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Inherited Chorioretinal Dystrophies pp 13–18Cite as

Electrophysiological Testing

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Abstract

The objective data provided by the electrophysiological assessment of retinal function are fundamental to the accurate diagnosis and appropriate management of patients with genetically determined retinal disease. In some disorders, the abnormalities visible upon ophthalmoscopy may suggest the diagnosis when taken in clinical context. An example of that is the group of disorders collectively known as retinitis pigmentosa (RP), in which there is intraretinal bone-spicule pigment deposition consequent upon photoreceptor cell death. However, the extent and severity of the fundus appearance may correlate poorly with the results of objective electroretinographic examination, and the latter greatly facilitates counselling in relation to prognosis. Equally, there are some disorders, such as “cone dystrophy with supernormal rod ERG”, related to mutation in KCNV2, where the diagnosis is made by the pathognomonic electrophysiological features. Furthermore, those disorders where the site of dysfunction is primarily inner retinal may be associated with a normal fundus appearance.

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References

  1. Arden GB, Wolf J, Berninger TA, Hogg CR, Tzekov R, Holder GE. S-cone ERGs elicited by a simple technique in normals and in tritanopes. Vision Res. 1999;39:641–50.

    Article  CAS  PubMed  Google Scholar 

  2. Brown M, Marmor MF, Vaegan, Zrenner E, Brigell M, Bach M. ISCEV standard for clinical electro-oculography (EOG). Doc Ophthalmol. 2006;113:205–12.

    Article  PubMed Central  PubMed  Google Scholar 

  3. Bush RA, Sieving PA. A proximal retinal component in the primate photopic ERG a-wave. Invest Ophthalmol Vis Sci. 1994;35:635–45.

    CAS  PubMed  Google Scholar 

  4. Bush RA, Sieving PA. Inner retinal contributions to the primate photopic fast flicker electroretinogram. J Opt Soc Am A Opt Image Sci Vis. 1996;13:557–65.

    Article  CAS  PubMed  Google Scholar 

  5. Frishman LJ. Origins of the electroretinogram. In: Heckenlively JR, Arden GB, editors. Principles and practice of clinical electrophysiology of vision. 2nd ed. Cambridge, MA: MIT Press; 2006. p. 139–83.

    Google Scholar 

  6. Harrison JM, O’Connor PS, Young RS, Kincaid M, Bentley R. The pattern ERG in man following surgical resection of the optic nerve. Invest Ophthalmol Vis Sci. 1987;28:492–9.

    CAS  PubMed  Google Scholar 

  7. Holder GE. Significance of abnormal pattern electroretinography in anterior visual pathway dysfunction. Br J Ophthalmol. 1987;71:166–71.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  8. Holder GE. Pattern ERG, and an integrated approach to visual pathway diagnosis. Prog Retin Eye Res. 2001;20:531–61.

    Article  CAS  PubMed  Google Scholar 

  9. Holder GE. Pattern ERG. In: Fishman GA, Birch DG, Holder GE Brigell MG, editors. Electrophysiologic testing in disorders of the retina, optic nerve and visual pathway, Ophthalmology monographs No.2. 2nd ed. San Francisco: The Foundation of the American Academy of Ophthalmology; 2001. p. 197–237.

    Google Scholar 

  10. Holder GE, Brigell MG, Hawlina M, Meigen T, Vaegan, Bach M. ISCEV standard for clinical pattern electroretinography – 2007 update. Doc Ophthalmol. 2007;114:111–6.

    Article  PubMed Central  PubMed  Google Scholar 

  11. Holder GE, Votruba M, Carter AC, Bhattacharya SS, Fitzke FW, Moore AT. Electrophysiological findings in Dominant Optic Atrophy (DOA) linking to the OPA1 locus on chromosome 3q 28-qter. Doc Ophthalmol. 1999;95:217–28.

    Article  CAS  Google Scholar 

  12. Hood DC. Assessing retinal function with the multifocal technique. Prog Retin Eye Res. 2000;19:607–46.

    Article  CAS  PubMed  Google Scholar 

  13. Hood DC, Bach M, Brigell M, Keating D, Kondo M, Lyons JS, Palmowski-Wolfe AM. ISCEV Guidelines for clinical multifocal electroretinography (2007 edition). Doc Ophthalmol. 2008;116:1–11. Epub 2007 Oct 31.

    Article  PubMed Central  PubMed  Google Scholar 

  14. Hood DC, Birch DG. The a-wave of the human ERG and rod receptor function. Invest Ophthalmol Vis Sci. 1990;31:2070–81.

    CAS  PubMed  Google Scholar 

  15. Hood DC, Birch DG. B-wave of the scotopic (rod) electroretinogram as a measure of the activity of human on-bipolar cells. J Opt Soc Am. 1996;13:623–33.

    Article  CAS  Google Scholar 

  16. Koh AH, Hogg CR, Holder GE. The incidence of negative ERG in clinical practice. Doc Ophthalmol. 2001;102:19–30.

    Article  CAS  PubMed  Google Scholar 

  17. Lamb TD, Pugh Jr EN. A quantitative account of the activation steps involved in phototransduction in amphibian photoreceptors. J Physiol. 1992;449:719–58.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  18. McBain VA, Egan CA, Pieris SJ, Supramaniam G, Webster AR, Bird AC, Holder GE. Functional observations in vitamin A deficiency: diagnosis and time-course of recovery. Eye. 2007;21:367–76.

    Article  CAS  PubMed  Google Scholar 

  19. Marmor MF, Fulton AB, Holder GE, Miyake Y, Brigell M, Bach M, International Society for Clinical Electrophysiology of Vision. ISCEV Standard for full-field clinical electroretinography (2008 update). Doc Ophthalmol. 2009;118:69–77.

    Article  CAS  PubMed  Google Scholar 

  20. Miyake Y, Yagasaki K, Horiguchi M, Kawase Y, Kanda T. Congenital stationary night blindness with negative electroretinogram: a new classification. Arch Ophthalmol. 1986;104:1013–20.

    Article  CAS  PubMed  Google Scholar 

  21. Robson AG, El-Amir A, Bailey C, Egan CA, Fitzke FW, Webster AR, Bird AC, Holder GE. Pattern ERG correlates of abnormal fundus autofluorescence in patients with retinitis pigmentosa and normal visual acuity. Invest Ophthalmol Vis Sci. 2003;44:3544–50.

    Article  PubMed  Google Scholar 

  22. Ryan S, Arden GB. Electrophysiological discrimination between retinal and optic nerve disorders. Doc Ophthalmol. 1988;68:247–55.

    Article  CAS  PubMed  Google Scholar 

  23. Sieving PA. Photopic ON, and OFF –pathway abnormalities in retinal dystrophies. Trans Am Ophthalmol Soc. 1993;91:701–73.

    PubMed Central  CAS  PubMed  Google Scholar 

  24. Tsang SH, Vaclavik V, Bird AC, Robson AG, Holder GE. Novel phenotypic and genotypic findings in X-linked retinoschisis (XLRS). Arch Ophthalmol. 2007;125:259–67.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  25. Ueno S, Kondo M, Ueno M, Miyata K, Terasaki H, Miyake Y. Contribution of retinal neurons to d-wave of primate photopic electroretinograms. Vision Res. 2006;46:658–64.

    Article  PubMed  Google Scholar 

  26. Viswanathan S, Frishman LJ, Robson JG. The uniform field and pattern ERG in macaques with experimental glaucoma: removal of spiking activity. Invest Ophthalmol Vis Sci. 2000;41:2797–810.

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Electrophysiology, Moorfields Eye Hospital, City Road, London, EC1V 2PD, UK

    Graham E. Holder

  2. UCL Institute of Ophthalmology, 11-43 Bath Street, London, EC1V 9EL, UK

    Graham E. Holder

Authors
  1. Graham E. Holder
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Correspondence to Graham E. Holder .

Editor information

Editors and Affiliations

  1. Service d’Exploration de la Vision et Neuro-ophtalmologie, Centre Hospitalier Universitaire de Lille, Hôpital Roger Salengro, Lille Cedex, France

    Bernard Puech

  2. Department of Ophthalmology, Ghent University Hospital, Ghent University, Ghent, Belgium

    Jean-Jacques De Laey

  3. Department of Electrophysiology, Moorfields Eye Hospital, London, United Kingdom

    Graham E. Holder

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© 2014 Springer-Verlag Berlin Heidelberg

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Holder, G.E. (2014). Electrophysiological Testing. In: Puech, B., De Laey, JJ., Holder, G. (eds) Inherited Chorioretinal Dystrophies. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-69466-3_2

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  • DOI: https://doi.org/10.1007/978-3-540-69466-3_2

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-69464-9

  • Online ISBN: 978-3-540-69466-3

  • eBook Packages: MedicineMedicine (R0)

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