Abstract

The patient of this study is a protanope who is afflicted with retinitis pigmentosa. As with other patients studied, an abnormality in Stiles’s pi-1 mechanism was found in the patient’s worse eye (20/35 visual acuity). From his rejection of monochromatic color matches, we inferred that the sensitivity of the short-wave cones is reduced, but their signals are not abnormally attenuated by the middle-wave cones. The patient’s pi-4 thresholds, however, were within the range of normal control values.

© 1982 Optical Society of America

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References

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  1. M. F. Marmor, “Visual loss in retinitis pigmentosa,” Am. J. Ophthalmol. 89, 692–698 (1980).
    [PubMed]
  2. R. S. L. Young and G. A. Fishman, “Color matches of patients with retinitis pigmentosa,” Invest. Ophthalmol. Vis. Sci. 19, 967–972 (1980).
    [PubMed]
  3. M. A. Sandberg and E. L. Berson, “Blue and green cone mechanisms in retinitis pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149–157 (1977).
  4. R. S. L. Young and G. A. Fishman, “Sensitivity losses in a long wavelength sensitive mechanism of patients with retinitis pigmentosa,” Vision Res. (to be published).
  5. E. L. Berson and E. A. Simonoff, “Dominant retinitis pigmentosa with reduced penetrance,” Arch. Ophthalmol. 97, 1286–1291 (1979).
    [Crossref] [PubMed]
  6. W. S. Stiles, “Separation of the blue and green mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. London Ser. B 133, 418–434 (1946).
    [Crossref]
  7. D. Kirk, “Color discrimination at threshold,” Invest. Ophthalmol. Vis. Sci. Suppl. 19, 135 (1980).
  8. V. C. Smith and J. Pokorny, “Spectral sensitivity of the foveal cone photopigments between 400 and 500 nm,” Vision Res. 15, 161–171 (1975).
    [Crossref] [PubMed]
  9. E. N. Pugh, “The nature of the pi 1 colour mechanism of W. S. Stiles,” J. Physiol. 257, 713–747 (1976).
  10. E. N. Pugh and J. D. Mollon, “A theory of the pi 1 and pi 3 color mechanisms of Stiles,” Vision Res. 19, 293–312 (1979).
    [Crossref]
  11. H. Kolb and P. Gouras, “Electron microscopic observations of human retinitis pigmentosa, dominantly inherited,” Invest. Ophthalmol. 13, 487–498 (1974).
    [PubMed]
  12. R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
    [PubMed]
  13. G. Wyszecki and W. S. Stiles, Color Science: Concepts and Methods, Quantitative Data and Formulas (Wiley, New York, 1967), p. 408.
  14. F. Flamant and W. S. Stiles, “The directional and spectral sensitivities of the retinal rods to adapting fields of different wavelengths,” J. Physiol. 107, 187–202 (1948).

1980 (3)

M. F. Marmor, “Visual loss in retinitis pigmentosa,” Am. J. Ophthalmol. 89, 692–698 (1980).
[PubMed]

R. S. L. Young and G. A. Fishman, “Color matches of patients with retinitis pigmentosa,” Invest. Ophthalmol. Vis. Sci. 19, 967–972 (1980).
[PubMed]

D. Kirk, “Color discrimination at threshold,” Invest. Ophthalmol. Vis. Sci. Suppl. 19, 135 (1980).

1979 (3)

E. N. Pugh and J. D. Mollon, “A theory of the pi 1 and pi 3 color mechanisms of Stiles,” Vision Res. 19, 293–312 (1979).
[Crossref]

E. L. Berson and E. A. Simonoff, “Dominant retinitis pigmentosa with reduced penetrance,” Arch. Ophthalmol. 97, 1286–1291 (1979).
[Crossref] [PubMed]

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

1977 (1)

M. A. Sandberg and E. L. Berson, “Blue and green cone mechanisms in retinitis pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149–157 (1977).

1976 (1)

E. N. Pugh, “The nature of the pi 1 colour mechanism of W. S. Stiles,” J. Physiol. 257, 713–747 (1976).

1975 (1)

V. C. Smith and J. Pokorny, “Spectral sensitivity of the foveal cone photopigments between 400 and 500 nm,” Vision Res. 15, 161–171 (1975).
[Crossref] [PubMed]

1974 (1)

H. Kolb and P. Gouras, “Electron microscopic observations of human retinitis pigmentosa, dominantly inherited,” Invest. Ophthalmol. 13, 487–498 (1974).
[PubMed]

1948 (1)

F. Flamant and W. S. Stiles, “The directional and spectral sensitivities of the retinal rods to adapting fields of different wavelengths,” J. Physiol. 107, 187–202 (1948).

1946 (1)

W. S. Stiles, “Separation of the blue and green mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. London Ser. B 133, 418–434 (1946).
[Crossref]

Berson, E. L.

E. L. Berson and E. A. Simonoff, “Dominant retinitis pigmentosa with reduced penetrance,” Arch. Ophthalmol. 97, 1286–1291 (1979).
[Crossref] [PubMed]

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

M. A. Sandberg and E. L. Berson, “Blue and green cone mechanisms in retinitis pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149–157 (1977).

Fishman, G. A.

R. S. L. Young and G. A. Fishman, “Color matches of patients with retinitis pigmentosa,” Invest. Ophthalmol. Vis. Sci. 19, 967–972 (1980).
[PubMed]

R. S. L. Young and G. A. Fishman, “Sensitivity losses in a long wavelength sensitive mechanism of patients with retinitis pigmentosa,” Vision Res. (to be published).

Flamant, F.

F. Flamant and W. S. Stiles, “The directional and spectral sensitivities of the retinal rods to adapting fields of different wavelengths,” J. Physiol. 107, 187–202 (1948).

Gouras, P.

H. Kolb and P. Gouras, “Electron microscopic observations of human retinitis pigmentosa, dominantly inherited,” Invest. Ophthalmol. 13, 487–498 (1974).
[PubMed]

Kirk, D.

D. Kirk, “Color discrimination at threshold,” Invest. Ophthalmol. Vis. Sci. Suppl. 19, 135 (1980).

Klein, R.

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

Kolb, H.

H. Kolb and P. Gouras, “Electron microscopic observations of human retinitis pigmentosa, dominantly inherited,” Invest. Ophthalmol. 13, 487–498 (1974).
[PubMed]

Marmor, M. F.

M. F. Marmor, “Visual loss in retinitis pigmentosa,” Am. J. Ophthalmol. 89, 692–698 (1980).
[PubMed]

Meyers, S.

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

Mollon, J. D.

E. N. Pugh and J. D. Mollon, “A theory of the pi 1 and pi 3 color mechanisms of Stiles,” Vision Res. 19, 293–312 (1979).
[Crossref]

Pokorny, J.

V. C. Smith and J. Pokorny, “Spectral sensitivity of the foveal cone photopigments between 400 and 500 nm,” Vision Res. 15, 161–171 (1975).
[Crossref] [PubMed]

Pugh, E. N.

E. N. Pugh and J. D. Mollon, “A theory of the pi 1 and pi 3 color mechanisms of Stiles,” Vision Res. 19, 293–312 (1979).
[Crossref]

E. N. Pugh, “The nature of the pi 1 colour mechanism of W. S. Stiles,” J. Physiol. 257, 713–747 (1976).

Sandberg, M. A.

M. A. Sandberg and E. L. Berson, “Blue and green cone mechanisms in retinitis pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149–157 (1977).

Simonoff, E. A.

E. L. Berson and E. A. Simonoff, “Dominant retinitis pigmentosa with reduced penetrance,” Arch. Ophthalmol. 97, 1286–1291 (1979).
[Crossref] [PubMed]

Smith, V. C.

V. C. Smith and J. Pokorny, “Spectral sensitivity of the foveal cone photopigments between 400 and 500 nm,” Vision Res. 15, 161–171 (1975).
[Crossref] [PubMed]

Stiles, W. S.

F. Flamant and W. S. Stiles, “The directional and spectral sensitivities of the retinal rods to adapting fields of different wavelengths,” J. Physiol. 107, 187–202 (1948).

W. S. Stiles, “Separation of the blue and green mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. London Ser. B 133, 418–434 (1946).
[Crossref]

G. Wyszecki and W. S. Stiles, Color Science: Concepts and Methods, Quantitative Data and Formulas (Wiley, New York, 1967), p. 408.

Szamier, R. B.

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

Wyszecki, G.

G. Wyszecki and W. S. Stiles, Color Science: Concepts and Methods, Quantitative Data and Formulas (Wiley, New York, 1967), p. 408.

Young, R. S. L.

R. S. L. Young and G. A. Fishman, “Color matches of patients with retinitis pigmentosa,” Invest. Ophthalmol. Vis. Sci. 19, 967–972 (1980).
[PubMed]

R. S. L. Young and G. A. Fishman, “Sensitivity losses in a long wavelength sensitive mechanism of patients with retinitis pigmentosa,” Vision Res. (to be published).

Am. J. Ophthalmol. (1)

M. F. Marmor, “Visual loss in retinitis pigmentosa,” Am. J. Ophthalmol. 89, 692–698 (1980).
[PubMed]

Arch. Ophthalmol. (1)

E. L. Berson and E. A. Simonoff, “Dominant retinitis pigmentosa with reduced penetrance,” Arch. Ophthalmol. 97, 1286–1291 (1979).
[Crossref] [PubMed]

Invest. Ophthalmol. (1)

H. Kolb and P. Gouras, “Electron microscopic observations of human retinitis pigmentosa, dominantly inherited,” Invest. Ophthalmol. 13, 487–498 (1974).
[PubMed]

Invest. Ophthalmol. Vis. Sci. (2)

R. B. Szamier, E. L. Berson, R. Klein, and S. Meyers, “Sex-linked retinitis pigmentosa: ultrastructure of photoreceptors and pigment epithelium,” Invest. Ophthalmol. Vis. Sci. 18, 145–160 (1979).
[PubMed]

R. S. L. Young and G. A. Fishman, “Color matches of patients with retinitis pigmentosa,” Invest. Ophthalmol. Vis. Sci. 19, 967–972 (1980).
[PubMed]

Invest. Ophthalmol. Vis. Sci. Suppl. (1)

D. Kirk, “Color discrimination at threshold,” Invest. Ophthalmol. Vis. Sci. Suppl. 19, 135 (1980).

Invest. Ophthalmol. Visual Sci. (1)

M. A. Sandberg and E. L. Berson, “Blue and green cone mechanisms in retinitis pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149–157 (1977).

J. Physiol. (2)

E. N. Pugh, “The nature of the pi 1 colour mechanism of W. S. Stiles,” J. Physiol. 257, 713–747 (1976).

F. Flamant and W. S. Stiles, “The directional and spectral sensitivities of the retinal rods to adapting fields of different wavelengths,” J. Physiol. 107, 187–202 (1948).

Proc. R. Soc. London Ser. B (1)

W. S. Stiles, “Separation of the blue and green mechanisms of foveal vision by measurements of increment thresholds,” Proc. R. Soc. London Ser. B 133, 418–434 (1946).
[Crossref]

Vision Res. (2)

V. C. Smith and J. Pokorny, “Spectral sensitivity of the foveal cone photopigments between 400 and 500 nm,” Vision Res. 15, 161–171 (1975).
[Crossref] [PubMed]

E. N. Pugh and J. D. Mollon, “A theory of the pi 1 and pi 3 color mechanisms of Stiles,” Vision Res. 19, 293–312 (1979).
[Crossref]

Other (2)

G. Wyszecki and W. S. Stiles, Color Science: Concepts and Methods, Quantitative Data and Formulas (Wiley, New York, 1967), p. 408.

R. S. L. Young and G. A. Fishman, “Sensitivity losses in a long wavelength sensitive mechanism of patients with retinitis pigmentosa,” Vision Res. (to be published).

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Figures (7)

Fig. 1
Fig. 1

Four-channel Maxwellian-view optical system used as a colorimeter. FS1 and FS2 served as 1° hemicircular field stops, i.e., for the bipartite stimulus. Light traversing the monochromator, MC, illuminates FS1. Light from the other two ports of the xenon lamp, S1, provides the color-mixing primaries for FS2. Channel 0 was not used in the present experiments. AL, an achromatizing lens, compensated for achromatic aberration originating from the human eye. W1-3 are servo-controlled variable-density wedges. AS, M, L1-8, BS, and SH are aperture stops, mirrors, lenses, beam-splitter cubes, and an electromechanical shutter, respectively.

Fig. 2
Fig. 2

Foveal (1°) color-matching functions of protanopes. Data for patient GP (filled symbols) and for normal congenital protanope CL (open symbols) are the mean of three replications. Solid curves show protanopic color-matching functions13 that were normalized at 430 and 560 nm, the wavelengths of the primaries used in the present study.

Fig. 3
Fig. 3

Stiles’s pi 1 increment-threshold branch in the patient’s left (bottom)—but not right (top)—fovea. The test wavelength was 482 nm, and the field wavelength was 610 nm. The data points represent the mean ±2 sem (n = 4 or n = 6 replications). Solid curve is a template curve drawn to provide best fit to the pi 4 branch of the increment threshold in the left fovea (bottom) and was displaced vertically to fit the data for the right fovea (top). Dashed curves (top) illustrate the hypothesis that the test sensitivity of the pi 1 mechanism in the right—as compared with the left—fovea was reduced by 101.1, i.e., the sensitivity reduction inferred from color matches (Fig. 6).

Fig. 4
Fig. 4

Stiles’s pi mechanisms in the foveae of eight normal protanopes. The test flash was either 476 or 482 nm; the field was either 610 nm (top) or 550 nm (bottom). In the text, pi 4 refers to the lower branch (solid curve) and pi 1 mechanism refers to the upper branch (dashed curve) of each increment-threshold function. The solid curve was visually fitted to the lower portion of the data of each observer by horizontal and vertical translations of a template curve; the dashed curve was drawn to conform to the general trend of the upper portion.

Fig. 5
Fig. 5

Foveal increment-threshold functions (patient GP) for three test wavelengths (430, 548, and 656 nm) against a 550-nm background field. The solid curve (top) is the same template as that used in Figs. 3 and 4. Displaced vertically, the template curve provides good fit to the data of the 548 nm but a poor fit to the data of the 430-nm condition. The dashed curves (bottom) illustrate the hypothesis that the test sensitivity of the pi 1 mechanism in the right—as compared with the left—fovea is reduced by 101.1, the sensitivity reduction inferred from color matches (Fig. 6). (Note that 548-nm data have been arbitrarily vertically displaced by 2.5 and the 656-nm data by 1.5 log quantum flux for the sake of clarity.)

Fig. 6
Fig. 6

Relative sensitivity of the patient’s sws cone mechanisms estimated from foveal color matches between 610- and 513-nm halves of a 1° bipartite field (circles, OS; triangle, OD). As the intensity of the 513-nm field was varied in 100.3 steps, the patient attempted to match the two primary fields only by adjusting the 610-nm flux. Note that the data for the left eye (circles) have been displaced vertically by 101.5 for clarity. Solid lines have a slope of unity and represent the expected color-matching settings, assuming that the matches were mediated only by mws cones (see Fig. 7). At levels greater than some 513-nm flux, the patient was no longer able to match the two primaries. The mid-value 513 flux (dashed line) provides an estimate of the sws cone threshold.

Fig. 7
Fig. 7

Patient’s mws (pi 4) test- and field-sensitivity spectra in the right fovea. Field sensitivities (right ordinate) were determined with either 482- or 656-nm test flash by using a method similar to that described in Ref. 14. Test sensitivities (left ordinate) were measured in the dark. The two sets of data have been displaced vertically to illustrate the spectral similarity between the field sensitivity and test sensitivity. Solid curve is an estimate of the mws cone action spectrum, adapted from Smith and Pokorny.8

Tables (1)

Tables Icon

Table 1 Test and Field Sensitivities of Pi 4 Mechanisma