The relationship between visual disability and visual scores in patients with retinitis pigmentosa.

PURPOSE: To evaluate the relationship between visual disability and visual scores in patients with retinitis pigmentosa. METHODS: The relationship between visual disability and visual scores (visual acuity and visual field) was investigated in 93 patients with retinitis pigmentosa. The visual disability of each patient was evaluated using a questionnaire (a total of 35 questions, in 7 sections, regarding daily life). The reproducibility and validity of the data obtained by the questionnaire had been established by a similar investigation in glaucoma patients. Mean (+/-SD) age of patients was 52.6 +/- 15.1 years, the mean visual acuity of the logarithm of the minimum angle of resolution (log10MAR) was 0.5 +/- 0.4, and the mean deviation of visual field with the Humphrey Field Analyzer program 30-2 was -22.0 +/- 10.9 dB. RESULTS: The visual acuity of log10MAR in the better eye (r = 0.66 to 0.81) and the mean sensitivity within the central 10 degrees of the visual field (r = -0.76 to -0.62) had a definite relationship to the visual disability index of each section and their sum (P < .0001). This relationship was also confirmed in multiple regression analysis, which showed a high correlation coefficient (R2 = 0.57 to 0.77, P < .0001). CONCLUSIONS: The retinal sensitivity within the central 10 degrees and the visual acuity of log10MAR in the better eye had a significant influence on a patient's daily life. We suggest that in patients with retinitis pigmentosa, visual disability in daily life can be precisely evaluated with the retinal sensitivity within the central 10 degrees and the visual acuity in the logarithm of the minimum angle of resolution in the better eye.

[The relationships between visual disability and visual scores in patients with retinitis pigmentosa].

PURPOSE: To evaluate the relationship between visual disability and visual scores in patients with retinitis pigmentosa. METHODS: The relationship between visual disability and visual scores (visual acuity and visual field) were investigated in 93 patients with retinitis pigmentosa. The visual disability of each patient was evaluated using a questionnaire (a total of 35 questions in 7 sections regarding daily life). Reproducibility and validity of the questionnaire had been established by an investigation in glaucoma patients. The mean (+/- standard deviation) age of patients was 52.6 +/- 15.1 years, the mean visual acuity (log10 MAR) was 0.6 +/- 0.4, and the mean deviation of visual field with Humphrey program 30-2 was -21.1 +/- 10.0 dB. RESULTS: The visual acuity in the better eye (r = 0.66-0.81) and the mean sensitivity within the central 10 degrees of the visual field (r = -0.76(-)-0.62) had a definite relationship to the visual disability index of each section (section DI) and their sum (total DI) (p < .0001). This relationship was also confirmed in stepwise multiple linear regression analysis which showed a high correlation coefficient (R2 = 0.57-0.77, p < .0001). CONCLUSIONS: Besides the visual acuity, the mean sensitivity within the central 10 degrees of the visual field is important in evaluating the visual disability of patients with retinitis pigmentosa.

[A study of para-foveal color vision in normal-tension and primary open angle glaucoma eyes].

A perimetric color vision test developed by Iijima et al. for evaluating parafoveal color vision provides information regarding local retinal function which can not be evaluated by other color vision tests. Using this method, we investigated para-foveal color sensitivity in normal-tension glaucoma (NTG) and primary open angle glaucoma (POAG) eyes whose glaucomatous visual field change determined with the central 30-2 program of the Humphrey visual field analyzer was confined to either the upper or the lower hemifield. The examined eyes had no abnormalities in ocular media with best corrected visual acuity of 1.0 or higher, and age, refraction, mean deviation, and extent of visual field loss were matched between NTG and POAG eyes. In the para-foveal area of the spared hemifield, acquired blue-yellow dyschromatopsia was detected in 52.3% of the POAG eyes and in 11.3% of the NTG eyes (p < 0.02, chi 2-test), and in damaged hemifield, it was found in 76.1% of the POAG eyes and 80.7% of the NTG eyes. We suggest that visual function is more diffusely damaged in POAG than in NTG and that the perimetric color vision test is useful for detecting glaucomatous damage which cannot be detected with conventional light threshold measurements.

Cellular immune responses to retinal antigens in retinitis pigmentosa.

Patients with retinitis pigmentosa and a group of controls were tested for their cellular immune response toward two retinal proteins, S-antigen and interphotoreceptor retinoid-binding protein (IRBP), as well as their reaction against two synthetic peptides ("M" and "N") derived from the sequence of S-antigen and peptide "R14", derived from IRBP. Positive responses to the retinal antigens were found in larger proportions and with higher levels in the patient group than in the controls. The difference between the two groups was statistically significant in their response to S-antigen, but the patients reacted better than the controls against the other antigens as well. Of particular interest was the finding that several patients responded to both retinal proteins and/or to their peptides. These patients suffered from severe retinal changes and the data are thus interpreted as suggesting that the responses to the retinal antigens are secondary to these changes and to nonphysiological release of retinal antigens.

Central pigmentary retinal dystrophy and its angiographic classification.

Twenty-one cases of central pigmentary retinal dystrophy were compiled and their clinical features were described. On the basis of fluorescein fundus angiography, these cases were classified into three types according to the changes in the choriocapillaris. In Type 1, the choriocapillaris showed annular or bow-shaped atrophy around the vascular arcade in the posterior pole of the fundus, but little change in the foveal region. Type 2 exhibited round or irregularly shaped atrophy of the choriocapillaris in the center of the posterior pole of the fundus. Type 3 had no atrophy of the choriocapillaris and showed only hyperfluorescence in the posterior pole of the fundus, due to damage of the retinal pigment epithelium. These types differed from each other not only from the morphological point of view, but also in other clinical findings that included age of onset, progression of visual impairment, pattern of color vision defects or recordability of the electroretinogram. It was discussed that central pigmentary retinal dystrophy consists of heterogenous conditions.

Color vision defects in pigmentary retinal dystrophy.

Color vision was studied, using the Farnsworth Panel D-15 test, in 72 patients (115 eyes) with primary pigmentary retina dystrophy of autosomal recessive inheritance, and the results were correlated with the visual acuity and visual field. The incidence of color vision defects and the degree of disturbance increased as the visual acuity and the visual field deteriorated. However, even in cases with the visual acuity better than 0.7, type III acquired blue-yellow defect was found in 22% of the cases. This type of color vision defect was also found in 52% of the cases with the visual acuity between 0.4 and 0.6. In the group with the visual acuity of 0.1 or less, total achromatopsia was found in 64% of the cases. The increment thresholds of the blue and green cone mechanisms in the fovea were determined by the two-color threshold technique of Stiles in 12 patients with the visual acuity better than 0.8. The thresholds of the blue cone mechanism (pi 1) and of the green cone mechanism (pi 4) were found to be elevated over the normal values. The increases in the former and the latter thresholds were correlated linearly with the slope of the regression of 0.64. The increase in the threshold of the blue cone mechanism was more pronounced than that of the green cone mechanism. Due to the difference in the density of both cones in the fovea, this result does not necessarily support the hypothesis that the blue cone mechanism is affected preferentially more than the green cone mechanism.

The early receptor potential in the human eye. III. ERP in dichromats.

The human early receptor potential (ERP) was studied in 42 dichromats, 13 cases of protanopia and 29 cases of deuteranopia, and the results were compared with those in 28 normal subjects. The amplitudes of the R2 wave of the ERP in the protanopia as well as the deuteranopia cases were significantly smaller than that of the normal subjects. The sensitivity of the R2 wave of the ERP in the dichromats was higher than that of the normal subjects. Possibilities are discussed that dichromats have fewer cones or that they have lower pigment density in individual cones than normal subjects.