Abstract

Rod system increment threshold functions (ITFs) were studied in patients with cone–rod dystrophy (CRD). Rod thresholds (It) for a 104-min, 500-nm test stimulus (TS), superimposed on an 11° long wavelength background (luminance Ib), were measured in eleven CRD patients and fourteen normal subjects. Thresholds in normals were also measured using a 7-min TS. A modified version of Weber’s law [logIt=logK + log(Ibn +c)] was fitted to the data from each subject and test condition to yield a description of the ITF in terms of the free parameters K, c, and n. Four of the CRD patients exhibited an abnormally high absolute threshold; of these, two showed abnormalities in K and c consistent with a reduced efficiency of quantum capture by the rods. Abnormalities in the ITFs of CRD patients did not resemble the effect of reducing the diameter of the TS from 104 to 7 min in normals. This suggests that threshold abnormalities in the CRD patients did not result from altered spatial summation. The results illustrate use of a parametric representation of the ITF to evaluate the loss of sensitivity in visual disorders.

© 1989 Optical Society of America

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  1. G. Goodman, H. Ripps, I. M. Siegel, “Cone Dysfunction Syndromes,” Arch. Ophthalmol. 70, 214 (1963).
    [CrossRef] [PubMed]
  2. E. L. Berson, P. Gouras, R. D. Gunkel, “Progressive Cone–Rod Degeneration,” Arch. Ophthalmol. 80, 68 (1968).
    [CrossRef] [PubMed]
  3. A. E. Krill, A. F. Deutman, M. Fishman, “The Cone Degenerations,” Doc. Ophthalmol. 35, 1 (1973).
    [CrossRef] [PubMed]
  4. R. E. Carr, I. M. Siegel, “Cone Dysfunctions in Man,” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 653 (1976).
  5. A. E. Krill, “Cone Degenerations,” in Krill’s Hereditary Retinal and Choroidal Diseases. Vol. II. Clinical Characteristics, A.E. Krill, Ed. (Harper & Row, Hagerstown, MD, 1977), p. 421.
  6. G. A. Fishman, “Progressive Human Cone-Rod Dysfunction (Dystrophy),” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 716 (1976).
  7. G. A. Fishman, “Electroretinography and Inherited Macular Dystrophies,” Retina 5, 172 (1985).
    [CrossRef] [PubMed]
  8. R. G. Weleber, A. Eisner, “Cone Degenerations (Bull’s-Eye Dystrophies) and Color Vision Defects,” in Retinal Dystrophies and Degenerations, D. A. Newsome, Ed. (Raven Press, New York, 1988), p. 223.
  9. J. Babel, N. Stangos, “Progressive Degeneration of the Photopic System,” Am. J. Ophthalmol. 75, 511 (1973).
    [PubMed]
  10. D. G. Birch, G. E. Fish, “Rod ERGs in Retinitis Pigmentosa and Cone–Rod Degeneration,” Invest. Ophthalmol. Visual Sci. 28, 140 (1987).
  11. S. Hecht, “Rods, Cones, and the Chemical Basis of Vision,” Physiol. Rev. 17, 239 (1937).
  12. M. Aguilar, W. S. Stiles, “Saturation of the Rod Mechanism of the Retina at High Levels of Stimulation,” Opt. Acta 1, 59 (1954).
    [CrossRef]
  13. H. B Barlow, “Dark and Light Adaptation: Psychophysics,” in Handbook of Sensory Physiology. Vol. VII/4, D. Jameson, L. M. Hurvich, Eds. (Springer-Verlag, Berlin, 1972), p. 1.
    [CrossRef]
  14. D. C. Hood, V. C. Greenstein, “Increment Threshold (tvi) Data and the Site of Disease Action,” in Technical Digest of Topical Meeting on Noninvasive Assessment of the Visual System (Optical Society of America, Washington, DC, 1988), Vol. 3, p. 2.
  15. R. S. L. Young, J. Price, J. Harrison, “Psychophysical Study of Rod Adaptation in Patients with Congenital Stationary Night Blindness,” Clin. Vis. Sci. 1, 137 (1986).
  16. B. Stabell, K. Nordby, U. Stabell, “Light-Adaptation of the Human Rod System,” Clin. Vis. Sci. 2, 83 (1987).
  17. D. C. Hood, V. C. Greenstein, “An Approach to Testing Alternative Hypotheses of Changes in Visual Sensitivity Due to Retinal Disease,” Invest. Ophthalmol. Visual Sci. 23, 96 (1982).
  18. W. S. Geisler, “Mechanisms of Visual Sensitivity: Backgrounds and Early Dark Adaptation,” Vision Res. 12, 1423 (1983).
    [CrossRef]
  19. R. W. Massof, S. Marcus, G. Dagnelie, D. Choy, J. Sunness, M. Albert, “Theoretical Interpretation and Derivation of Flash-on-Flash Threshold Parameters in Visual System Diseases,” Appl. Opt. 27, 1014 (1988).
    [CrossRef] [PubMed]
  20. D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).
  21. G. A. Fishman, G. Buckman, T. van Every, “Fundus Flavimaculatus: a Clinical Classification,” Doc. Opthalmol. 13, 213 (1977).
  22. The ERG responses of patients 8, 10, and 11 to a single short wavelength flash were nondetectable. However, in patients 8 and 10, markedly reduced but detectable responses were apparent to a 10-Hz short wavelength flickering stimulus when several responses were averaged. Furthermore, the responses of these two patients to a white flash was appreciably greater in darkadapted (vs light-adapted) conditions, strongly suggesting that the dark-adapted response was rod-mediated. Patient 11 was somewhat atypical, in that her response to even a bright white flash was <50 μV. Nevertheless, this patient did not indicate night blindness as her initial or major complaint and did not manifest any clinical evidence of peripheral retinal degeneration. The visual acuity of 20/30 in this patient is consistent with the diagnosis of CRD, as was her impaired color vision.
  23. R. W. Massof, D. Finkelstein, “Two Forms of Autosomal Dominant Primary Retinitis Pigmentosa,” Doc. Ophthalmol. 51, 289 (1981).
    [CrossRef] [PubMed]
  24. K. R. Alexander, G. A. Fishman, “Prolonged Rod Dark Adaptation in Retinitis Pigmentosa,” Br. J. Ophthalmol. 68, 561 (1984).
    [CrossRef] [PubMed]
  25. S. L. Guth, R. W. Massof, T. Benzschawel, “Vector Model for Normal and Dichromatic Color Vision,” J. Opt. Soc. Am. 70, 197 (1980).
    [CrossRef] [PubMed]
  26. C. B. Blakemore, W. A. H. Rushton, “Dark Adaptation and Increment Threshold in a Rod Monochromat,” J. Physiol. (London) 181, 612 (1965).
  27. D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
    [CrossRef] [PubMed]
  28. J. W. Clack, D. R. Pepperberg, “Desensitization of Skate Photoreceptors by Bleaching and Background Light,” J. Gen. Physiol. 80, 863 (1982).
    [CrossRef] [PubMed]
  29. D. A. Baylor, B. J. Nunn, J. L. Schnapf, “The Photocurrent, Noise and Spectral Sensitivity of Rods of the Monkey, Macaca Fascicularis,” J. Physiol. (London) 357, 575 (1984).
  30. R. Shapley, C. Enroth-Cugell, “Visual Adaptation and Retinal Gain Controls,” Prog. Retinal Res. 3, 263 (1984).
    [CrossRef]
  31. H. Ripps, D. R. Pepperberg, “Photoreceptor Processes in Visual Adaptation,” Neurosci. Res. Suppl. 6, S87 (1987).
  32. V. N. Highman, R. A. Weale, “Rhodopsin Density and Visual Threshold in Retinitis Pigmentosa,” Am. J. Ophthalmol. 75, 822 (1973).
    [PubMed]
  33. H. Ripps, K. P. Brin, R. A. Weale, “Rhodopsin and Visual Threshold in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 17, 735 (1978).
  34. I. Perlman, E. Auerbach, “The Relationship Between Visual Sensitivity and Rhodopsin Density in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 20, 758 (1981).
  35. H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
    [PubMed]
  36. M. A. Sandberg, E. L. Berson, “Blue and Green Cone Mechanisms in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149 (1977).

1988 (1)

1987 (5)

D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).

H. Ripps, D. R. Pepperberg, “Photoreceptor Processes in Visual Adaptation,” Neurosci. Res. Suppl. 6, S87 (1987).

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

D. G. Birch, G. E. Fish, “Rod ERGs in Retinitis Pigmentosa and Cone–Rod Degeneration,” Invest. Ophthalmol. Visual Sci. 28, 140 (1987).

B. Stabell, K. Nordby, U. Stabell, “Light-Adaptation of the Human Rod System,” Clin. Vis. Sci. 2, 83 (1987).

1986 (1)

R. S. L. Young, J. Price, J. Harrison, “Psychophysical Study of Rod Adaptation in Patients with Congenital Stationary Night Blindness,” Clin. Vis. Sci. 1, 137 (1986).

1985 (1)

G. A. Fishman, “Electroretinography and Inherited Macular Dystrophies,” Retina 5, 172 (1985).
[CrossRef] [PubMed]

1984 (3)

D. A. Baylor, B. J. Nunn, J. L. Schnapf, “The Photocurrent, Noise and Spectral Sensitivity of Rods of the Monkey, Macaca Fascicularis,” J. Physiol. (London) 357, 575 (1984).

R. Shapley, C. Enroth-Cugell, “Visual Adaptation and Retinal Gain Controls,” Prog. Retinal Res. 3, 263 (1984).
[CrossRef]

K. R. Alexander, G. A. Fishman, “Prolonged Rod Dark Adaptation in Retinitis Pigmentosa,” Br. J. Ophthalmol. 68, 561 (1984).
[CrossRef] [PubMed]

1983 (1)

W. S. Geisler, “Mechanisms of Visual Sensitivity: Backgrounds and Early Dark Adaptation,” Vision Res. 12, 1423 (1983).
[CrossRef]

1982 (2)

D. C. Hood, V. C. Greenstein, “An Approach to Testing Alternative Hypotheses of Changes in Visual Sensitivity Due to Retinal Disease,” Invest. Ophthalmol. Visual Sci. 23, 96 (1982).

J. W. Clack, D. R. Pepperberg, “Desensitization of Skate Photoreceptors by Bleaching and Background Light,” J. Gen. Physiol. 80, 863 (1982).
[CrossRef] [PubMed]

1981 (2)

I. Perlman, E. Auerbach, “The Relationship Between Visual Sensitivity and Rhodopsin Density in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 20, 758 (1981).

R. W. Massof, D. Finkelstein, “Two Forms of Autosomal Dominant Primary Retinitis Pigmentosa,” Doc. Ophthalmol. 51, 289 (1981).
[CrossRef] [PubMed]

1980 (1)

1978 (1)

H. Ripps, K. P. Brin, R. A. Weale, “Rhodopsin and Visual Threshold in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 17, 735 (1978).

1977 (2)

M. A. Sandberg, E. L. Berson, “Blue and Green Cone Mechanisms in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149 (1977).

G. A. Fishman, G. Buckman, T. van Every, “Fundus Flavimaculatus: a Clinical Classification,” Doc. Opthalmol. 13, 213 (1977).

1976 (2)

R. E. Carr, I. M. Siegel, “Cone Dysfunctions in Man,” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 653 (1976).

G. A. Fishman, “Progressive Human Cone-Rod Dysfunction (Dystrophy),” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 716 (1976).

1975 (1)

D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
[CrossRef] [PubMed]

1973 (3)

A. E. Krill, A. F. Deutman, M. Fishman, “The Cone Degenerations,” Doc. Ophthalmol. 35, 1 (1973).
[CrossRef] [PubMed]

J. Babel, N. Stangos, “Progressive Degeneration of the Photopic System,” Am. J. Ophthalmol. 75, 511 (1973).
[PubMed]

V. N. Highman, R. A. Weale, “Rhodopsin Density and Visual Threshold in Retinitis Pigmentosa,” Am. J. Ophthalmol. 75, 822 (1973).
[PubMed]

1968 (1)

E. L. Berson, P. Gouras, R. D. Gunkel, “Progressive Cone–Rod Degeneration,” Arch. Ophthalmol. 80, 68 (1968).
[CrossRef] [PubMed]

1965 (1)

C. B. Blakemore, W. A. H. Rushton, “Dark Adaptation and Increment Threshold in a Rod Monochromat,” J. Physiol. (London) 181, 612 (1965).

1963 (1)

G. Goodman, H. Ripps, I. M. Siegel, “Cone Dysfunction Syndromes,” Arch. Ophthalmol. 70, 214 (1963).
[CrossRef] [PubMed]

1954 (1)

M. Aguilar, W. S. Stiles, “Saturation of the Rod Mechanism of the Retina at High Levels of Stimulation,” Opt. Acta 1, 59 (1954).
[CrossRef]

1937 (1)

S. Hecht, “Rods, Cones, and the Chemical Basis of Vision,” Physiol. Rev. 17, 239 (1937).

Aguilar, M.

M. Aguilar, W. S. Stiles, “Saturation of the Rod Mechanism of the Retina at High Levels of Stimulation,” Opt. Acta 1, 59 (1954).
[CrossRef]

Albert, M.

Alexander, K. R.

D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).

K. R. Alexander, G. A. Fishman, “Prolonged Rod Dark Adaptation in Retinitis Pigmentosa,” Br. J. Ophthalmol. 68, 561 (1984).
[CrossRef] [PubMed]

Auerbach, E.

I. Perlman, E. Auerbach, “The Relationship Between Visual Sensitivity and Rhodopsin Density in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 20, 758 (1981).

Babel, J.

J. Babel, N. Stangos, “Progressive Degeneration of the Photopic System,” Am. J. Ophthalmol. 75, 511 (1973).
[PubMed]

Barlow, H. B

H. B Barlow, “Dark and Light Adaptation: Psychophysics,” in Handbook of Sensory Physiology. Vol. VII/4, D. Jameson, L. M. Hurvich, Eds. (Springer-Verlag, Berlin, 1972), p. 1.
[CrossRef]

Baylor, D. A.

D. A. Baylor, B. J. Nunn, J. L. Schnapf, “The Photocurrent, Noise and Spectral Sensitivity of Rods of the Monkey, Macaca Fascicularis,” J. Physiol. (London) 357, 575 (1984).

Benzschawel, T.

Berson, E. L.

M. A. Sandberg, E. L. Berson, “Blue and Green Cone Mechanisms in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149 (1977).

E. L. Berson, P. Gouras, R. D. Gunkel, “Progressive Cone–Rod Degeneration,” Arch. Ophthalmol. 80, 68 (1968).
[CrossRef] [PubMed]

Birch, D. G.

D. G. Birch, G. E. Fish, “Rod ERGs in Retinitis Pigmentosa and Cone–Rod Degeneration,” Invest. Ophthalmol. Visual Sci. 28, 140 (1987).

Blakemore, C. B.

C. B. Blakemore, W. A. H. Rushton, “Dark Adaptation and Increment Threshold in a Rod Monochromat,” J. Physiol. (London) 181, 612 (1965).

Brin, K. P.

H. Ripps, K. P. Brin, R. A. Weale, “Rhodopsin and Visual Threshold in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 17, 735 (1978).

Buckman, G.

G. A. Fishman, G. Buckman, T. van Every, “Fundus Flavimaculatus: a Clinical Classification,” Doc. Opthalmol. 13, 213 (1977).

Carr, R. E.

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

R. E. Carr, I. M. Siegel, “Cone Dysfunctions in Man,” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 653 (1976).

Choy, D.

Clack, J. W.

J. W. Clack, D. R. Pepperberg, “Desensitization of Skate Photoreceptors by Bleaching and Background Light,” J. Gen. Physiol. 80, 863 (1982).
[CrossRef] [PubMed]

Dagnelie, G.

Derlacki, D. J.

D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).

Deutman, A. F.

A. E. Krill, A. F. Deutman, M. Fishman, “The Cone Degenerations,” Doc. Ophthalmol. 35, 1 (1973).
[CrossRef] [PubMed]

Dowling, J. E.

D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
[CrossRef] [PubMed]

Eisner, A.

R. G. Weleber, A. Eisner, “Cone Degenerations (Bull’s-Eye Dystrophies) and Color Vision Defects,” in Retinal Dystrophies and Degenerations, D. A. Newsome, Ed. (Raven Press, New York, 1988), p. 223.

Enroth-Cugell, C.

R. Shapley, C. Enroth-Cugell, “Visual Adaptation and Retinal Gain Controls,” Prog. Retinal Res. 3, 263 (1984).
[CrossRef]

Finkelstein, D.

R. W. Massof, D. Finkelstein, “Two Forms of Autosomal Dominant Primary Retinitis Pigmentosa,” Doc. Ophthalmol. 51, 289 (1981).
[CrossRef] [PubMed]

Fish, G. E.

D. G. Birch, G. E. Fish, “Rod ERGs in Retinitis Pigmentosa and Cone–Rod Degeneration,” Invest. Ophthalmol. Visual Sci. 28, 140 (1987).

Fishman, G. A.

D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).

G. A. Fishman, “Electroretinography and Inherited Macular Dystrophies,” Retina 5, 172 (1985).
[CrossRef] [PubMed]

K. R. Alexander, G. A. Fishman, “Prolonged Rod Dark Adaptation in Retinitis Pigmentosa,” Br. J. Ophthalmol. 68, 561 (1984).
[CrossRef] [PubMed]

G. A. Fishman, G. Buckman, T. van Every, “Fundus Flavimaculatus: a Clinical Classification,” Doc. Opthalmol. 13, 213 (1977).

G. A. Fishman, “Progressive Human Cone-Rod Dysfunction (Dystrophy),” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 716 (1976).

Fishman, M.

A. E. Krill, A. F. Deutman, M. Fishman, “The Cone Degenerations,” Doc. Ophthalmol. 35, 1 (1973).
[CrossRef] [PubMed]

Geisler, W. S.

W. S. Geisler, “Mechanisms of Visual Sensitivity: Backgrounds and Early Dark Adaptation,” Vision Res. 12, 1423 (1983).
[CrossRef]

Goodman, G.

G. Goodman, H. Ripps, I. M. Siegel, “Cone Dysfunction Syndromes,” Arch. Ophthalmol. 70, 214 (1963).
[CrossRef] [PubMed]

Gouras, P.

E. L. Berson, P. Gouras, R. D. Gunkel, “Progressive Cone–Rod Degeneration,” Arch. Ophthalmol. 80, 68 (1968).
[CrossRef] [PubMed]

Green, D. G.

D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
[CrossRef] [PubMed]

Greenstein, V. C.

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

D. C. Hood, V. C. Greenstein, “An Approach to Testing Alternative Hypotheses of Changes in Visual Sensitivity Due to Retinal Disease,” Invest. Ophthalmol. Visual Sci. 23, 96 (1982).

D. C. Hood, V. C. Greenstein, “Increment Threshold (tvi) Data and the Site of Disease Action,” in Technical Digest of Topical Meeting on Noninvasive Assessment of the Visual System (Optical Society of America, Washington, DC, 1988), Vol. 3, p. 2.

Gunkel, R. D.

E. L. Berson, P. Gouras, R. D. Gunkel, “Progressive Cone–Rod Degeneration,” Arch. Ophthalmol. 80, 68 (1968).
[CrossRef] [PubMed]

Guth, S. L.

Harrison, J.

R. S. L. Young, J. Price, J. Harrison, “Psychophysical Study of Rod Adaptation in Patients with Congenital Stationary Night Blindness,” Clin. Vis. Sci. 1, 137 (1986).

Hecht, S.

S. Hecht, “Rods, Cones, and the Chemical Basis of Vision,” Physiol. Rev. 17, 239 (1937).

Highman, V. N.

V. N. Highman, R. A. Weale, “Rhodopsin Density and Visual Threshold in Retinitis Pigmentosa,” Am. J. Ophthalmol. 75, 822 (1973).
[PubMed]

Hood, D. C.

D. C. Hood, V. C. Greenstein, “An Approach to Testing Alternative Hypotheses of Changes in Visual Sensitivity Due to Retinal Disease,” Invest. Ophthalmol. Visual Sci. 23, 96 (1982).

D. C. Hood, V. C. Greenstein, “Increment Threshold (tvi) Data and the Site of Disease Action,” in Technical Digest of Topical Meeting on Noninvasive Assessment of the Visual System (Optical Society of America, Washington, DC, 1988), Vol. 3, p. 2.

Krill, A. E.

A. E. Krill, A. F. Deutman, M. Fishman, “The Cone Degenerations,” Doc. Ophthalmol. 35, 1 (1973).
[CrossRef] [PubMed]

A. E. Krill, “Cone Degenerations,” in Krill’s Hereditary Retinal and Choroidal Diseases. Vol. II. Clinical Characteristics, A.E. Krill, Ed. (Harper & Row, Hagerstown, MD, 1977), p. 421.

Marcus, S.

Massof, R. W.

Noble, K. G.

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

Nordby, K.

B. Stabell, K. Nordby, U. Stabell, “Light-Adaptation of the Human Rod System,” Clin. Vis. Sci. 2, 83 (1987).

Nunn, B. J.

D. A. Baylor, B. J. Nunn, J. L. Schnapf, “The Photocurrent, Noise and Spectral Sensitivity of Rods of the Monkey, Macaca Fascicularis,” J. Physiol. (London) 357, 575 (1984).

Pepperberg, D. R.

D. W. Yates, D. J. Derlacki, D. R. Pepperberg, G. A. Fishman, K. R. Alexander, “Rod Increment Threshold Function in Cone–Rod Dystrophy,” Invest. Ophthalmol. Visual Sci. (ARVO Suppl.) 28, 112 (1987).

H. Ripps, D. R. Pepperberg, “Photoreceptor Processes in Visual Adaptation,” Neurosci. Res. Suppl. 6, S87 (1987).

J. W. Clack, D. R. Pepperberg, “Desensitization of Skate Photoreceptors by Bleaching and Background Light,” J. Gen. Physiol. 80, 863 (1982).
[CrossRef] [PubMed]

Perlman, I.

I. Perlman, E. Auerbach, “The Relationship Between Visual Sensitivity and Rhodopsin Density in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 20, 758 (1981).

Price, J.

R. S. L. Young, J. Price, J. Harrison, “Psychophysical Study of Rod Adaptation in Patients with Congenital Stationary Night Blindness,” Clin. Vis. Sci. 1, 137 (1986).

Ripps, H.

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

H. Ripps, D. R. Pepperberg, “Photoreceptor Processes in Visual Adaptation,” Neurosci. Res. Suppl. 6, S87 (1987).

H. Ripps, K. P. Brin, R. A. Weale, “Rhodopsin and Visual Threshold in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 17, 735 (1978).

D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
[CrossRef] [PubMed]

G. Goodman, H. Ripps, I. M. Siegel, “Cone Dysfunction Syndromes,” Arch. Ophthalmol. 70, 214 (1963).
[CrossRef] [PubMed]

Rushton, W. A. H.

C. B. Blakemore, W. A. H. Rushton, “Dark Adaptation and Increment Threshold in a Rod Monochromat,” J. Physiol. (London) 181, 612 (1965).

Sandberg, M. A.

M. A. Sandberg, E. L. Berson, “Blue and Green Cone Mechanisms in Retinitis Pigmentosa,” Invest. Ophthalmol. Visual Sci. 16, 149 (1977).

Schnapf, J. L.

D. A. Baylor, B. J. Nunn, J. L. Schnapf, “The Photocurrent, Noise and Spectral Sensitivity of Rods of the Monkey, Macaca Fascicularis,” J. Physiol. (London) 357, 575 (1984).

Shapley, R.

R. Shapley, C. Enroth-Cugell, “Visual Adaptation and Retinal Gain Controls,” Prog. Retinal Res. 3, 263 (1984).
[CrossRef]

Siegel, I. M.

H. Ripps, K. G. Noble, V. C. Greenstein, I. M. Siegel, R. E. Carr, “Progressive Cone Dystrophy,” Ophthalmology 94, 1401 (1987).
[PubMed]

R. E. Carr, I. M. Siegel, “Cone Dysfunctions in Man,” Trans. Am. Acad. Ophthalmol. Otolaryngol. 81, 653 (1976).

D. G. Green, J. E. Dowling, I. M. Siegel, H. Ripps, “Retinal Mechanisms of Visual Adaptation in the Skate,” J. Gen. Physiol. 65, 483 (1975).
[CrossRef] [PubMed]

G. Goodman, H. Ripps, I. M. Siegel, “Cone Dysfunction Syndromes,” Arch. Ophthalmol. 70, 214 (1963).
[CrossRef] [PubMed]

Stabell, B.

B. Stabell, K. Nordby, U. Stabell, “Light-Adaptation of the Human Rod System,” Clin. Vis. Sci. 2, 83 (1987).

Stabell, U.

B. Stabell, K. Nordby, U. Stabell, “Light-Adaptation of the Human Rod System,” Clin. Vis. Sci. 2, 83 (1987).

Stangos, N.

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The ERG responses of patients 8, 10, and 11 to a single short wavelength flash were nondetectable. However, in patients 8 and 10, markedly reduced but detectable responses were apparent to a 10-Hz short wavelength flickering stimulus when several responses were averaged. Furthermore, the responses of these two patients to a white flash was appreciably greater in darkadapted (vs light-adapted) conditions, strongly suggesting that the dark-adapted response was rod-mediated. Patient 11 was somewhat atypical, in that her response to even a bright white flash was <50 μV. Nevertheless, this patient did not indicate night blindness as her initial or major complaint and did not manifest any clinical evidence of peripheral retinal degeneration. The visual acuity of 20/30 in this patient is consistent with the diagnosis of CRD, as was her impaired color vision.

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Figures (4)

Fig. 1
Fig. 1

ITFs of a normal subject (number 14) to a 104-min TS (circles) and a 7-min TS (triangles). Each data point represents the average of three trials. Solid curves plot the least-squares fit of Eq. (1) to each set of measured thresholds.

Fig.2
Fig.2

ITFs of CRD patients 8–11 [(a)–(d), respectively]. Solid curves plot the least-squares solution of Eq. (1). The shaded area indicates the range of thresholds measured in normal subjects.

Fig. 3
Fig. 3

Parametric representation of ITF data. (a)–(c) Values of logK, logc, and n resulting from least-squares fit of Eq. (1) to the measured thresholds. (d) Absolute thresholds. Data within each panel show results obtained from normals, 7-min TS (left column); normals, 104-min TS (center column); and CRD patients, 104-min TS (right column). Numbers in the right-hand columns identify CRD patients. Pairs of curves above the parametric data illustrate the dependence of the shape and position of the ITF on variation of a single parameter. Relative to a reference (solid curve of each pair), the dashed curves show the change due to (a) an increase in logK; (b) an increase in logc; and (c) a decrease in n.

Fig. 4
Fig. 4

Plot of logK vs logc (104-min TS) for CRD patients and normals (filled and open circles, respectively). Numbers identify CRD patients 8–11. Dashed lines identify the normal ranges of logc and logK. Solid lines enclose a region describing the variation of the ITFs of normals with change in the parameter α. See text for further details.

Tables (1)

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Table I Electroretinographic Data Obtained from CRD Patients

Equations (5)

Equations on this page are rendered with MathJax. Learn more.

log I t =  log K +  log ( I b n + c ) .
I b = I b / α ; I t = I t / α ; α > 1.
I t = α 1 n K I b n + α K c = K I b n + K c ,
Δ log K = log K log K ; Δ log c = log c log c .
Δ log K = [ ( 1 n ) / n ] Δ log c .

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