Advertisement

Cone and rod ERGs in degenerations of central retina

  • G. Niemeyer
  • E. Demant
Article

Abstract

Sixty-four patients with retinal degenerations at the posterior pole were reviewed and their ERGs analysed. On the basis of symptoms, visual acuity, ophthalmoscopy and cone/rod ERG the patients were divided into five diagnostic groups: Stargardt's disease, fundus flavimaculatus, cone degeneration, dominant drusen and central retinitis pigmentosa (RP). Stargardt's disease and fundus flavimaculatus show low-normal or subnormal cone b-wave amplitudes, often with prolonged peak times; the rod ERG is rarely abnormal. Cone degeneration presents with reduced visual acuity, photophobia, nystagmus and minimal fundus changes. The ERG shows severely reduced cone b-waves and increased peak times; the rod b-waves are in the low-normal or subnormal range. Dominantly inherited drusen, included for comparison, revealed little change in the ERG in spite of widespread ophthalmoscopic changes: b-wave amplitudes fall mostly in the low-normal range, and their peak times may be prolonged. RP of the central type reveals considerable variability in all clinical aspects, but the cone and rod ERGs are consistently greatly reduced, showing markedly increased peak times of the cone b-waves. Recording of the Ganzfeld ERG with cone/rod separation thus proves useful in differentiating degenerations of the central retina.

Keywords

Visual Acuity Peak Time Retinitis Pigmentosa Retinal Degeneration Central Retina 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Babel J, Stangos N, Korol S, Spiritus M (1977) Ocular electrophysiology. Georg Thieme, StuttgartGoogle Scholar
  2. Berson EL, Gouras P, Gunkel RD (1968a) Rod responses in retinitis pigmentosa, dominantly inherited. Arch Opthalmol 80: 58–67Google Scholar
  3. Berson EL, Gouras P, Gunkel RD (1968b) Progressive cone-rod degeneration. Arch Ophthalmol 80: 68–76Google Scholar
  4. Berson EL, Gouras P, Gunkel RD (1968c) Progressive cone degeneration, dominantly inherited. Arch Ophthalmol 80: 77–87Google Scholar
  5. Carr RE, Siegel IM (1982) Visual electrodiagnostic testing: a practical guide for the clinician. Williams and Wilkins, BaltimoreGoogle Scholar
  6. Deutman A (1971) The hereditary dystrophies of the posterior pole of the eye. Van Gorcum and Co., Assen, pp 35–41, 172–180Google Scholar
  7. Deutman A (1974) Macular dystrophies. In: Goldberg MF (ed) Genetic and metabolic eye disease. Little, Brown and Co., Boston, pp 367–429Google Scholar
  8. Dušek J. Streicher T, Schmidt R (1982) Hereditäre Drusen der Bruch'schen Membran. II. Untersuchung von Semidünnschnitten und elektronenmikroskopischen Ergebnissen. Klin Monatsbl Augenheilkd 18: 79–83Google Scholar
  9. Farkas T, Sylvester V, Archer D (1971) The ultrastructure of drusen. Am J Opthalmol 71: 1196–1205Google Scholar
  10. Fishman GA (1976a) Fundus flavimaculatus: a clinical classification. Arch Ophthalmol 94: 2061–2067PubMedGoogle Scholar
  11. Fishman GA (1976b) Progressive human cone-rod dysfunction (dystrophy). Trans Am Acad Ophthalmol Otolaryngol 81: 716–724Google Scholar
  12. Forni S, Babel J (1962) Etude clinique et histologique de la malattia leventinese. Opthalmologica 143: 313–321Google Scholar
  13. Franceschetti A, François J (1965) Fundus flavimaculatus. Arch Ophthalmol (Paris) 25: 505–530Google Scholar
  14. Franceschetti A, François J, Babel J (1974) Chorioretinal heredodegenerations. Charles C. Thomas, Springfield, Illinois, pp 275–289Google Scholar
  15. Goodman G, Ripps H, Siegel IM (1966) Progressive cone degeneration. In: Burian HM, Jacobson JH (eds) Clinical electroretinography, Proc. IIIrd ISCERG Symp. Pergamon Press, New York, p 363Google Scholar
  16. Gouras P (1970) Electroretinography: some basic principles. Invest Ophthalmol 9: 557–569PubMedGoogle Scholar
  17. Gurewitsch K, Niemeyer G (1982) Rod/cone separation by electroretinography, fundus changes and visual fields in retinitis pigmentosa. In: Niemeyer G, Huber CH, (eds) XIXth ISCEV Symposium. Doc Ophthalmol Proc. Series 31: 145Google Scholar
  18. Hadden OB, Gass JDM (1976) Fundus flavimaculatus and Stargardt's disease. Am J Ophthalmol 82: 527–539PubMedGoogle Scholar
  19. Hatt M, Niemeyer G (1976) Electroetinographie bei Morbus Behçet. Graefe's Arch Clin Exp Ophthalmol 198: 113–120Google Scholar
  20. Hess F, Niemeyer G (1983) EOG changes in dominantly inherited drusen (Malattia leventinese). XXth ISCEV Symposium, Iowa City, Oct 1982, Doc Ophthalmol Proc Series, in pressGoogle Scholar
  21. Hittner HM, Murphree AL, Garcia CA, Justice J, Chokshi DB (1975) Dominant cone-rod dystrophy. Doc Ophthalmol 39: 29–52CrossRefPubMedGoogle Scholar
  22. Irvine AR, Wergeland FL (1972) Stargardt's hereditary progressive macular degeneration. Br J Ophthalmol 56: 817–826PubMedGoogle Scholar
  23. Jaeger W, Alexandridis E, Kraus E, Tenner A, Käfer O (1975) Hereditäre Makuladegenerationen. DOG 1973; Bergmann, Berlin, pp 695–735Google Scholar
  24. Jaeger W, Krastel H, Blankenagel A (1979) Zur Symptomatik der Zapfendystrophie. Ber Zusammenkunft Dtsch Ophthalmol Ges 76: 397–408Google Scholar
  25. Klien BA, Krill AE (1967) Fundus flavimaculatus: clinical functional and histopathological observations. Am J Ophthalmol 64: 3–23PubMedGoogle Scholar
  26. Krill AE (1977) Hereditary retinal and choroidal diseases, vol 2. Clinical characteristics. Harper and Row, HagerstownGoogle Scholar
  27. Krill AE, Deutman AF (1972) Dominant macular degenerations. The cone dystrophies. Am J Ophthalmol 73: 352–369PubMedGoogle Scholar
  28. Krill AE, Deutman AF, Fishman M (1973) The cone degenerations. Doc Ophthalmol 35: 1–80CrossRefPubMedGoogle Scholar
  29. Krill AE, Klien BA (1965) Flecked retina syndrome. Arch Ophthalmol 74: 496–508PubMedGoogle Scholar
  30. Niemeyer G (1969) Electroretinographie bei Maculadegenerationen. Graefe's Arch Clin Exp Ophthalmol 177: 39–51Google Scholar
  31. Niemeyer G (1976) Stäbchen- und Zapfenaktivität im klinischen Elektroretinogramm. Ophthalmologica 172: 175–180PubMedGoogle Scholar
  32. Niemeyer G (1978) Rod- and cone-function in Malattia leventinese and in retinitis pigmentosa. Doc Ophthalmol Proc Series 17: 337–344Google Scholar
  33. Niemeyer G (1979) Information von der Netzhaut durch Elektroretinographie. Graefe's Arch Clin Exp Ophthalmol 211: 129–137Google Scholar
  34. Niemeyer G, Gurewitsch K (1982) Variabilität von Visus, Dunkel-adaptation und Elektroretinogramm bei Retinitis pigmentosa. Klin Monatsbl Augenheilkd 180: 401–404PubMedGoogle Scholar
  35. Noble KG, Carr RE (1979) Stargardt's disease and fundus flavimaculatus. Arch Ophthalmol 97: 1281–1285PubMedGoogle Scholar
  36. Pearlman JT, Owen WG, Brounley DW, Sheppard JJ (1974) Cone dystrophy with dominant inheritance. Part 1: Clinical case histories. Part 2: Special colour vision tests. Doc Ophthalmol Proc Series, XIth ISCERG Symp, Dr W Junk Publishers, The Hague, pp 113–122, 123–125Google Scholar
  37. Pinckers A, Deutman AF (1977) Peripheral cone disease. Ophthalmologica 174: 145–150PubMedGoogle Scholar
  38. Scarpatetti A, Forni S, Niemeyer G (1978) Die Netzhautfunktion bei Malattia leventinese (dominant drusen). Klin Monatsbl Augenheilkd 172: 590–597PubMedGoogle Scholar
  39. Skalka HW (1978): Electrophysiological variability in fundus flavimaculatus. In: Tazawa Y (ed) Proc. 16th ISCEV Symp. Jpn J Ophthalmol, Tokyo, Japan 75–79Google Scholar
  40. Streicher T, Schmidt K, Dusek J (1982) Hereditäre Drusen der Bruch'schen Membran. I. Klinische und lichtmikroskopische Beobachtungen. Klin Monatsbl Augenheilkd 181: 27–31PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1983

Authors and Affiliations

  • G. Niemeyer
    • 1
  • E. Demant
    • 1
  1. 1.Department of OphthalmologyUniversitätsspitalZürichSwitzerland

Personalised recommendations