Vitreous fluorophotometry in patients with fundus flavimaculatus.
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Adult↗
Biomedical subjects
G A Fishman
Publications and source records attributed to G A Fishman.
Sensitivity losses in a long wavelength sensitive mechanism of patients with retinitis pigmentosa.
the foveal vision of certain patients afflicted with retinitis pigmentosa may be mediated solely by a long wavelength sensitivity (lws) mechanism, because (1) increment threshold data could always be fitted to a single template curve with unity slope, (2) test and field sensitivities differ only by a proportionality constant over the visible spectrum, (3) intense background fields have additive (quantum catch) effects on thresholds, and (4) wavelength differences of bipartite foveal stimuli could not be distinguished. Both test and field sensitivities were appreciably lower than the lws sensitivity values for unaffected deuteranopes. The action spectrum was similar (but not identical) to estimates of the erythrolabe absorption spectrum. The results provide a better understanding of the photopic vision of patients with retinitis pigmentosa and further evidence that their effective optical density of residual cones is reduced.
Adult↗
Refractive errors in patients with fundus flavimaculatus.
A total of 59 patients (118 eyes) with fundus flavimaculatus who had atrophic macular lesions were examined for refractive error. Myopic refractions of greater than -0.1 dioptre were seen in 12% of a normal population and in 76% of our patient population. The spherical equivalent refractive errors described a single-peaked skewed distribution with a mean of -1.12 dioptres, which is 2.12 dioptres more myopic than that of a normal population. In addition astigmatic refractive errors of greater than +0.5 dioptres were found in 42% of this patient population, which is considerably more than the 18.6% observed in a normal population.
Adolescent↗
Spectral transmission characteristics of tinted lenses.
A total of 90 prescription and commercially available tinted lenses were evaluated for spectra transmission characteristics. An inappropriately large number of these lenses transmitted unnecessarily high amounts of light in the near ultraviolet, visible, and near infrared portions of the spectrum. No criteria regarding lens composition, color, or cost could reliably predict the effectiveness of individual lenses.
Color↗
Vitreous fluorophotometry in patients with retinitis pigmentosa.
Fifteen patients with various genetic types of retinitis pigmentosa and three carriers of the X-linked recessive type were examined with vitreous fluorophotometry. All patients with retinitis pigmentosa showed abnormally high concentrations of fluorescein within the vitreous that reflected an abnormality of the blood-retinal barrier. The amount of fluorescein within the vitreous correlated with the extent of photoreceptor and retinal pigment epithelial disease, as well as with the presence of leakage from retinal capillaries. Vitreous fluorophotometric measurements can show breakdown of the blood-retinal barrier in patients with retinitis pigmentosa who have no ophthalmoscopically apparent abnormalities and only minor changes on the electroretinogram (ERG). Furthermore, a breakdown of the blood-retinal barrier can be apparent in carrier of the X-linked recessive from of retinitis pigmentosa, even though the findings from a fundus examination and ERG recordings are normal.
Adolescent↗
Carriers of X-linked recessive retinitis pigmentosa: investigation by vitreous fluorophotometry.
The blood-retinal barrier was assessed by vitreous fluorophotometry in nine carriers of X-linked recessive retinitis pigmentosa. A breakdown of the blood-retinal barrier was apparent in all patients. In one carrier, the barrier was compromised even when abnormalities were not apparent in the retina ophthalmoscopically or by electroretinogram testing. The degree of abnormality of the blood-retinal barrier tended to parallel the extent of abnormalities noted on ophthalmoscopy, visual field examination, and electroretinogram testing.
Adolescent↗
Contrast sensitivity in retinitis pigmentosa.
Arden gratings were used to measure contrast sensitivity in 40 patients with retinitis pigmentosa whose Snellen visual acuity was 6/12 or better. When compared with a group of 30 normal subjects the patients with retinitis pigmentosa had substantially decreased contrast sensitivity, especially at high frequencies. The Arden grating test appeared to be a sensitive test of abnormal central visual function in patients with retinitis pigmentosa.
Adolescent↗
Driving performance of retinitis pigmentosa patients.
The driving performance of 42 patients with retinitis pigmentosa (RP) was compared with that of 87 control subjects. Although the patients with RP were involved in more isolated road accidents than the control group, 50% were not involved in any accident over the most recent 5-year driving period. When the associations between driving performance and case/control status were examined (the number of driving hours per week and driving years being taken into account), differences in the number of accidents between the 2 groups were significant because of a disproportionately high number of accidents caused by a subgroup of female RP patients. No significant correlations were apparent between central visual efficiency or peripheral field efficiency and number of road accidents.
Accidents, Traffic↗
Color vision defects in retinitis pigmentosa.
Sixty-seven patients with retinitis pigmentosa underwent color vision testing with the Nagel anomaloscope and Farnsworth-Munsell (FM) 100-hue test. Results showed both similarities and differences among different genetic types. The presence of an atrophic-appearing foveal lesion found in individual cases served as a reliable indicator of performance on color vision testing as did a reduction in visual acuity to less than 20/30. When no foveal lesion was apparent in patients with visual acuity better than 20/30, patients with autosomal dominant disease showed superior performance on color vision testing when compared to autosomal recessive, X-linked recessive, and isolated cases. Regardless of genetic type, the FM 100-hue test was more sensitive in detecting poor color vision performance than the Nagel anomaloscope.
Adolescent↗
X-linked recessive retinitis pigmentosa and vitreous fluorophotometry. A study of female heterozygotes.
Vitreous fluorophotometry was used to assess the functional integrity of the blood-retinal barrier in four female carriers of X-linked recessive retinitis pigmentosa. Abnormal findings in all four carriers suggest that a defect in the blood-retinal barrier, detectable by vitreous fluorophotometry, is a sensitive means by which carriers can be identified.
Adolescent↗
Rubella maculopathy.
Three patients with known history of congenital rubella and sudden decrease of vision are presented. Two of the 3 patients had previous eye examinations which showed typical rubella (salt and pepper) retinopathy. All 3 showed macular lesions associated with presumed subretinal neovascularisation.
Child↗
Loss of color vision and Stiles' II1 mechanism in a patient with cerebral infarction.
A 70-year old man developed achromatopsia with bilateral loss of superior visual fields and an inability to recognize familiar faces (prosopagnosia). Ophthalmologic examination results were normal. Visual acuity was 20/25 in either eye. Computerized axial tomography of the brain revealed infarction of the inferior aspect of the temporal occipital cortex in both hemispheres. The patient's complaint that objects appeared only in shades of gray was supported by large errors made throughout the spectrum on the Farnsworth-Munsell 100 hue test and by matches over the entire red/green range on the Nagel anomaloscope. Although absolute scotopic and photopic thresholds were unremarkable, the increment thresholds to a 482-nm test on a red background increased monotonically as if the II1 mechanism were absent. In addition, the spectral sensitivity to large test flashes on an intense red background peaked in the middle rather than in the short-wave portion of the spectrum, as is normally found. We speculate that the chromatic channel is compromised. The patient's residual vision is mediated by a luminance channel that is subserved by the middle and long--but not the short--wave cone mechanisms.
Aged↗
Traumatically acquired color vision defect.
A 24-year-old man acquired a color vision defect shortly after an accident in which he struck the back of his head. Results of the Farnsworth-Munsell 100-hue test showed that the patient had poor color discrimination in both eyes. His color matches on the Nagel anomaloscope suggested a red/green disturbance. Results from increment threshold testing demonstrated on absence of the blue mechanism. Results of field sensitivity measurements confirmed that foveal vision was mediated by the red or green mechanism. This case showed both similarities and differences to previously reported cases of acquired color vision defects secondary to cortical trauma.
Accidents, Traffic↗
Color matches of patients with retinitis pigmentosa.
The color matches of 46 retinitis pigmentosa (RP) patients were found to differ significantly from those of 55 unaffected observers. The color matches of group I (trichromatic) RP observers were more protanomalous (i.e., required a greater red/green mixture ratio) than those of unaffected observers. The matches of group II (dichromatic) RP observers were more protanopic (i.e., required less yellow to match the red primary) than those of the group II unaffected observers. In view of current evidence, this difference can be attributed to reductions in the pigment optical density of cone photoreceptors in eyes of RP patients.
Adolescent↗
Electro-oculogram testing in fundus flavimaculatus.
Electro-oculogram (EOG) measurements were obtained on seven patients with moderately extensive lesions of fundus flavimaculatus. Each patient was tested by two different methods of measuring EOG light-peak to dark-trough ratios. The results show that erroneously low ratios can be observed unless a diffusing sphere is employed in the determination of light-peak to dark-trough ratios in fundus flavimaculatus and presumably other diseases that affect primarily the posterior pole.
Adult↗
Dark adaptation testing in heterozygotes of Usher's syndrome.
Fourteen heterozygous carriers of Usher's syndrome were evaluated by ophthalmoscopy and dark adaptation testing. The normal findings in our study are discussed and compared with those of previous reports, which suggested that abnormal dark adaptation thresholds and fundus abnormalities may be seen in heterozygotes.
Adult↗
Dominantly inherited cystoid macular edema.
Four patients of Greek ancestry had dominantly inherited cystoid macular edema. Characteristics of this syndrome include the following: an early onset and prolonged course of cystoid changes in the macula, followed by atrophy of the macula in later stages. Some patients also show leakage of fluorescein from the optic disc capillaries, subnormal EOG Lp/Dt ratios, elevated rod dark adaptation thresholds, red-green and blue-yellow color deficiencies, normal ERG findings, hyperopia, peripheral pigmentary retinopathy, and vitreous opacities. Dominantly inherited cystoid macular edema is a distinct genetic trait among the dominantly inherited macular dystrophies.
Adult↗
Retinitis pigmentosa. Genetic percentages.
A total of 173 patients, from 124 families, with retinitis pigmentosa were evaluated for the presence of various forms of genetic transmission. Of the 124 pedigrees, it was evident that some form of genetic transmission had occurred in 59 (48%). In 65 families (52%), the proband was the only known affected family member (isolated case). In those families with know genetic transmission, 17% (10 patients) were X-linked recessive, 39% (23 patients) were either probable or certain autosomal dominant, 41% (24 patients) were certain or probable autosomal recessive, and 3% (2 patients) were of a genetic type that could not be determined with certainty. These findings are, to my knowledge, the first published series from the United States to indicate that the X-linked recessive form of retinitis pigmentosa is a more prevalent genetic trait than has been emphasized previously.
Chromosome Aberrations↗