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.
Similar content being viewed by others
References
Babel J, Stangos N, Korol S, Spiritus M (1977) Ocular electrophysiology. Georg Thieme, Stuttgart
Berson EL, Gouras P, Gunkel RD (1968a) Rod responses in retinitis pigmentosa, dominantly inherited. Arch Opthalmol 80: 58–67
Berson EL, Gouras P, Gunkel RD (1968b) Progressive cone-rod degeneration. Arch Ophthalmol 80: 68–76
Berson EL, Gouras P, Gunkel RD (1968c) Progressive cone degeneration, dominantly inherited. Arch Ophthalmol 80: 77–87
Carr RE, Siegel IM (1982) Visual electrodiagnostic testing: a practical guide for the clinician. Williams and Wilkins, Baltimore
Deutman A (1971) The hereditary dystrophies of the posterior pole of the eye. Van Gorcum and Co., Assen, pp 35–41, 172–180
Deutman A (1974) Macular dystrophies. In: Goldberg MF (ed) Genetic and metabolic eye disease. Little, Brown and Co., Boston, pp 367–429
Dušek J. Streicher T, Schmidt R (1982) Hereditäre Drusen der Bruch'schen Membran. II. Untersuchung von Semidünnschnitten und elektronenmikroskopischen Ergebnissen. Klin Monatsbl Augenheilkd 18: 79–83
Farkas T, Sylvester V, Archer D (1971) The ultrastructure of drusen. Am J Opthalmol 71: 1196–1205
Fishman GA (1976a) Fundus flavimaculatus: a clinical classification. Arch Ophthalmol 94: 2061–2067
Fishman GA (1976b) Progressive human cone-rod dysfunction (dystrophy). Trans Am Acad Ophthalmol Otolaryngol 81: 716–724
Forni S, Babel J (1962) Etude clinique et histologique de la malattia leventinese. Opthalmologica 143: 313–321
Franceschetti A, François J (1965) Fundus flavimaculatus. Arch Ophthalmol (Paris) 25: 505–530
Franceschetti A, François J, Babel J (1974) Chorioretinal heredodegenerations. Charles C. Thomas, Springfield, Illinois, pp 275–289
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 363
Gouras P (1970) Electroretinography: some basic principles. Invest Ophthalmol 9: 557–569
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: 145
Hadden OB, Gass JDM (1976) Fundus flavimaculatus and Stargardt's disease. Am J Ophthalmol 82: 527–539
Hatt M, Niemeyer G (1976) Electroetinographie bei Morbus Behçet. Graefe's Arch Clin Exp Ophthalmol 198: 113–120
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 press
Hittner HM, Murphree AL, Garcia CA, Justice J, Chokshi DB (1975) Dominant cone-rod dystrophy. Doc Ophthalmol 39: 29–52
Irvine AR, Wergeland FL (1972) Stargardt's hereditary progressive macular degeneration. Br J Ophthalmol 56: 817–826
Jaeger W, Alexandridis E, Kraus E, Tenner A, Käfer O (1975) Hereditäre Makuladegenerationen. DOG 1973; Bergmann, Berlin, pp 695–735
Jaeger W, Krastel H, Blankenagel A (1979) Zur Symptomatik der Zapfendystrophie. Ber Zusammenkunft Dtsch Ophthalmol Ges 76: 397–408
Klien BA, Krill AE (1967) Fundus flavimaculatus: clinical functional and histopathological observations. Am J Ophthalmol 64: 3–23
Krill AE (1977) Hereditary retinal and choroidal diseases, vol 2. Clinical characteristics. Harper and Row, Hagerstown
Krill AE, Deutman AF (1972) Dominant macular degenerations. The cone dystrophies. Am J Ophthalmol 73: 352–369
Krill AE, Deutman AF, Fishman M (1973) The cone degenerations. Doc Ophthalmol 35: 1–80
Krill AE, Klien BA (1965) Flecked retina syndrome. Arch Ophthalmol 74: 496–508
Niemeyer G (1969) Electroretinographie bei Maculadegenerationen. Graefe's Arch Clin Exp Ophthalmol 177: 39–51
Niemeyer G (1976) Stäbchen- und Zapfenaktivität im klinischen Elektroretinogramm. Ophthalmologica 172: 175–180
Niemeyer G (1978) Rod- and cone-function in Malattia leventinese and in retinitis pigmentosa. Doc Ophthalmol Proc Series 17: 337–344
Niemeyer G (1979) Information von der Netzhaut durch Elektroretinographie. Graefe's Arch Clin Exp Ophthalmol 211: 129–137
Niemeyer G, Gurewitsch K (1982) Variabilität von Visus, Dunkel-adaptation und Elektroretinogramm bei Retinitis pigmentosa. Klin Monatsbl Augenheilkd 180: 401–404
Noble KG, Carr RE (1979) Stargardt's disease and fundus flavimaculatus. Arch Ophthalmol 97: 1281–1285
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, 123–125
Pinckers A, Deutman AF (1977) Peripheral cone disease. Ophthalmologica 174: 145–150
Scarpatetti A, Forni S, Niemeyer G (1978) Die Netzhautfunktion bei Malattia leventinese (dominant drusen). Klin Monatsbl Augenheilkd 172: 590–597
Skalka HW (1978): Electrophysiological variability in fundus flavimaculatus. In: Tazawa Y (ed) Proc. 16th ISCEV Symp. Jpn J Ophthalmol, Tokyo, Japan 75–79
Streicher T, Schmidt K, Dusek J (1982) Hereditäre Drusen der Bruch'schen Membran. I. Klinische und lichtmikroskopische Beobachtungen. Klin Monatsbl Augenheilkd 181: 27–31
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Niemeyer, G., Demant, E. Cone and rod ERGs in degenerations of central retina. Graefe's Arch Clin Exp Ophthalmol 220, 201–208 (1983). https://doi.org/10.1007/BF02308075
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF02308075