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The incidence of negative ERG in clinical practice

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Abstract

Introduction: A negative electroretinogram (ERG) is one in which there is a selective reduction in amplitude of the b-wave, such that it does not exceed that of the a-wave. The purpose of this study was to determine the incidence and clinical causes of negative ERGs at a tertiary referral centre. In addition, interesting and previously unreported aetiologies are described. Patients and methods: Retrospective review of all ERGs done at Moorfields Eye Hospital from November 1995 to December 1998 under ISCEV standard conditions. Many patients had photopic ON- and OFF-response recording in addition to conventional ISCEV Standard ganzfeld ERG. Results: A total of 2,640 ERGs were performed during the study period. 128 cases (4.8%) showed a negative ERG. The causes, where a firm clinical diagnosis was possible, include X-linked juvenile retinoschisis, congenital stationary night blindness, central retinal artery occlusion, birdshot chorioretinopathy and melanoma-associated retinopathy (MAR). Unilateral negative ERG waveforms with normal fundal appearances were seen in 7 patients. Photopic ON- responses could be selectively affected. Conclusions: The incidence of negative ERGs over a 34-month period presenting to a large tertiary centre was almost 5%. The presence of a negative ERG may be instrumental in demonstrating the site of visual dysfunction, with many cases showing minimal or no fundus abnormality. ON- and OFF-response recording yielded additional information regarding photopic post-receptoral/phototransduction function.

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References

  1. Karpe G, Uchermann A. The clinical electroretinogram. IV. The electroretinogram in circulatory disturbances of the retina. Acta Ophthalmol (Copenh) 1955; 33: 493–516.

    Google Scholar 

  2. Peachey NS, Fishman GA, Derlacki DJ, Brigell Mg. Psychophysical and electroretinographic findings in X-linked juvenile retinoschisis. Arch Ophthalmol 1987; 105: 513–516.

    Google Scholar 

  3. 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: 81–87.

    Google Scholar 

  4. Berson EL, Lessell S. Paraneoplastic night blindness with malignant melanoma. Am J Ophthalmol 1988; 106: 307–311.

    Google Scholar 

  5. Schechner R, Miller B, Merksamer E, Perlman I. A long term follow up of ocular siderosis: quantitative assessment of the electroretinogram. Doc Ophthalmol 1991; 76: 231–240.

    Google Scholar 

  6. Bacon P, Spalton DJ, Smith SE. Blindness from quinine toxicity. Br J Ophthalmol 1988; 72: 219–224.

    Google Scholar 

  7. Pillers DA, Seltzer WK, Powell BR, et al. Negative-configuration electroretinogram in Oregon eye disease. Consistent phenotype in Xp21 deletion syndrome. Arch Ophthalmol 1993; 111: 1558–1563.

    Google Scholar 

  8. Jensen H, Warburg M, Sjo 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; 32: 348–351.

    Google Scholar 

  9. Miyake Y, Horiguchi M, Suzuki S, Kondo M, Tanikawa A. Electrophysiological findings in patients with Oguchi' disease. Jpn J Ophthalmol 1996; 40: 511–519.

    Google Scholar 

  10. Ripps H, Carr RE, Siegel IM, Greenstein VC. Functional abnormalities in vincristineinduced night blindness. Invest Ophthalmol Vis Sci 1984; 25: 787–794.

    Google Scholar 

  11. Priem HA, De Rouck A, De Laey JJ, Bird AC. Electrophysiologic studies in birdshot chorioretinopathy. Am J Ophthalmol 1988; 106: 430–436.

    Google Scholar 

  12. Marshman WE, Lee JP, Jones B, Schalit G, Holder GE. Duane' retraction syndrome and juvenile Batten' disease: a new association? Aust NZ J Ophthalmol 1998; 26: 251–254.

    Google Scholar 

  13. McKellar MJ, Hidajat RR, Elder MJ. Acute ocular methanol toxicity: clinical and electrophysiological features. Aust NZ J Ophthalmol 1997; 25: 225–230.

    Google Scholar 

  14. Miyake Y, Shiroyama N, Horiguchi M, Saito A, Yagasaki K. Bull'-eye maculopathy and negative electroretinogram. Retina 1989; 9: 210–215.

    Google Scholar 

  15. Weleber RG, Pillers DA, Powell BR, Hanna CE, Magenis RE, Buist NR. Aland Island eye disease (Forsius-Eriksson syndrome) associated with contiguous deletion syndrome at Xp21. Similarity to incomplete congenital stationary night blindness. Arch Ophthalmol 1989; 107: 1170–1179.

    Google Scholar 

  16. George ND, Yates JR, Moore AT. X linked retinoschisis. Br J Ophthalmol 1995; 79: 697–702.

    Google Scholar 

  17. Fishman GA, Alexander KR, Milam AH, Derlacki DJ. Acquired unilateral night blindness associated with a negative electroretinogram waveform. Ophthalmology 1996; 103: 96–104.

    Google Scholar 

  18. Alexander KR, Fishman GA, Peachey NS, Marchese AL, Tso MO. ‘On’ response defect in paraneoplastic night blindness with cutaneous malignant melanoma. Invest Ophthalmol Vis Sci 1992; 33: 477–483.

    Google Scholar 

  19. Holder GE. Electrophysiological features of melanoma associated retinopathy (MAR). Invest Ophthalmol Vis Sci 2000; 41: S568.

    Google Scholar 

  20. Stanga P, Chong NV, Reck AC, Hardcastle AJ, Holder GE. Optical Coherence Tomography (OCT) and Electrophysiology in X-linked Juvenile Retinoschisis Associated with a Novel Mutation in the XLRS1 Gene. Retina 2001; 21: 78–80.

    Google Scholar 

  21. Milam AH, Saari JC, Jacobson SG, Lubinski WP, Feun LG, Alexander KR. Autoantibodies against retinal bipolar cells in cutaneous melanoma-associated retinopathy. Invest Ophthalmol Vis Sci 1993; 34: 91–100.

    Google Scholar 

  22. Kim RY, Retsas S, Fitzke FW, Arden GB, Bird AC. Cutaneous melanoma-associated retinopathy. Ophthalmology 1994; 101: 1837–1843.

    Google Scholar 

  23. Kellner U, Bornfeld N, Foerster MH. Severe course of cutaneous melanoma associated paraneoplastic retinopathy. Br J Ophthalmol 1995; 79: 746–752.

    Google Scholar 

  24. Thirkill CE, Tait RC, Tyler NK, Roth AM, Keltner JL. The cancer-associated retinopathy antigen is a recoverin-like protein. Invest Ophthalmol Vis Sci 1992; 33: 2768–2772.

    Google Scholar 

  25. Guy J, Aptsiauri N. Treatment of paraneoplastic visual loss with intravenous immunoglobulin: report of 3 cases. Arch Ophthalmol 1999; 117: 471–477.

    Google Scholar 

  26. Adamus G, Amundson D, Seigel GM, Machnicki M. Anti-enolase-alpha autoantibodies in cancer-associated retinopathy: epitope mapping and cytotoxicity on retinal cells. J Autoimmun 1998; 11: 671–677.

    Google Scholar 

  27. Cideciyan AV, Jacobson SG. Negative electroretinograms in retinitis pigmentosa. Invest Ophthalmol Vis Sci 1993; 34: 3253–3263.

    Google Scholar 

  28. Fujii N, Shiono T, Wada Y, Nakazawa M, Tamai M, Yamada N. Autosomal dominant cone-rod dystrophy with negative electroretinogram. Br J Ophthalmol 1995; 79: 916–921.

    Google Scholar 

  29. Kellner U, Foerster MH. Cone dystrophies with negative photopic electroretinogram. Br J Ophthalmol 1993; 77: 404–409.

    Google Scholar 

  30. Milam AH, Li ZY, Fariss RN. Histopathology of the human retina in retinitis pigmentosa. Prog Ret Eye Res 1998; 17: 175–205.

    Google Scholar 

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Koh, A., Hogg, C. & Holder, G. The incidence of negative ERG in clinical practice. Doc Ophthalmol 102, 19–30 (2001). https://doi.org/10.1023/A:1017586118749

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