Skip to main content
SpringerLink
Log in

Color vision in Stargardt's disease

  • Original Papers
  • Published:
International Ophthalmology Aims and scope Submit manuscript

Abstract

The color vision of nine patients aged from 13 to 52 years with Stargardt's disease was studied with the following tests: Standard Pseudoisochromatic Plates part 2 (SSP2), Farnsworth-Munsell 100 hue test (FM100), Nagel (red-green) anomaloscope and Besançon (blue) anomalometer. At the beginning of the disease, a very slight defect in red-green color vision could be demonstrated. Later, a distinct acquired red (pseudo-protanomalous) defect in the Nagel anomaloscope and an abnormal error score in the FM100 test were observed. In advanced stages, the red defect became stronger (scotopization) and the FM100 test showed a red-green axis. In the course of the disease, a blue defect with the SPP2 plates and with the Besançon anomalometer could also be found. The visual acuities of the patients had a significant correlation with the matching ranges of the Rayleigh equation and the Moreland equation. The duration of the disease did not show any correlation with the color vision tests.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Amalric P, Kment H, Remky H. Fundus flavimaculatus. Klin Monatsbl Augenheilkd 1967; 150: 625–36.

    Google Scholar 

  2. Anmarkrud N. Fundus fluorescein angiography in fundus flavimaculatus and Stargardts disease. Acta Ophthalmol 1979; 57: 172–82.

    Google Scholar 

  3. Birch J. Diagnosis of defective colour vision using the Nagel anomaloscope. In: Verriest G, editor. ‘Colour vision deficiencies VI. Doc Ophthalmol Proc Ser 33. The Hague: Dr W Junk Publishers, 1982: 231–5.

    Google Scholar 

  4. Birch J, Chisholm IA, Kinnear P, Pinckers AJLG, Pokorny J, Smith VC, Verriest G. Clinical testing methods. In: Pokorny J, Smith VC, Verriest G, Pinckers AJLG, editors. ‘Congenital and acquired color vision defects’. New York: Grune & Stratton, 1979: 83–135.

    Google Scholar 

  5. Blacharski PA. Fundus flavimaculatus. In: Newsome DA, editor. ‘Retinal dystrophies and degenerations. New York: Raven Press, 1988: 135–59.

    Google Scholar 

  6. Carr RE. Fundus flavimaculatus. Arch Ophthalmol 1965; 74: 163–8.

    Google Scholar 

  7. Cibis GW, Morey M, Harris DJ. Dominantly inherited macular dystrophy with flecks (Stargardt). Arch Ophthalmol 1980; 98: 1785–9.

    Google Scholar 

  8. Deutman AF. The hereditary dystrophies of the posterior pole of the eye. Assen, The Netherlands: Van Gorcum & Comp. N.V., 1971: 100–71.

    Google Scholar 

  9. Doka DS, Fishman GA, Anderson RJ. Refractive errors in patients with fundus flavimaculatus. Br J Ophthalmol 1982; 66: 227–9.

    Google Scholar 

  10. Eagle RC Jr, Lucier AC, Bernardino VB Jr, Yanoff M. Retinal pigment epithelial abnormalities in fundus flavimaculatus. Ophthalmology 1980; 87: 1189–1200.

    Google Scholar 

  11. Ernest JT, Krill AE. Fluorescein studies in fundus flavimaculatus and drusen. Am J Ophthalmol 1966; 62: 1–6.

    Google Scholar 

  12. Farnsworth D. The Farnsworth-Munsell 100-hue test. Baltimore: Munsell Color Company, 1957.

    Google Scholar 

  13. Fishman GA. Electrophysiology and inherited retinal disorders. Doc Ophthalmol 1985; 60: 107–19.

    Google Scholar 

  14. Fishman GA, Farber M, Patel BS, Derlacki DJ. Visual acuity loss in patients with Stargardt's macular dystrophy. Ophthalmology 1987; 94: 809–14.

    Google Scholar 

  15. Fonda G, Gardner LR, Characteristics and low vision corrections in Stargardt's disease. Ophthalmology 1985; 92: 1084–91.

    Google Scholar 

  16. François J, Verriest G, De Rouck A, Humblet M. Dégénérescence maculaire juvénile avec atteinte prédominante de la vision photopique. Ophthalmologica 1956; 131: 393–402.

    Google Scholar 

  17. Grützner P. Typische erworbene Farbensinnstörungen bei heredodegenerativen Maculaleiden. Graefe's Arch Ophthalmol 1961; 163: 99–116.

    Google Scholar 

  18. Hammerstein W, Leide E. Die Bedeutung der Fluoreszenzangiographie für die Diagnose der Stargardtschen Makuladegeneration. Klin Monatsbl Augenheilkd 1981; 178: 20–3.

    Google Scholar 

  19. Hermés D, Roth A, Borot N. The two equation method. II. Results in retinal and optic nerve disorders. In: Drum B, Verriest G, editors. Colour vision deficiencies IX. Doc Ophthalmol Proc Ser 52. Dordrecht: Kluwer Academic Publishers, 1989: 325–37.

    Google Scholar 

  20. Ichikawa H, Hukami K, Tanabe S. Standard Pseudoisochromatic Plates part 2. Tokyo: Igaku-Shoin Ltd, 1983.

    Google Scholar 

  21. Ichikawa K, Ichikawa H, Tanabe S. Detection of acquired color vision defects by Standard Pseudoisochromatic Plates Part 2. In: Verriest G, editor. Colour vision deficiencies VIII. Doc Ophthalmol Proc Ser 46. Dordrecht: Martinus Nijhoff/Dr W Junk Publishers, 1987: 133–40.

    Google Scholar 

  22. Imaizumi K, Takahashi R, Mita K, Hoshi H. Clinical and electrophysiological findings of a case of fundus flavimaculatus. In: Alfieri R, Solé P, editors. XIIth ISCERG Symposium. Doc Ophthalmol Proc Ser, Vol 10. The Hague: Dr W Junk Publishers, 1976: 69–75.

    Google Scholar 

  23. Irvine AR, Wergeland FL, Jr. Stargardt's hereditary progressive macular degeneration. Br J Ophthalmol 1972; 56: 817–26.

    Google Scholar 

  24. Kinnear PR. Proposals for scoring and assessing the 100-hue test. Vision Res 1970; 10: 423–33.

    Google Scholar 

  25. Klien BA, Krill AE. Fundus flavimaculatus. Am J Ophthalmol 1967; 64: 3–23.

    Google Scholar 

  26. Lachapelle P, Little JM, Roy MR. The electroretinogram in Stargardt's disease and fundus flavimaculatus. Doc Ophthalmol 1990; 73: 395–404.

    Google Scholar 

  27. Lopez PF, Maumenee IH, De La Cruz Z, Green WR. Autosomal-dominant fundus flavimaculatus. Ophthalmology 1990; 97: 798–809.

    Google Scholar 

  28. Moloney JB, Mooney DJ, O'Connor MA. Retinal function in Stargardt's disease and fundus flavimaculatus. Am J Ophthalmol 1983; 96: 57–65.

    Google Scholar 

  29. Morse PH, Smith VC, Pokorny J, Burch JV. Fundus flavimaculatus with cystoid macular changes and abnormal Stiles-Crawford effect. Am J Ophthalmol 1981; 91: 190–6.

    Google Scholar 

  30. Newell FW, Krill AE, Farkas TG. Drusen and fundus flavimaculatus: clinical, functional, and histologic characteristics. Trans Am Acad Opthalmol Otolaryngol 1972; 76: 88–100.

    Google Scholar 

  31. Niemeyer G, Demant E. Cone and rod ERGs in degenerations of central retina. Graefe's Arch Clin Exp Ophthalmol 1983; 220: 201–8.

    Google Scholar 

  32. Noble KG, Carr RE. Stargardt's disease and fundus flavimaculatus. Arch Ophthalmol 1979; 97: 1281–5.

    Google Scholar 

  33. Pinckers A. La maladie de Stargardt. Ann Oculist 1971; 204: 1331–46.

    Google Scholar 

  34. Roth A. Metameric matches relevant for assessment of color vision II. Practical aspects. In: Verriest G, editor. Colour vision deficiencies VII. Doc Ophthalmol Proc Ser 39. The Hague: Dr W Junk Publishers, 1984: 95–109.

    Google Scholar 

  35. Roth A, Pelizzone M, Hermés D. The two equation method. I. Results in normal color vision. In: Drum B, Verriest G, editors. Colour vision deficiencies IX. Doc Ophthalmol Proc Ser 52. Dordrecht: Kluwer Academic Publishers, 1989: 317–23.

    Google Scholar 

  36. Smith VC, Pokorny J, Pass AS. Color-axis determination on the Farnsworth-Munsell 100-hue test. Am J Ophthalmol 1985; 100: 176–82.

    Google Scholar 

  37. Stargardt K. Über familiäre, progressive Degeneration in der Maculagegend des Auges. Graefe's Arch Ophthalmol 1909; 71: 534–50.

    Google Scholar 

  38. Verriest G, Van Lathem J, Uvijls A. A new assessment of the normal ranges of the Farnsworth-Munsell 100-hue test scores. Am J Ophthalmol 1982; 93: 635–42.

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Ophthalmology, University Hospital of Kuopio, P.O. Box 1777, 70211, Kuopio, Finland

    Maija Mäntyjärvi & Kaija Tuppurainen

Authors
  1. Maija Mäntyjärvi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Kaija Tuppurainen
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mäntyjärvi, M., Tuppurainen, K. Color vision in Stargardt's disease. Int Ophthalmol 16, 423–428 (1992). https://doi.org/10.1007/BF00918432

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00918432

Key words

Search

Navigation