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The dyschromatopsia of optic neuritis is determined in part by the foveal/perifoveal distribution of visual field damage
Abstract
Most hypotheses of acquired dyschromatopsia invoke the mechanism of selective damage to specific components of the afferent visual system to explain the predominance of red-green and blue-yellow hue-discrimination defects found in neural and retinal disorders, respectively. However, this pattern of hue-discrimination disturbance in ocular disease may vary. There are frequent exceptions which are inadequately explained by existing hypotheses. In an effort to explain the pattern and pathogenesis of acquired dyschromatopsias better, the authors examined patients with nonproliferative diabetic retinopathy (DR) and late-stage retrobulbar neuritis (RBN) using age-corrected Farnsworth-Munsell 100-hue testing and threshold static perimetry. As expected, most DR eyes showed some degree of relative blue-yellow dyschromatopsia (89%) with few showing a greater weighting towards red-green dyschromatopsia (11%). However, an approximately equal number of RBN eyes had a relative blue-yellow (48%) versus red-green dyschromatopsia (52%). For RBN, the authors found a strong association between the spatial distribution of field defect and the type of relative hue-discrimination disturbance. Eyes with greater field depression at the fovea relative to the perifovea showed a relative preponderance of red-green dyschromatopsia (68%) as opposed to blue-yellow dyschromatopsia (32%), whereas eyes with greater relative perifoveal impairment showed a relative preponderance of blue-yellow dyschromatopsia (100%). This relationship between the relative spatial distribution of visual field damage and the relative hue-discrimination deficit in RBN was statistically significant (P = 0.002). Such an association was not found for DR.(ABSTRACT TRUNCATED AT 250 WORDS)
... Данные о большей уязвимости крупных ГК при глаукоме подразумевают, что психофизические и электрофизиологические тесты для раннего выявления заболевания должны быть направлены на оценку активности ГК М-системы. Действительно, при нормальных параметрах автоматической периметрии, тесты зрительных функций, обслуживаемых М-путями, например чувствительность к высокочастотным мельканиям и движению, бинокулярное зрение, выявляли аномалию у многих пациентов с подозрением на глаукому [30][31][32]. Однако во многих работах отмечается, что зрительные функции, обслуживаемые преимущественно Р-путями, также часто аномальны при начальной ПОУГ. К ним относятся данные цветовой периметрии, различительная цветовая чувствительность. ...
... Типичные изменения цветового зрения у больных с НЗН не соответствуют правилу Кельнера [32,40]. Но имеется сильная корреляция между пространственным распределением области дефекта в поле зрения и типом дисхроматопсии. ...
... В то же время в 100% случаев в глазах с большим ослаблением перифовеальной чувствительности отмечено относительное преобладание сине-желтой дисхроматопсии. Эта корреляция между относительным пространственным распределением дефектов поля зрения и относительным дефицитом цветовосприятия статистически значима для НЗН, но не обнаружена для диабетической ретинопатии [32]. ...
The review discusses literature data on the clinical relevance of functional assessment of magno- (M), parvo- (P), and koniocellular (K) pathways. It also covers the differential contribution of the M, P, and K channels to visual impairments and how it determines the prognosis, early diagnosis, and treatment choice in patients with neurodegenerative diseases of the retina and brain. Selective changes in the performance of the visual channels are investigated by the example of glaucoma and optic neuritis in multiple sclerosis (MS) patients. A detailed analysis of pattern electroretinogram and visual evoked potentials in response to pattern stimuli of varying luminance and chromatic contrast in glaucoma and MS and characteristic functional alterations (objective markers of pathology of the visual pathways) are presented.
... Other studies mostly show both red-green and blue-yellow colour defects with neither type dominating. [25][26][27] Results of one study showed that greater foveal fi eld depression was more likely to be associated with red-green than with blue-yellow defects, but in patients with visual fi elds dominated by perifoveal defects the converse was recorded. 25 Periocular pain, exacerbated by eye movement, is usually mild but present in most patients and usually settles within days. ...
... [25][26][27] Results of one study showed that greater foveal fi eld depression was more likely to be associated with red-green than with blue-yellow defects, but in patients with visual fi elds dominated by perifoveal defects the converse was recorded. 25 Periocular pain, exacerbated by eye movement, is usually mild but present in most patients and usually settles within days. It can precede or begin with the onset of visual dysfunction. ...
Acute optic neuritis is the most common optic neuropathy affecting young adults. Exciting developments have occurred over the past decade in understanding of optic neuritis pathophysiology, and these developments have been translated into treatment trials. In its typical form, optic neuritis presents as an inflammatory demyelinating disorder of the optic nerve, which can be associated with multiple sclerosis. Atypical forms of optic neuritis can occur, either in association with other inflammatory disorders or in isolation. Differential diagnosis includes various optic nerve and retinal disorders. Diagnostic investigations include MRI, visual evoked potentials, and CSF examination. Optical coherence tomography can show retinal axonal loss, which correlates with measures of persistent visual dysfunction. Treatment of typical forms with high-dose corticosteroids shortens the period of acute visual dysfunction but does not affect the final visual outcome. Atypical forms can necessitate prolonged immunosuppressive regimens. Optical coherence tomography and visual evoked potential measures are suitable for detection of neuroaxonal loss and myelin repair after optic neuritis. Clinical trials are underway to identify potential neuroprotective or remyelinating treatments for acutely symptomatic inflammatory demyelinating CNS lesions.
... * Antonio Barreiro-González antoniobarreiro3@gmail.com 1 Ophthalmology Department, University and Polytechnic Hospital La Fe, Avenida Fernando Abril Martorell 106, 46026 Valencia, Spain of imaging biomarkers with useful clinical and research implications [8,9]. Unlike other dimensions of visual function more widespread in clinical and research practice [10], color vision in MS patients has been less handled and the mechanisms involved in their dysfunction are poorly understood [11,12]. ...
To formulate and validate a dyschromatopsia linear regression model in patients with multiple sclerosis (MS). 64 MS patients (50 to formulate the model and 14 for its validation) underwent neurological (Expanded Disability Status Scale, EDSS), color vision (Farnsworth D15 test), and peripapillary retinal nerve fiber layer (pRNFL) and retinal evaluation with spectral-domain optical coherence tomography (SD-OCT). Neuroradiological examination permitted to obtain brain parenchymal fraction (BPF) and cervical spinal cord volume (SC). Ophthalmic parameters were calculated as the average of both non-optic neuritis (ON) eyes, and in case the patient had previous ON, the value of the fellow non-ON eye was taken. The influence of sex, age,
disease duration, and history of disease-modifying treatment (first- or second-line DMT) was tested as covariables that could influence color perception. Color confusion index (log CCI) correlated with pRNFL (r = − 0.322, p = 0.009), ganglion cell layer (GCL, r = − 0.321, p = 0.01), BPF (r = − 0.287, p = 0.021), SC volume (r = − 0.33, p = 0.008), patients’ age (r = 0.417, p = 0.001), disease duration (r = 0.371, p = 0.003), and EDSS (r = 0.44, p = 0.001). The following CCI equation was obtained: log (CCI) = 0.316–0.224 BPF − 0.187 SC volume (mm3) + 0.226 age (years) + 0.012 disease duration (years) − 0.372 GCL (μm). CCI correlates with MS clinical and paraclinical established biomarkers suggesting chronic diffuse neurodegeneration in MS operates at brain, SC, and retina linking all three compartments. Color vision outcome can be calculated through the aforementioned variables for clinical and research purposes.
... In our study, half of patients with temporal RNFL defects had colour vision deficiency. The fovea is abundant in the red-green cone, and the surrounding perifoveal region is abundant in the blue cones 25 . It is supposed that the large proportion of red-green colour vision deficiency relates to the high incidence of central/cecocentral scotoma and decrease of visual acuity. ...
Glaucomatous visual field (VF) damage usually involves in the Bjerrum area, which refers to outside the central 10° region. However, some reports suggest that structural damage to the macula occurs even in the early stages of glaucoma. We investigated the characteristics of normal tension glaucoma (NTG) patients with temporal retinal nerve fibre layer (RNFL) defects. Ninety eyes from 90 subjects including 30 normal eyes, 30 eyes of 30 patients with normal-tension glaucoma with temporal RNFL defects, and 30 eyes of 30 patients with normal-tension glaucoma with inferotemporal or superotemporal RNFL defects were enrolled. The best-corrected visual acuity (BCVA) decreased significantly in glaucomatous eyes with temporal RNFL defects as compared with in controls and glaucomatous eyes with inferotemporal or superotemporal RNFL defects. VF tests showed more frequent central or cecocentral VF defects involving the central 10° region in glaucomatous eyes with temporal RNFL defects. VF defects were more frequently detected on short-wavelength automated perimetry (SWAP). Eyes with temporal RNFL defects had generally reduced ganglion cell-inner plexiform layer (GCIPL) thickness. In addition, the BCVA, GCIPL thicknesses, and SWAP findings were significantly different in glaucoma patients with temporal RNFL defects according to their colour vision deficiency, not RNFL thickness or standard automated perimetry (SAP) results.
... It has been suggested that the defect type in optic neuritis patients is related to the severity and type of spatial defect and fixation location. 32 " 34 We found no association between defect type and spatial vision. One may question whether this lack of difference between groups can be attributed to our relatively lenient criterion for selectivity, which results in mixed (but unequal) defects being classified as selective. ...
To describe the types of color vision defects present in the acute phase of the disease and 6 months into recovery in the 438 participants of the Optic Neuritis Treatment Trial.
Patients meeting strict eligibility criteria were seen within 8 days of the onset of symptoms and then at regular follow-up visits. At the first and 6-month visits (and subsequent annual visits), spatial vision (acuity, contrast sensitivity), visual fields, and color vision were measured. Farnsworth-Munsell 100-hue tests were scored by a variant of the method of quadrant analysis described by Smith et al (Am J Ophthalmol. 1985; 100:176-182).
Most persons show mixed red-green (RG) and blue-yellow (BY) color defects (one type predominating, accompanied by a lesser defect of the other type). BY defects tend to be slightly more common in the acute phase of the disease, with slightly more RG defects at 6 months. Persons may shift defect type over time. Defect type was not related to any of the spatial vision measures at either test time or to treatment group; however, severity of color defect was related to both spatial vision measures and treatment group.
Contrary to common clinical wisdom, optic neuritis is not characterized by selective RG defects. Color defect type cannot be used for differential diagnosis of optic neuritis.
The principal aim of this chapter is to introduce the reader to clinical applications of color assessment and to describe how knowledge of color vision loss is used in clinical practice. The chapter starts with an overview of important aspects of normal, trichromatic color vision. Congenital deficiencies are then reviewed briefly and the factors that cause the large variability in congenital deficiency are discussed. This is then followed by a detailed classification of the retinal, optic nerve and systemic diseases that can cause loss of color vision. The principal color vision tests that are used routinely in clinical practice are then introduced. Emphasis is placed on detection of small changes in color vision that can precede the earliest, clinical signs of a disease, the classification of the patient’s class of color vision (i.e., normal trichromacy, deutan, protan, tritan, or acquired deficiency) and the test’s ability to quantify the severity of color vision loss. Important diseases of the eye and the visual system are reviewed and the associated loss of red/green and yellow/blue chromatic sensitivity discussed and illustrated with emphasis on new findings and future developments. Finally, a typical approach for the use of color vision tests in clinical practice is explained with case reports that illustrate key findings.
People with congenital dyschromatopsias are frequently not aware that their color perception differs from those with normal, trichromatic color vision. Others have learned to adapt to their limited perception of certain colors. Thus, a person with faulty red/green color discrimination will often describe a dark green color as red. Some of these people know that they cannot properly identify colors accurately in certain ranges of hue and luminance. Patients with acquired dyschromatopsias are often unaware of the changes in their color perception, due to the subtle onset and gradual progression of the damage. Not infrequently, this allows major color vision disturbances to go unnoticed. Unilateral dyschromatopsias - if not associated with cataract - should be examined with tests of color saturation in the affected eye, since this is frequently the clinical presentation for an optic neuropathy. Other neuro-ophthalmic disorders (see □ Table 6.1) should initiate a targeted search for an acquired dyschromatopsia.
Die Manifestation einer Neuritis nervi optici im Rahmen der Multiplen Sklerose (MS) ist seit den klassischen Arbeiten des Berliner Augenarztes W. Uhthoff (1890) sehr gut bekannt. In einer Analyse von 1271 Patienten zeigen Wikström et al. (1980), dass eine Optikusneuritis in 34,7% bei klinischer Erstmanifestation der MS und in 16,6% sogar als isoliertes Initialsymptom eruierbar ist. Diese Befunde konnten von weiteren Arbeitsgruppen bestätigt werden (Poser 1984, Paty u. Ebers 1998).
The aim of this review is to summarize the latest information about optic neuritis, its differential diagnosis and management. Optic Neuritis (ON) is defined as inflammation of the optic nerve, which is mostly idiopathic. However it can be associated with variable causes (demyelinating lesions, autoimmune disorders, infectious and inflammatory conditions). Out of these, multiple sclerosis (MS) is the most common cause of demyelinating ON. ON occurs due to inflammatory processes which lead to activation of T-cells that can cross the blood brain barrier and cause hypersensitivity reaction to neuronal structures. For unknown reasons, ON mostly occurs in adult women and people who live in high latitude. The clinical diagnosis of ON consists of the classic triad of visual loss, periocular pain and dyschromatopsia which requires careful ophthalmic, neurologic and systemic examinations to distinguish between typical and atypical ON. ON in neuromyelitis optica (NMO) is initially misdiagnosed as ON in MS or other conditions such as Anterior Ischemic Optic Neuropathy (AION) and Leber's disease. Therefore, differential diagnosis is necessary to make a proper treatment plan. According to Optic Neuritis Treatment Trial (ONTT) the first line of treatment is intravenous methylprednisolone with faster recovery and less chance of recurrence of ON and conversion to MS. However oral prednisolone alone is contraindicated due to increased risk of a second episode. Controlled High-Risk Subjects Avonex(®) Multiple Sclerosis Prevention Study "CHAMPS", Betaferon in Newly Emerging Multiple Sclerosis for Initial Treatment "BENEFIT" and Early Treatment of MS study "ETOMS" have reported that treatment with interferon β-1a,b results in reduced risk of MS and MRI characteristics of ON. Contrast sensitivity, color vision and visual field are the parameters which remain impaired mostly even after good recovery of visual acuity.
This study evaluated the color discrimination of adults who were exposed to long durations of sunlight during their lifetime.
The Farnsworth-Munsell 100-Hue test was used to examine the color vision of 141 subjects (mean age, 46.5 years), who had no
ocular diseases and whose visual acuities ranged from 20/40 to 20/16. Results showed higher total error scores than previously
published norms. There was a tendency for blue-yellow axis, especially in men older than 45 years. Long exposures of ambient
light may cause color vision deficiencies.
Multiple Sclerosis (MS) frequently causes injury to the anterior visual pathway (AVP), impairing quality of life due to visual dysfunction. Development of biomarkers in MS is a high priority and both low-contrast visual acuity (LCVA) and time-domain optical coherence tomography (TD-OCT) have been proposed as candidates for this purpose. We sought to assess whether psychophysical assessments of color vision are similarly correlated with structural measures of AVP injury, and therefore augment measures of visual disability in MS.
We studied the association between high-contrast visual acuity (HCVA), LCVA, color vision (Hardy-Rand-Rittler plates (HRR) and Lanthony D15 tests) and OCT, using both high-resolution spectral-domain OCT (SD-OCT; Spectralis, Heidelberg Engineering, Germany) and TD-OCT (Stratus, Carl Zeiss, US) in a cohort of 213 MS patients (52 with previous optic neuritis) and 47 matched controls in a cross-sectional study.
We found that MS patients have impairments in HCVA and LCVA (p < 0.001) but that they suffer from even more profound abnormalities in color discrimination (p < 0.0001). We found strong correlation between color vision and SD-OCT measures of retinal nerve fiber layer (RNFL) thickness (average RNFL, r = 0.594, p < 0.001) and papillomacular bundle thickness (r = -0.565, p < 0.001). The correlation between OCT scores and functional visual impairments of all types was much stronger for SD-OCT than for TD-OCT.
Our results indicate that color vision is highly correlated with these OCT scores when compared with traditional measures of visual acuity. Also we found that SD-OCT is superior to TD-OCT for detecting anterior visual pathway damage in MS. This makes both color-visual measures and SD-OCT strong candidate biomarkers of disease progression.
Acute optic neuritis due to an inflammatory demyelinating lesion of the optic nerve is often seen in association with multiple sclerosis. Although functional recovery usually follows the acute episode of visual loss, persistent visual deficits are common and are probably due to axonal loss. The mechanisms of axonal loss and early features that predict it are not well defined. We investigated clinical, electrophysiological, and imaging measures at presentation and after 3 months as potential markers of axonal loss following optic neuritis.
We followed 21 patients after their first attack of acute unilateral optic neuritis for up to 18 months. Axonal loss was inferred from optical coherence tomography measures of retinal nerve fiber layer (RNFL) thickness at least 6 months following the episode. Visual function, visual evoked potential, and optic nerve magnetic resonance imaging measures obtained during the acute episode and 3 months later were investigated for their association with later axonal loss.
After multivariate analysis, prolonged visual evoked potential latency and impaired color vision, at baseline and after 3 months, were significantly and independently associated with RNFL thinning. Low-contrast acuity measures exhibited significant univariate associations with RNFL thinning.
The association of RNFL loss with a prolonged visual evoked potential (VEP) latency suggests that acute and persistent demyelination is associated with increased vulnerability of axons. VEP latency and visual function tests that capture optic nerve function, such as color and contrast, may help identify subjects with a higher risk for axonal loss who are thus more suitable for experimental neuroprotection trials.
The diagnosis of optic neuritis (ON) is made clinically based on typical signs and symptoms such as reduced vision and painful eye movements. Very often, ON is part of or associated with a systemic disease, in particular, multiple sclerosis (MS). Differential diagnosis is necessary in untypical cases with bilateral involvement, unusual age at manifestation or associated systemic and/or neurological symptoms. Neurological examination and cerebral MRI are standard in ON work-up. CSF analysis together with MRI scans allows MS risk assessment in ON patients. Further work-up is necessary in suspected systemic autoimmune disorders, sarcoidosis or infectious causes of ON such as borreliosis.
© Georg Thieme Verlag KG Stuttgart · New York.
Zusammenfassung:
Die akute Neuritis nervi optici ist eine klinische Diagnose und typischerweise durch eine vor allem bei Augenbewegung schmerzhafte Sehminderung charakterisiert. Häufig ist sie Ausdruck einer entzündlichen Systemerkrankung, insbesondere der Multiplen Sklerose. Untypische Verläufe wie das beidseitige Auftreten, ein ungewöhnliches Erkrankungsalter oder klinische Präsentation wie auch systemische und neurologische Begleitsymptome erfordern differenzialdiagnostische Überlegungen. Neben der routinemäßigen neurologisch-klinischen Untersuchung hat sich die MRT des Kopfes als Standarddiagnostik etabliert. Die Bildgebung ermöglicht neben der Lumbalpunktion eine Aussage über das Multiple-Sklerose-Risiko. Die Zusatzdiagnostik bei akuter Neuritis nervi optici hat in den letzten Jahren in der Entscheidung über die immunmodulatorische Frühbehandlung des klinisch isolierten Syndroms an Bedeutung gewonnen. Weitere serologische und bildgebende Untersuchungen im Zusammenhang mit einer Neuritis nervi optici haben bei Verdacht auf eine infektiöse Ursache, eine Sarkoidose oder systemische Autoimmunerkrankung ihren differenzialdiagnostischen Stellenwert.
We sought to characterize the dyschromatopsia of optic neuritis, to determine the type and severity of color defect present and its relation to central vision and spatial acuity, to examine changes in this dyschromatopsia over time, and to determine the applicability of Köllner's rule to patients with optic neuritis.
We analyzed the raw data on color vision performance as assembled within the Optic Neuritis Treatment Trial (ONTT). The ONTT was designed to evaluate corticosteroids as a treatment for acute demyelinating optic neuritis and to allow long-term outcome and natural history analyses. Between July 1, 1988 and June 30, 1991, 488 patients were enrolled in this trial. All patients underwent extensive neurologic and ophthalmologic examinations including standardized testing of visual function that included testing of color vision. The ONTT population thus afforded a unique opportunity to characterize acquired dyschromatopsias in a large, homogenous, well-characterized cohort of patients with optic neuritis. We used quantitative analysis of FM-100 scores from this patient cohort to determine the severity of the dyschromatopsia, the selectivity of the dyschromatopsia (polarity of errors) and the type of dyschromatopsia (axis of confusion) by employing quadrant analysis of FM-100 scores.
The results of high-and low-selectivity analyses of the FM-100 data showed that during the acute phase of optic neuritis, blue/yellow, red/ green, and non-selective color defects occurred; among patients with pure defects, blue/yellow defects were more frequent than red/green defects. At 6 months after the acute event, however, analyses showed that red/green defects were more common than blue/yellow defects. Among patients with selective color defects both acutely and at 6 months, the defect was as likely to change over time as remain the same. The likelihood of persistent dyschromatopsia at 6 months was related to the severity of initial central acuity loss, but the type of dyschromatopsia present (red/green versus blue/yellow) was not.
Our results suggest that at the time of the acute attack of optic neuritis, the majority of selective color defects were blue/yellow defects, whereas at 6 months, more of the selective defects were red/green defects, though both types of defects (as well as nonselective defects) were seen acutely and at 6 months. Despite the rigorous inclusion criteria of the ONTT, the large number of patients we studied, correlation of color vision with visual acuity, and longitudinal follow up, this study showed that no single type of color defect was consistently associated with optic neuritis. Demyelinating optic neuritis does not obey Köllner's rule. Moreover, the type of defect present changed in some patients over the course of recovery. Thus, the type of defect may not even be consistent in individual patients as they recover. The type of defect appeared to be related to spatial vision at the time of the test, but the type of defect present at 6 months was not related to the severity of the initial visual loss. Therefore, in evaluating color defects associated with optic neuritis, the level of central visual function must be considered.
First, to study the cellular mechanisms of acquired color vision loss in retinal detachment and diabetic retinopathy. Second, to learn why, in glaucoma, the type of color vision deficit that is observed is more characteristic of a retinal injury than it is of an optic neuropathy. Third, to test a hypothesis of photoreceptor-induced, ganglion cell death in glaucoma.
Various histologic techniques were employed to distinguish the L/M-cones (long/medium wavelength-sensitive cones, or red/green sensitive cones) from the S-cones (short wavelength-sensitive cones, or blue sensitive cones) in humans and monkeys with retinal detachment, humans with diabetic retinopathy, and both humans and monkeys with glaucoma. To test if the photoreceptors were contributing to ganglion cell death, laser photocoagulation was used in a experimental model of glaucoma to focally eliminate the photoreceptors. As a control, optic nerve transection was done following retinal laser photocoagulation in one animal.
Selective and widespread loss of the S-cones was found in retinal detachment as well as diabetic retinopathy. By contrast, in human as well as experimental glaucoma, marked swelling of the L/M-cones was the predominant histopathologic feature. Retinal laser photocoagulation followed by experimental glaucoma resulted in selective protection of ganglion cells overlying the laser spots. This was not seen with retinal laser photocoagulation by optic nerve transection.
In retinal detachment and diabetic retinopathy, acquired tritan-like color vision loss could be caused, or contributed to, by selective loss of the S-cones. Both L- and M-cones are affected in glaucoma, which is also consistent with a tritan-like deficit. Although not a therapeutic option, protection of ganglion cells by retinal laser in experimental glaucoma is consistent with an hypothesis of anterograde, photoreceptor-induced, ganglion cell death.
Most of the histopathological and psychophysical studies in glaucoma reveal a preferential damage to the magnocellular (M) pathway although a few of them support a damage to the parvocellular (P) pathway as well. In glaucoma, the visual fields are usually evaluated by conventional perimetry. However, it has been demonstrated that 20-40% of ganglion cells are lost before field defects are detected using conventional perimetry. Therefore, new psychophysical tests have recently been designed in order to specifically isolate and evaluate the visual mechanisms that are impaired at the early stages of glaucoma. In this context, several authors have addressed the issue of motion perception under the hypothesis of a predominant damage of the M pathway in glaucoma, and that motion perception is mediated mainly by M pathway. The results of these studies depict a large variation in the percentage of patients showing anomalous motion perception. Overall, motion thresholds are elevated in both glaucoma and ocular hypertensive patients as compared to control subjects, irrespective of the stimulus size and eccentricity. The test which discriminates best between patients and normal subjects is motion perimetry. The visual field defects in glaucoma patients identified by conventional perimetry and motion perimetry are similar, but the sizes of the defects are usually larger with motion perimetry. However, motion tests in central vision have no correlation with visual field defect on conventional perimetry. In glaucoma, loss of performance on motion perception tests does not necessarily support the existence of a specific deficit in the M pathway, because some behavioral studies suggest that the P pathway can also mediate motion perception. It is also difficult to conclude that motion perception is specifically affected in glaucoma because most of these studies do not yield a comparison with other visual functions. Despite these difficulties, localized motion perception tests at eccentricities of more than 15 degrees can be considered as a promising diagnostic tool.
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.
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.
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.
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.
To describe visual field parameters at baseline examination of 80 participants in a longitudinal cohort study of birdshot chorioretinopathy and to identify relationships between these parameters and visual acuity, symptoms, clinical findings, and results of laboratory tests.
Single-center cross-sectional study.
Standardized Fastpac, full-threshold Humphrey 30-2 (Carl Zeiss Meditec, Dublin, California, USA) visual field studies were performed for both eyes of all patients. A standardized protocol identified foveal threshold and mean deviation, specified categories of total deviation, and assigned visual field pattern descriptors. These parameters were compared with best-corrected visual acuity (BCVA), symptoms, color confusion score (CCS), cataract, vitreous inflammatory reactions, retinal vasculitis, birdshot lesion characteristics, and ocular coherence tomography (OCT) and fluorescein angiography parameters.
Each visual field parameter was closely related to the others, although mean deviation could be abnormal in the presence of a near normal foveal threshold. Mean deviation was related to BCVA, but the correlation was moderate (the Spearman correlation, -0.55; P < .001). It was also related to CCS and the symptoms of blurry vision, poor contrast sensitivity, and nyctalopia. The most common visual field patterns were multiple foci and arcuate defects. Among clinical and laboratory findings, visual field parameters were most closely related to absence of the third highly reflective band on OCT (P < .001).
Patients with birdshot chorioretinopathy may have a variety of visual field abnormalities, even with normal BCVA. Abnormalities seem to be associated with retinal damage. Automated visual field testing may provide objective measures for monitoring disease activity.
Intravitreal injection of some fluorescent and nonfluorescent tissue-reactive dyes results in selective intracellular staining of a specific population of cones of macaque retina that have been identified tentatively as blue-sensitive cones. This paper describes quantitative density profiles of these cones as a function of retinal eccentricity. These profiles were measured from 0 deg to about 60 deg eccentricity along the nasal and temporal segments of the horizontal meridian of macaque retina. Stained cones were found to be absent from the very center of the fovea. These cones reach peak densities at 0.75-1.50 deg eccentricity, and decrease with greater eccentricity, more rapidly on the temporal than on the nasal segment of the horizontal meridian. Peak densities were found to be slightly closer to the foveal center of the retinas of adult male than of adult female macaques. Packing patterns of stained and unstained cones are discussed as is the mathematic expression of stained cone distribution. The spatial properties of the retinal distribution of stained cones agree very closely with those obtained in psychophysical human studies and other anatomic simian studies of blue-sensitive cones.
Color contrast perimetry was used to study the central visual field defects of patients with acquired dyschromatopsias resulting from diseases of the macula or optic nerve. Patients were classified according to the apparent axis of their hue discrimination defect: (1) predominantly red/green; (2) predominantly blue/yellow; (3) global hue discrimination deficit. A computer controlled color video tangent screen was used to examine the central visual field of all patients. Comparisons were made to automated threshold static perimetry. Patients with hue discrimination defects predominantly in a blue/yellow axis were also found to have relative preservation of both luminance and color contrast sensitivity at the fovea. Conversely, those patients that had hue discrimination defects predominantly in a red/green axis were found to have central visual field defects for both luminance and color contrast that failed to preserve foveal function. It is proposed that the apparent hue discrimination defects in acquired dyschromatopsias are principally the result of the heterogenous spatial distribution of central visual field defects and the physiologic heterogeneity of color opponent mechanisms in the foveal and parafoveal visual field. This would further suggest that hue discrimination defects are usually not produced by selective impairment of opponent red/green or blue/yellow channels in the afferent visual system.
Some three-quarters of a century ago the various forms of acquired colour vision deficiencies had already been well studied by Koenig, von Kries, Koellner, and other German scientists using spectral devices. However, these studies have been greatly neglected, and it is only within the last few years that there has been a renewed interest in this field, of which the first approach has been greatly facilitated in the meantime by the Panel D-15 and the 100-Hue of Farnsworth. Generally speaking, nearly all ocular diseases give rise to absorption systems, while lesions in the deeper layers of the retina elicit a predominant reduction of the blue-yellow sense, and lesions in the ganglion layer and in the optic nerve mainly result in a reduction of the red-green sense.
Visual acuity, color vision, pupillary reaction, induced Pulfrich phenomenon, kinetic fields, static fields, afterimage testing, and ophthalmoscopic evaluation were studied in nine patients with a history of retrobulbar neuritis. The most consistently reliable test for determining the presence of an old optic nerve defect in these patients was meridional 0 to 180 degrees static perimetry. There was a uniform decrease in brightness discrimination to either side of the foveal peak.
In an attempt to elucidate more fully the pathophysiologic basis of early visual dysfunction in patients with diabetes mellitus, color vision (hue discrimination) and spatial resolution (contrast sensitivity) were tested in diabetic patients with little or no retinopathy (n = 57) and age-matched visual normals (n = 35). Some evidence of visual dysfunction was observed in 37.8% of the diabetics with no retinopathy and 60.0% of the diabetics with background retinopathy. Although significant hue discrimination and contrast sensitivity deficits were observed in both groups of diabetic patients, contrast sensitivity was abnormal more frequently than hue discrimination. However, only 5.4% of the diabetics with no retinopathy and 10.0% of the diabetics with background retinopathy exhibited both abnormal hue discrimination and abnormal contrast sensitivity. Contrary to previous reports, blue-yellow (B-Y) and red-green (R-G) hue discrimination deficits were observed with approximately equal frequency. In the diabetic group, contrast sensitivity was reduced at all spatial frequencies tested, but for individual diabetic patients, significant deficits were only evident for the mid-range spatial frequencies. Among diabetic patients, the hue discrimination deficits, but not the contrast sensitivity abnormalities, were correlated with the patients' hemoglobin A1 level. A negative correlation between contrast sensitivity at 6.0 cpd and the duration of diabetes also was observed.
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.
Theories of color vision have been founded on behavioral observations of how the human eye distinguishes colors and mixtures of colors. Studies of congenital dyschromatopsias (inherited disorders of color vision) have been important to the development of these theories. Subsequent studies of acquired dyschromatopsias (disorders of color vision caused by disease) were understandably influenced by these concepts. Theories to explain the patterns of color vision impairment found in acquired diseases (for example, preferential hue discrimination defects) have stressed the likelihood of selective damage to specific components of the afferent visual system (photoreceptors, ganglion cells, synaptic elements, axons etc.). More recent evidence suggests, however, that impairment of color vision by diseases of the retina and optic nerve is commonly nonspecific, and not the result of selective impairment of individual neural mechanisms responsible for mediating color vision. Rather, the patterns of acquired dyschromatopsias often appear to be related to a physiologically heterogeneous distribution of color vision in the foveal and perifoveal visual field, coupled with a tendency for the visual field defects caused by acquired diseases to be unevenly distributed in these same areas.
Four color vision tests were used to assess color vision in 51 insulin-dependent diabetic patients and 41 normal controls. Right and left eyes of diabetic patients, selected because they had minimal retinopathy, had significantly more color vision defects than controls on Lanthony desaturated D-15, Farnsworth-Munsell 100-Hue, and chromagraph tests. The 100-Hue scores were significantly higher in both right and left eyes of diabetic patients than in controls. There were no significant associations between presence or absence of a color vision defect and age, sex, age at onset, duration of diabetes, or its metabolic control.
Contrast sensitivity was measured at nine locations within the central 10° of the visual field in cases of recovered optic neuritis having varying degrees of residual deficit. A sample of 82 patches of visual field was obtained in 14 cases. Circular patches of vertically orientated sinusoidal gratings, 2.5° in diameter, were used. The gratings were modulated in time at 8 Hz and the effect of spatial frequency on the threshold loss determined at each visual field location. As anticipated from what is known of visual field changes in the disorder there was considerable variation in the magnitude of the contrast threshold elevation at different locations in the visual field in any one case. The variability was more marked in case with greater overall deficit.
Three types of spatial loss were encountered. The most common was a loss which increased at higher spatial frequencies, found in 65 of the 82 patches of visual field examined. In 11 the loss was unaffected by spatial frequency and in the remaining 6 the loss was maximal at an intermediate spatial frequency. There was no instance of a loss maximal at low spatial frequencies. Overall the results indicate that sensitivity to higher spatial frequencies is more likely to be impaired following an attack of optic neuritis. In the combined results the effect of spatial frequency on the threshold elevation was statistically significant at all eccentricities (P < 0.001).
Analysis of the combined results revealed no difference in the mean contrast sensitivity loss at eccentricities of zero, 3.75° or 7.5° for intermediate and low spatial frequencies. There is no evidence from these results to suggest that the central foveal projection(papillomacular bundle) is more likely to be affected following an attack of optic neuritis than the projections of other eccentricities within the central 10° as far as mechanisms subserving luminance vision are concerned at these spatial frequencies. Overall there was slightly greater reduction in acuity within the central 5° than at 7.5° eccentricity (P < 0.05). This may be accounted for by the finding that higher spatial frequencies are more affected, rather than being related to eccentricity per se.
Error scores on the Farnsworth-Munsell 100-hue test were partitioned into those representing red-green and those representing blue-yellow losses. Data from two groups of normal observers were used. One group showed results characteristic of published norms; one group showed superior performance. Both observers showed a correlation between red-green and blue-yellow scores indicative of a strong performance factor in this test. The difference between blue-yellow and red-green scores eliminates their correlated variance and allows evaluation of the axis. Both groups showed an increase in difference scores, with age indicating development of a blue-yellow axis. This increase was significant for the observers characteristic of the norms. We suggest cutoff scores to allow a decision as to whether a given patient shows a blue-yellow or red-green axis.
Color contrast perimetry was used to evaluate central visual field defects in a group of 28 patients with visual loss resulting from optic nerve or retinal diseases. Kinetic perimetry was performed using colored test objects of constant luminance, equated to a white surround of 10 ft lamberts. Colored test objects were varied in size and in extent of color saturation. Test object color saturation was varied from a white that matched the color and luminance of the adapting background toward either the blue or the red color maxima of a video tangent screen. All central visual field defects that were demonstrable by luminance contrast perimetry were also detected by color contrast testing, and no defects were found for color contrast detection that could not also be demonstrated by conventional luminance increment perimetry. Retinal diseases usually produced scotomas for both color and luminance contrast detection, while optic nerve disorders tended to produce global depressions of both color and luminance contrast sensitivity across the entire visual field in addition to scotomas. There was no systematic difference in visual field defects for either class of disease when comparing color contrast in the blue (tritan) versus the red (protan) axes of color space. The apparent tritan or protan/deutan axes of color confusion found by hue discrimination testing in acquired dyschromatopsias may be determined by the relative spatial distribution of defects in the central visual field rather than by selective impairment of neural mechanisms for color or luminance information processing.
The results of Farnsworth-Munsell 100-hue, visual acuity, and visual field testing were compared with the severity of retinopathy in a group of 90 diabetic patients. The patients showed significantly higher than expected Farnsworth-Munsell 100-hue scores, with a tritanlike axis, compared with published age norms for nondiabetic individuals. The magnitude of the acquired blue-yellow hue discrimination defect correlated significantly and to a similar extent with both the severity of overall diabetic retinopathy and the severity of macular edema and hard exudate formation. Visual acuity loss correlated somewhat more significantly with macular edema than with overall retinopathy, whereas the converse was true for visual fields. For all visual function tests, the correlations were more significant for fluorescein leakage in the macula than for capillary nonperfusion in the macula. Abnormal hue discrimination was found in 65% (32/49) of eyes with proliferative diabetic retinopathy, suggesting a potential role for this test in screening for proliferative diabetic retinopathy in primary care facilities. Also, because the ability of diabetic patients with color vision deficiency to perform color-dependent tests for urinary and blood glucose may be impaired, such patients should be made aware of this potential problem.
A test procedure is outlined and norms are given for inter-eye comparisons on the 100 Hue test. The norms are applied by taking the square root of the error score in each eye and obtaining the difference between the two square roots. This difference is compared with values given for the 0.05 and 0.01 probability levels. The calculation applies for all age groups and the norms are given for error scores less than 200. The method is particularly recommended in the clinical situation where a possible unilateral acquired dyschromatopsia can be assessed against a matched control.
Threshold static perimetry was performed using test object patterns that covered contiguous areas of the central visual field. Computer imaging methods were used to display a three-dimensional surface that was interpolated between the sensitivity values at each of the test object locations. The examinations covered the area out to and including 10 degrees of eccentricity from the point of fixation, corresponding to the same area of the visual field covered by the Amsler grid. The normal visual field surface appears as a high plateau with a smoothly rising level of sensitivity forming a peak at the point of fixation. It was found that in a variety of macular diseases, including those caused by vascular, as well as primary degenerative disorders, central scotomas were characterized by relative sparing of visual sensitivity at the point of fixation. The pattern thus produced was one of a ring-shaped depression within the central 10 degrees of the visual field. This phenomenon was present in 20% of cases with central scotomas resulting from macular disease, but was not found in any eye of 64 patients suffering from central scotomas as a result of optic nerve disease. This pattern of visual field loss may be common, though not frequently recognized. It is proposed that the phenomenon of preservation of foveal sensitivity may be a marker for macular disease, as distinct from central visual field defects arising from optic nerve disease.
A method for color perimetry is proposed in which colored test objects are presented in a white surround, so that the luminance of the object and its surround are identical. The color of the test object then may be varied in its degree of saturation, while maintaining a constant luminance. A color video instrument controlled by a microcomputer is used as a tangent screen. Foveally viewed, colored test objects are adjusted initially in luminance by heterochromatic flicker photometry to match the luminance of a white background at 100 apostilb. The relative foveal scotoma for blue light requires that test objects large enough to include the perifoveal retina be used for flicker photometry of blue test objects. Due to the progressively increasing threshold for luminance contrast detection in extrafoveal retina, differences in luminance between the colored objects and the white surrounding, as the test objects are moved into the extrafoveal visual field, appear to remain subthreshold. Test object detection can thus be expected to be a perimetric measure of color contrast detection, relatively unaffected by luminance contrast detection. This strategy should simplify the use of colored objects for clinical perimetric testing and should provide a specific test of color vision in the extrafoveal visual field.
A color video tangent screen has been devised, using microcomputer control of a video display to produce colored perimetric test objects matched in luminance to a white surround at 10-foot lamberts . Perimetric isopters for varying degrees of color saturation were determined by kinetic perimetry. This form of color perimetry was used to examine one eye of each of 40 patients with open-angle glaucoma as well as 20 glaucoma-suspect patients. For the first 23 eyes with manifest glaucomatous visual field defects, a masked comparison was made between the results of color perimetry and conventional perimetry with a Goldmann perimeter. For these 23 eyes, color perimetry did as well as luminance perimetry in 14, was less sensitive in 2, and was more sensitive in 7. All defects that were detectable by conventional perimetry were successfully demonstrated by the color method. Such defects often appeared to be greater in extent when mapped by the color method as compared to conventional luminance perimetry.
Thresholds were measured for a tiny, brief, violet flash on a long wavelength, B cone-isolating background in foveal locations spaced only 4 or 5′ of arc apart. Large spatial variations in B cone sensitivity were found just beyond the foveal tritanopic area even though thresholds for the same wavelength test flash hardly varied at all across these same retinal locations when the flash was detected by G cones. The relative constancy of G cone threshold suggests that these spatial variations are intrinsic to the blue-sensitive mechanism and cannot be explained by prereceptoral filtering. The spatial variations in B cone sensitivity are consistent with physiological evidence that B cones are scarce in the retina. In one observer, it was possible to discern discrete peaks in sensitivity spaced roughly 10′ of arc apart. A model is described which takes optical spread and eye movements into account to show that these peaks may represent individual B cones (or clumps of B cones).
First, the relationship between color discrimination, sex, and age is studied by giving the 100-Hue Test to 480 unselected subjects between the ages of 10 and 64. The mean total score of the 20–24-yr group is significantly lower than that of all other groups; the mean partial scores are always highest in the blue–green and red sections of the test and this tendency is accentuated in the age groups with a higher mean total score.
Differences between pcrimctric thresholds for white and equiluminous red. blue and yellow in a nerve fiber bundle defect
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Subsequent Visual Signs In Optic-Neuritis and its Differential Diagnosis
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Pcrkin GD and Rose FC: Subsequent Visual Signs. In Optic-Neuritis and its Differential Diagnosis. Pcrkin GD Ed. Oxford, Oxford University Press, 1979, pp. 204-215.
Basic phenomena of acquired color vision defects Bull Soc Beige Ophtal 215:17 Mullen KT and Plant GT: Anomalies in the appearance of colours and hue discrimination in optic neuritis
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Diabetic rctinopalhy and visual field sensitivity assessed with color pcrimetry. ARVO Abstracts
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Lutze M and Bresnick GH: Diabetic rctinopalhy and visual field sensitivity assessed with color pcrimetry. ARVO Abstracts. Invest Ophlhalmol Vis Sci 33 (Suppl):436, 1989.
Anomalies in the appearance of colours and hue discrimination in optic neuritis
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Mullen KT and Plant GT: Anomalies in the appearance of colours and hue discrimination in optic neuritis. Clin Vision Sci 1:303. 1987
Punctate sen-sitivity of the blue-sensitive mechanism
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Williams DR. MacLeod DIA. and Hayhoc MM: Punctate sen-sitivity of the blue-sensitive mechanism. Vision Res 21:1357. 1981
Diabetic rctinopalhy and visual field sensitivity assessed with color pcrimetry
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Lutze M and Bresnick GH: Diabetic rctinopalhy and visual
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The investigation of acquired colour vision deficiencies
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Die Storungcn des Farbcnsinncs: Ihrc klinischc Bcdcutung und ihrc Diagnose
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