pair likely to be confused by protan observers on the other map. Some of the colours on the actual competition maps differed noticeably from the standard IOF colours. Discussion: Orienteers with more severe forms of abnormal colour vision are likely to be disadvantaged by their inability to differentiate some colours used on orienteering maps. The IOF should choose different colours that are less likely to be confused or should employ a redundant code (such as a pattern or texture). There is need for better quality control of the colours of competition maps to ensure they do conform to the IOF standard colours.     

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.