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PubMed1977

Involutional diabetic retinopathy.

The end-stage or involutional phase of proliferative diabetic retinopathy may result in stabilization of vision for long periods of time. However, the clinical resemblance to the progressive tapetoretinal degenerations suggests that marked functional impairment of the retina is present in such eyes. We studied 19 eyes with involutional retinopathy to document the status of the retinal function. Studies included fluorescein angiography, visual field examination, dark adaptation testing, color vision testing, electro-oculography and electroretinography (ERG). The results indicated marked functional abnormalities in all eyes. The ERG tracings showed uniformly subnormal responses and delayed implicit times, similar to those of dominantly inherited retinal pigment degeneration, and indicative of a progressive retinal disorder. In two patients, color vision testing showed defects similar to those seen in inherited tritanopia; and in the remaining patients, defects were indicative of an acquired blue-yellow dyschomatopsia.

Adaptation, Ocular↗
PubMedDate not supplied

Prevalence of congenital color deficiencies in secondary-school students in Tehran.

PURPOSE: A population-based study was conducted to determine the prevalence of color deficiencies in secondary-school students (ages 12-14) in Tehran. METHODS: A total of 2,058 students (1,136 males, 922 females) were examined with Ishihara pseudoisochromatic color plates. RESULTS: In the study population, 97 cases of defective color vision were detected, including 93 males and 4 females. The affected individuals all had negative histories of previous systemic and ocular disease or chronic use of medications. The visual acuity was 20/20 and the fundus was normal in all affected students. Of the 93 cases of defective color vision in males (8.18%), 56 cases (4.93%) involved deuteranomaly, 13 (1.14%) protanomaly, 13 (1.14%) deuteranopia, and 11 (0.97%) protanopia. The four cases in females (0.43%) involved deuteranomaly in three cases (0.32%) and protanomaly in 1 case (0.11%). Deuteranopia and protanopia were not detected in females. CONCLUSION: This is the first study to determine the prevalence of congenital color blindness in Iran. The results agree with reports of prevalence of congenital color blindness from Western Europe.

Adolescent↗
PubMed2002

Cross-sectional study of the ophthalmological effects of carbon disulfide in Chinese viscose workers.

This article presents cross-sectional investigation results of ophthalmological effects for the occupational exposure to carbon disulfide of workers at a large viscose fibre factory in the middle part of China. The total of 271 exposed subjects (191 males, 80 females) and 133 workers (93 males, 40 females) not exposed to any toxic agent in the working environment underwent ophthalmological examination. The self-administered questionnaire collected data on the medical history and ophthalmological complaints during the past three months. The ophthalmologic examination included routine examination for retinal capillary anomalies and and color vision with the FM 100-Hue test method. Nearly all subjects did not use respirators, smocks or aprons, gloves or other personal protective devices during work time. The average personal CS2 exposure level in the present study was 13.7-20.05 mg/m3. The FM 100-Hue test results showed that the total error scores of the exposed group, whether male or female, were higher than that of the control, the discrimination of the green and blue zones was also impaired significantly. A fundus examination showed no retinal capillary anomalies or other serious ophthalmological symptoms that may be related to effects of CS2. In conclusion, color vision was disturbed in workers exposed to CS2, at levels below the present threshold value. Reduced color discrimination may be attributed to long-term carbon disulfide exposure and suggests that health surveillance of workers exposed to carbon disulfide should include the FM 100-Hue Test as a sensitive and easy method.

Adult↗
PubMed2001

Visual function is stable in patients who continue long-term vigabatrin therapy: implications for clinical decision making.

PURPOSE: Vigabatrin (VGB) has been shown to cause visual field constriction and other forms of mild visual dysfunction. We determined the safety of continuing VGB therapy in patients who had received prolonged treatment (>2 years) with the drug by serially monitoring changes in visual function over a 1-year period of continued therapy. We also followed up patients who discontinued VGB to see whether alternative therapies are effective. METHODS: Fifteen of 17 patients who continued VGB therapy had visual-function testing (visual acuity, color vision, kinetic and static perimetry) every 3 months for 1 year. Eighteen patients who discontinued VGB were given alternative antiepileptic drugs (AEDs); their seizure responses were measured after > or =3 months of treatment. RESULTS: Patients continuing VGB showed no worsening of visual acuity, color vision, or visual-field constriction beyond that measured in the initial test. Many patients who discontinued VGB had good seizure control with either newer or previously unsuccessful AEDs. CONCLUSIONS: For patients who have an excellent response to VGB and only mild visual changes, continued therapy may be safe with close visual monitoring. Patients who do not have a significant reduction in seizures or who experience considerable visual dysfunction with VGB may respond well to alternative therapies.

Adult↗
PubMed1997

Retinal function abnormalities in membranoproliferative glomerulonephritis type II.

PURPOSE: To characterize retinal electrophysiologic and psychophysical abnormalities associated with membranoproliferative glomerulonephritis type II. METHODS: Three adults with membranoproliferative glomerulonephritis type II were studied. Retinal function was measured psychophysically (automated perimetry, Farnsworth D-15 color vision testing, and dark adaptometry) and electrophysiologically (full-field flash electroretinography and electro-oculography). RESULTS: Two symptomatic individuals had prominent drusenlike deposits and retinal pigment epithelial disturbances, findings characteristic of membranoproliferative glomerulonephritis type II retinopathy. These individuals had mild visual field and color vision abnormalities, prolonged dark adaptation, and delayed electroretinographic dark-adapted dim and bright flash responses of normal amplitude. The electro-oculogram of the most severely affected individual was abnormal. The third individual who was asymptomatic had the mildest fundus abnormalities and exhibited normal psychophysical and electrophysiologic responses. CONCLUSIONS: Membranoproliferative glomerulonephritis type II retinopathy may be associated with symptomatic and measurable psychophysical and electrophysiologic abnormalities of retinal function.

Adult↗
PubMed1989

Autosomal dominantly inherited macular dystrophy with preferential short-wavelength sensitive cone involvement.

We found an apparently inherited tritan-like color vision defect in five members of a family, spanning three generations. The defect was associated with mild macular pigmentary changes, poor foveolar reflexes, or slightly reduced visual acuity in four of the affected individuals. The inheritance pattern appeared to be autosomal dominant. Results of various color vision tests indicated preferential involvement of the short-wavelength sensitive cone system, with relative preservation of the middle- and long-wavelength sensitive cone systems. Both anomaloscope testing with larger (8-degree) fields and short-wavelength sensitive electroretinography indicated some short-wavelength sensitive cone system involvement beyond the central macula in the three affected individuals on whom testing was performed. The condition appeared to be a familial macular dystrophy with preferential short-wavelength sensitive cone involvement. The abnormal macular findings and mild reduction in visual acuity distinguish this condition from congenital tritanopia; the normal optic disks distinguish it from autosomal dominant optic atrophy.

Adolescent↗
PubMed1980

The 1976 accident experience of civilian pilots with static physical defects.

The 1974 and 1975 aircraft accident experiences of civilian pilots with eight selected static physical defects have been examined and reorted previously. Three categories--blindness or absence of either eye, deficient color vision with a waiver, and deficient distant vision--had significantly more accidents than were expected on the basis of observed-to-expected ratios. In 1975, accident rates were calculated. The rates for air men with blindness or absence of an eye were still found to be significantly higher. Observed-to-expected ratios for 1976 were 1.91 for deficient color vision with a waiver, 1.28 for contact lens users, 1.37 for blindness or absence of either eye, and 1.62 for deficient distant vision. The accident rates per 100,000 h of cumulative and last 6 months' flying experience were significantly greater for contact lens users and monocular pilots than for the active airman population. The other groups had no consistently significant differences.

Accidents, Aviation↗
PubMed2005

Poor prognosis of visual outcome after visual loss from giant cell arteritis.

OBJECTIVE: To evaluate the incidence and extent of visual recovery in patients with giant cell arteritis (GCA) treated with high doses of systemic corticosteroids. DESIGN: Multicenter prospective case series. PARTICIPANTS: Thirty-four consecutive patients with biopsy-proven GCA and visual loss seen at either Wills Eye Hospital in Philadelphia or Auckland Hospital in New Zealand from 2001 to 2004 were evaluated prospectively. METHODS: All patients underwent a complete neuro-ophthalmic evaluation that included Snellen visual acuity, pseudoisochromatic Ishihara color plates, visual fields, intraocular pressure, slit-lamp examination, and binocular ophthalmoscopy. Formal visual field testing was performed on all patients who were capable of completing the test. All patients were treated with a standard protocol of 1 g of IV methylprednisolone daily for 3 days followed by oral prednisone 60 or 80 mg (depending on patient weight). Patients were evaluated initially at 2-week intervals (14-19 days) and then monthly (4-5 weeks), with subsequent dosages of prednisone modified based on erythrocyte sedimentation rate, C-reactive protein, and patient symptoms. At each visit, patients underwent a repeat complete neuro-ophthalmic evaluation. MAIN OUTCOME MEASURES: Alterations in visual acuity, visual field, and color vision. RESULTS: Patients with visual loss from GCA had a mean visual acuity of 20/400. Visual deterioration occurred in 27% of eyes within the first week despite high-dose IV corticosteroids. Fifteen percent of eyes showed an improvement of visual acuity within the first month, but only 5% have corresponding improvement in visual field. CONCLUSIONS: Visual recovery is uncommon in patients who lose vision from GCA. Recovery in visual acuity is not associated with visual field or color vision improvement in this series. Visual deterioration occurs in approximately 27% of eyes despite high-dose IV methylprednisolone. The greatest risk of visual deterioration is in the first 6 days.

Administration, Oral↗
PubMed2001

Disc excavation in dominant optic atrophy: differentiation from normal tension glaucoma.

OBJECTIVE: In patients with dominant optic atrophy (DOA, Kjer type), excavation of the optic nerve develops, and these patients may be misdiagnosed as having normal tension glaucoma (NTG). This study examined disc morphologic features in patients with DOA and explored features that help distinguish this condition from NTG. DESIGN: Noncomparative, observational case series. PARTICIPANTS: Patients with DOA who were seen at the Duke University Eye Center between 1987 and 1996 and who had bilateral optic nerve photographs. METHODS: Retrospective chart review of the results of visual acuity testing, visual field testing by Goldmann perimetry, color vision testing, intraocular pressure measurement, and observation of bilateral optic nerve photographs. MAIN OUTCOME MEASURES: Appearance of the optic disc and peripapillary zone in patients with DOA. RESULTS: Nine patients were identified. The mean age at the time of evaluation was 28 years (range, 11-62 years). Most patients had a mild to moderate reduction in visual acuity. Color vision as tested with Hardy-Rand-Rittler plates was reduced (4.0/10 +/- 4.2/10). A cup-to-disc ratio of more than 0.5 was observed in at least one eye of eight patients. A temporal wedge-shaped area of excavation was observed in 14 of the 18 eyes studied. Moderate to severe temporal pallor was observed in all of the eyes. Pallor of the remaining (noncupped) neuroretinal rim was also observed consistently, ranging from mild to moderate. A gray crescent and some degree of peripapillary atrophy were noted in all eyes. CONCLUSIONS: Several clinical features, including early age of onset, preferential loss of central vision, sparing of the peripheral fields, pallor of the remaining neuroretinal rim, and a family history of unexplained visual loss or optic atrophy, help to distinguish patients with DOA from those with NTG.

Adolescent↗
PubMed2003

Transcaruncular orbital decompression for management of compressive optic neuropathy in thyroid-related orbitopathy.

This study was conducted to assess the outcome of transcaruncular orbital decompression to treat compressive optic neuropathy in thyroid-related orbitopathy. It involved a retrospective, noncomparative case series of 18 eyes of 10 consecutive patients with documented vision loss secondary to thyroid-related orbitopathy. Bony decompression of the orbital apex was performed via a transcaruncular approach. Main outcome measures were visual acuity, color vision, presence of diplopia, and reduction of exophthalmos. Of 18 eyes, 16 (89 percent) had improved visual acuity after the operation. One eye had no improvement and one had worsening of vision in the setting of diabetic retinopathy. Color vision was improved in 12 eyes (67 percent). Five of the patients did not have diplopia before the operation; none of these patients developed double vision after intervention. Exophthalmos was decreased by an average of 2.6 mm. The authors conclude that transcaruncular orbital decompression for compressive optic neuropathy in thyroid-related orbitopathy is successful in restoring visual function. Compared with other approaches used for decompression surgery, the transcaruncular approach offers direct access to the medial wall and orbital apex without a cutaneous incision or disruption of the medial canthus. In addition, this approach allows a controlled, graded removal of the ethmoidal air cells and reduced recovery time.

Adult↗
PubMed1982

A new assessment of the normal ranges of the Farnsworth-Munsell 100-hue test scores.

We gave the Farnsworth-Munsell 100-hue color vision test to 232 normal subjects between 10 and 80 years of age. One half the subjects underwent binocular testing followed by monocular testing. In the other half monocular testing preceded binocular testing. Performance was better with both eyes than with either eye alone. The worst performance occurred on monocular tests in subjects without previous experience with the task (that is, those for whom this was the first test). The well-known age trend was apparent (children and elderly have the worst color vision). New data are provided for judging the point at which the total error score may be considered pathologic.

Adolescent↗
PubMed2006

Quantitative analysis of OCT characteristics in patients with achromatopsia and blue-cone monochromatism.

PURPOSE: To quantify optical coherence tomography (OCT) images of the central retina in patients with blue-cone monochromatism (BCM) and achromatopsia (ACH) compared with healthy control individuals. METHODS: The study included 15 patients with ACH, 6 with BCM, and 20 control subjects. Diagnosis of BCM and ACH was established by visual acuity testing, morphologic examination, color vision testing, and Ganzfeld ERG recording. OCT images were acquired with the Stratus OCT 3 (Carl Zeiss Meditec AG, Oberkochen, Germany). Foveal OCT images were analyzed by calculating longitudinal reflectivity profiles (LRPs) from scan lines. Profiles were analyzed quantitatively to determine foveal thickness and distances between reflectivity layers. RESULTS: Patients with ACH and BCM had a mean visual acuity of 20/200 and 20/60, respectively. Color vision testing results were characteristic of the diseases. The LRPs of control subjects yielded four peaks (P1-P4), presumably representing the RPE (P1), the ovoid region of the photoreceptors (P2), the external limiting membrane (ELM) (P3), and the internal limiting membrane (P4). In patients with ACH, P2 was absent, but foveal thickness (P1-P4) did not differ significantly from that in the control subjects (187 +/- 20 vs. 192 +/- 14 microm, respectively). The distance from P1 to P3 did not differ significantly (78 +/- 10 vs. 82 +/- 5 microm) between ACH and controls subjects. In patients with BCM, P3 was lacking, and P2 advanced toward P1 compared with the control subjects (32 +/- 6 vs. 48 +/- 4 microm). Foveal thickness (153 +/- 16 microm) was significantly reduced compared with that in control subjects and patients with ACH. CONCLUSIONS: Quantitative OCT image analysis reveals distinct patterns for controls subjects and patients with ACH and BCM, respectively. Quantitative analysis of OCT imaging can be useful in differentiating retinal diseases affecting photoreceptors. Foveal thickness is similar in both normal subjects and patients with ACH but is decreased in patients with BCM.

Adult↗
PubMed1992

Cone dystrophies: clinical and electrophysiological findings.

We analyzed the clinical and electrophysiological findings of 77 patients suffering from progressive cone or cone-rod dystrophies. The onset of symptoms was at the average age of 19.7 +/- 19.4 years. In some patients, the disease started within the 5th decade. The mean visual acuity was 0.19 +/- 0.2, while in 38%, the visual acuity was lower than 0.1. Color vision defects and visual field defects were found in most patients. The electrooculogram was recorded in 59 patients and was normal in only 19. On the electroretinogram (ERG), 60 patients had a reduction of the 30-Hz flicker amplitude and of the responses at maximum stimulus intensity when dark and light adapted. The ERG alterations showed a correlation to the visual field defects and to the reduction of the light rise on the electrooculogram. No correlation existed between the ERG amplitudes and visual acuity or color vision. Ophthalmoscopically, the posterior pole was normal in 25 patients. In the remaining patients, fundus changes ranged from mild pigment irregularities to severe pigment clumping. No correlation between fundus changes and functional findings existed.

Adolescent↗
PubMed2007

The use of tinted contact lenses in the management of achromatopsia.

BACKGROUND: Achromatopsia is a congenital, autosomal recessively inherited condition in which cones are either defective or absent. Complete achromatopsia results from having only rods as functioning photoreceptors. Many people with achromatopsia have small amounts of residual cone function that may provide minimal color vision under special circumstances. Clinical findings associated with the condition include reduced visual acuity, nystagmus, a greater than normal incidence of high ametropia, and severe photophobia. The photophobia resulting from achromatopsia can be debilitating even in normal indoor illumination. Tinted contact lenses have been reported to reduce photophobia and improve visual function in these patients. CASES: Two cases are reported here. A 32-year-old man presented with reduced and stable visual acuity, complete color blindness, nystagmus, and debilitating photophobia. The second patient was a 23-year-old woman who presented with reduced and stable visual acuity, severely impaired color vision, rotary nystagmus, and significant photophobia. Both of these patients were fit with centrally tinted contact lenses. Although visual acuity did not improve measurably in either patient with tinted compared with clear lenses, both experienced a dramatic reduction in photophobia with the lenses. CONCLUSION: Tinted spectacle or contact lenses may be useful in relieving photophobia associated with a number of cone disorders, including achromatopsia. In addition to decreasing light sensitivity, tinted lenses have been reported to improve visual acuity, decrease the size of central scotomata, enlarge peripheral visual field, and enhance visibility of long wavelength stimuli in bright illumination.

Adult↗
PubMed2000

[The clinical application of different brightness and different saturation D-15 tests].

PURPOSE: To compare the clinical application of different brightness and different saturation D-15 tests. METHODS: Eighteen normal subjects (30 eyes), 19 cases (38 eyes) of congenital color vision defects and 36 cases (59 eyes) of eye diseases were tested with Panel D-15 test, Hann's double D-15 test, middle and low saturation CAS-PI (Psychological Institute, Chinese Academy of Sciences) D-15 tests. RESULTS: A few of the normal eyes made minor errors in the low saturation D-15 tests. All of the protanopes and deuteranopes could be detected correctly. Protanomalias and deuteranomalias showed normal arrange or some type of abnormal arranges in all of the 5 sets of D-15 tests. In sick eyes, the abnormal rates were the highest in the low saturation D-15 tests and the lowest in Panel D-15 test. CONCLUSION: The five sets of different brightness and different saturation D-15 tests had similar efficiencies of detecting congenital color vision defects. The abnormal rates of CAS-PI(4/5)D-15 tests and the CAS-PI(2/5)D-15 tests were similar to those of Panel D-15 test and Hann's Double D-15 test. Both tests can be used in the clinical setting.

Adolescent↗
PubMed2004

Classification of Farnsworth-Munsell 100-hue test results in the early treatment diabetic retinopathy study.

PURPOSE: To classify and describe clinically meaningful classes of color vision defects using pretreatment Farnsworth-Munsell 100-hue results from the Early Treatment Diabetic Retinopathy Study (ETDRS) patients using standard statistical techniques. DESIGN: The ETDRS was a randomized trial investigating retinal photocoagulation and oral aspirin in diabetic retinopathy. METHODS: Farnsworth-Munsell (FM) 100-hue test was successfully administered before initiation of study treatment in each eye of 2701 of the 3711 ETDRS patients. Test results were converted into a Fourier series, classified by cluster analysis in the deferred-treatment group of eyes, and verified in the immediate-treatment group of eyes as separate samples. RESULTS: Cluster analysis uncovered thirteen distinct patterns. Pattern A (51% or 1366 of the eyes) showed unimpaired hue discrimination and was comprised of younger patients with no or little macular edema. Pattern B eyes (10% or 262) showed generalized impairment of hue discrimination with no main axis defect. Patterns C (C1, C2, C3), comprising 26% (or 698) of the eyes, showed increasing severity of the yellow-blue diabetic retinopathy defect, associated with increasing mean age and increasing macular edema severity. Patterns D (D1, D2), comprising 6% (or 164) of the eyes, were similar to the C patterns but showed a stronger yellow-blue defect. Patterns E (E1, E2, E3), or approximately 2% (or 38) of the eyes and predominantly male, exhibited the expected pattern for congenital protan defect. Patterns F, G, and H, approximately 6% (or 153) of the eyes, showed distinct patterns of one-sided axes. The nomenclature is arbitrary. CONCLUSIONS: Cluster analysis of FM 100-hue test results has found 13 patterns of impaired hue discrimination, helpful in understanding color vision defects in diabetes mellitus.

Adult↗
PubMed2006

Novel mutations in the OPA1 gene and associated clinical features in Japanese patients with optic atrophy.

PURPOSE: Autosomal dominant optic atrophy (ADOA) is characterized by symmetrical bilateral optic atrophy associated with reduced corrected visual acuity (VA), central or centrocecal scotoma, and color vision disturbances. The disease is genetically heterogeneous, and the OPA1 gene has been identified as the only causative gene. The aims of this study were to identify and report mutations in the OPA1 gene in Japanese patients with ADOA and to describe the clinical features associated with the mutations. DESIGN: Molecular genetic study and observational case reports. PARTICIPANTS: Nine unrelated Japanese families with optic atrophy and 8 isolated cases of optic atrophy. METHODS: Genomic DNA was extracted from peripheral leukocytes, and all exons containing the open reading frame of the OPA1 gene and the flanking intron splice sites were sequenced directly. Complete ophthalmologic examinations were performed. MAIN OUTCOME MEASURES: Direct sequencing of the OPA1 gene and clinical evaluations including VA, visual field, color vision, and disc appearance. RESULTS: Ten different heterozygous mutations, including 6 novel mutations, were detected in the OPA1 gene. The identified mutations included 5 deletions/insertions (c.2061delA, c.2098_2103delCTTAAA, c.2538insT, c.2591insC, and c.2708_2711delTTAG), 4 nonsense mutations (c.112C>T [p.R38X], c.181C>T [p.Q61X], c.946A>T [p.R316X], and c.2713C>T [p.R905X]), and 1 missense mutation (c.1635C>A [p.S545R]). The most common mutation in Caucasians (c.2708_2711delTTAG) was found in 3 unrelated families, suggesting that it is a mutational hot spot. We detected an OPA1 mutation in 8 of 9 familial cases of optic atrophy and in 4 of 8 cases that were initially considered to be sporadic from the patients' family histories. Examinations of family members of 2 sporadic probands revealed the existence of other family members with the OPA1 mutations whose phenotype was very mild or within normal limits. This indicates that patients with ADOA sometimes seem to be sporadic because of the extensive variation in the phenotype or, alternatively, a low penetrance of ADOA. CONCLUSIONS: OPA1 gene mutations are causative in most familial cases of ADOA in Japanese. Sporadic cases of optic atrophy frequently may be caused by OPA1 mutations in the Japanese population. Molecular genetic examinations are useful in determining the hereditary patterns in some cases of optic atrophy.

Adult↗