Skip to main content
PMC home page
Transactions of the American Ophthalmological Society logoLink to Transactions of the American Ophthalmological Society
. 2001;99:171–176.

The negative ERG is not synonymous with nightblindness.

G W Cibis 1, K M Fitzgerald 1
PMCID: PMC1359007  PMID: 11797304

Abstract

PURPOSE: To provide electroretinographic differentiation between 4 genetically distinct conditions associated with a negative. Schubert Bornschein type electroretinogram (ERG): Complete congenital stationary night blindness (cCSNB), incomplete CSNB (incCSNB), Duchenne muscular dystrophy, and a family with an autosomal dominantly inherited negative ERG. METHODS: ERGs were recorded in all subjects according to the ISCEV standards. Additionally, a long-duration flash was used under photopic testing conditions to separate depolarizing (ON) and hyperpolarizing (OFF) bipolar cell contributions. Dark adaptometry was obtained in cooperative adult subjects. RESULTS: We were unable to differentiate between these 4 genetically distinct conditions using the scotopic ERG response to the bright white flash only. The photopic, cone-derived ERG to both short- and long-duration flashes was more informative in making distinctions between these 4 disorders and understanding the possible mechanisms behind the abnormal ERG. CONCLUSION: None of these disorders are progressive or a result of abnormal photoreceptor phototransduction. We suggest that they each represent a signal transmission error at the photoreceptor to depolarizing bipolar cell synapse that affects both rod and cone output. We propose that vision is spared in the latter 2 conditions because of timing errors in transmission as opposed to a complete signaling block, as seen in cCSNB.

Full Text

The Full Text of this article is available as a PDF (102.9 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bech-Hansen N. T., Naylor M. J., Maybaum T. A., Pearce W. G., Koop B., Fishman G. A., Mets M., Musarella M. A., Boycott K. M. Loss-of-function mutations in a calcium-channel alpha1-subunit gene in Xp11.23 cause incomplete X-linked congenital stationary night blindness. Nat Genet. 1998 Jul;19(3):264–267. doi: 10.1038/947. [DOI] [PubMed] [Google Scholar]
  2. Bech-Hansen N. T., Naylor M. J., Maybaum T. A., Sparkes R. L., Koop B., Birch D. G., Bergen A. A., Prinsen C. F., Polomeno R. C., Gal A. Mutations in NYX, encoding the leucine-rich proteoglycan nyctalopin, cause X-linked complete congenital stationary night blindness. Nat Genet. 2000 Nov;26(3):319–323. doi: 10.1038/81619. [DOI] [PubMed] [Google Scholar]
  3. Cibis G. W., Burian H. M., Blodi F. C. Electroretinogram changes in acute quinine poisoning. Arch Ophthalmol. 1973 Oct;90(4):307–309. doi: 10.1001/archopht.1973.01000050309014. [DOI] [PubMed] [Google Scholar]
  4. Cibis G. W., Fitzgerald K. M., Harris D. J., Rothberg P. G., Rupani M. The effects of dystrophin gene mutations on the ERG in mice and humans. Invest Ophthalmol Vis Sci. 1993 Dec;34(13):3646–3652. [PubMed] [Google Scholar]
  5. Fitzgerald K. M., Cibis G. W., Giambrone S. A., Harris D. J. Retinal signal transmission in Duchenne muscular dystrophy: evidence for dysfunction in the photoreceptor/depolarizing bipolar cell pathway. J Clin Invest. 1994 Jun;93(6):2425–2430. doi: 10.1172/JCI117250. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Fitzgerald K. M., Hashimoto T., Hug T. E., Cibis G. W., Harris D. J. Autosomal dominant inheritance of a negative electroretinogram phenotype in three generations. Am J Ophthalmol. 2001 Apr;131(4):495–502. doi: 10.1016/s0002-9394(00)00849-7. [DOI] [PubMed] [Google Scholar]
  7. Green D. G., Kapousta-Bruneau N. V. A dissection of the electroretinogram from the isolated rat retina with microelectrodes and drugs. Vis Neurosci. 1999 Jul-Aug;16(4):727–741. doi: 10.1017/s0952523899164125. [DOI] [PubMed] [Google Scholar]
  8. Gurevich L., Slaughter M. M. Comparison of the waveforms of the ON bipolar neuron and the b-wave of the electroretinogram. Vision Res. 1993 Dec;33(17):2431–2435. doi: 10.1016/0042-6989(93)90122-d. [DOI] [PubMed] [Google Scholar]
  9. Jensen H., Warburg M., Sjö O., Schwartz M. Duchenne muscular dystrophy: negative electroretinograms and normal dark adaptation. Reappraisal of assignment of X linked incomplete congenital stationary night blindness. J Med Genet. 1995 May;32(5):348–351. doi: 10.1136/jmg.32.5.348. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Karwoski C. J., Xu X. Current source-density analysis of light-evoked field potentials in rabbit retina. Vis Neurosci. 1999 Mar-Apr;16(2):369–377. doi: 10.1017/s0952523899162163. [DOI] [PubMed] [Google Scholar]
  11. Kolb H. The architecture of functional neural circuits in the vertebrate retina. The Proctor Lecture. Invest Ophthalmol Vis Sci. 1994 Apr;35(5):2385–2404. [PubMed] [Google Scholar]
  12. Miyake Y., Yagasaki K., Horiguchi M. A rod-cone dysfunction syndrome with separate clinical entity: incomplete-type congenital stationary night blindness (Miyake). Prog Clin Biol Res. 1987;247:137–145. [PubMed] [Google Scholar]
  13. Miyake Y., Yagasaki K., Horiguchi M., Kawase Y. On- and off-responses in photopic electroretinogram in complete and incomplete types of congenital stationary night blindness. Jpn J Ophthalmol. 1987;31(1):81–87. [PubMed] [Google Scholar]
  14. Pillers D. A. Dystrophin and the retina. Mol Genet Metab. 1999 Oct;68(2):304–309. doi: 10.1006/mgme.1999.2929. [DOI] [PubMed] [Google Scholar]
  15. Pillers D. A., Fitzgerald K. M., Duncan N. M., Rash S. M., White R. A., Dwinnell S. J., Powell B. R., Schnur R. E., Ray P. N., Cibis G. W. Duchenne/Becker muscular dystrophy: correlation of phenotype by electroretinography with sites of dystrophin mutations. Hum Genet. 1999 Jul-Aug;105(1-2):2–9. doi: 10.1007/s004399900111. [DOI] [PubMed] [Google Scholar]
  16. SCHUBERT G., BORNSCHEIN H. Beitrag zur Analyse des menschlichen Elektroretinogramms. Ophthalmologica. 1952 Jun;123(6):396–413. doi: 10.1159/000301211. [DOI] [PubMed] [Google Scholar]
  17. Sieving P. A., Murayama K., Naarendorp F. Push-pull model of the primate photopic electroretinogram: a role for hyperpolarizing neurons in shaping the b-wave. Vis Neurosci. 1994 May-Jun;11(3):519–532. doi: 10.1017/s0952523800002431. [DOI] [PubMed] [Google Scholar]
  18. Sieving P. A. Photopic ON- and OFF-pathway abnormalities in retinal dystrophies. Trans Am Ophthalmol Soc. 1993;91:701–773. [PMC free article] [PubMed] [Google Scholar]
  19. Sigesmund D. A., Weleber R. G., Pillers D. A., Westall C. A., Panton C. M., Powell B. R., Héon E., Murphey W. H., Musarella M. A., Ray P. N. Characterization of the ocular phenotype of Duchenne and Becker muscular dystrophy. Ophthalmology. 1994 May;101(5):856–865. doi: 10.1016/s0161-6420(13)31249-4. [DOI] [PubMed] [Google Scholar]
  20. Stockton R. A., Slaughter M. M. B-wave of the electroretinogram. A reflection of ON bipolar cell activity. J Gen Physiol. 1989 Jan;93(1):101–122. doi: 10.1085/jgp.93.1.101. [DOI] [PMC free article] [PubMed] [Google Scholar]
  21. Tian N., Slaughter M. M. Correlation of dynamic responses in the ON bipolar neuron and the b-wave of the electroretinogram. Vision Res. 1995 May;35(10):1359–1364. doi: 10.1016/0042-6989(95)98715-l. [DOI] [PubMed] [Google Scholar]

Articles from Transactions of the American Ophthalmological Society are provided here courtesy of American Ophthalmological Society

RESOURCES