Skip to main content
SpringerLink
Log in

Scotopic Threshold Responses and Rod Intensity-Response Functions as Sensitive Indicators of the Carrier Status In X-Linked Recessive Retinitis Pigmentosa

  • Chapter
Degenerative Diseases of the Retina

  • 129 Accesses

Abstract

In previous studies several authors described modifications of ERG responses in X-linked recessive retinitis pigmentosa (xLRP) carriers (1–8). Investigations on the intensity-response functions of the rod ERG also demonstrated reduction of Vmax in subjects with ophthalmoscopic evidence of the carrier status (pigmentary changes and/or tapetal-like reflex) (4). In patients affected with RP abnormalities of the Oscillatory Potentials (OPs) have also been demonstrated (9,10), suggesting a coexisting impairment of the inner retinal layers. Abnormalities of rod sensitivity were also found at the psychophysical level to flickering stimuli (11).

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Berson, E.L., Rosen, J.B., and Simonoff, E.A., 1979, Electroretinographic testing as an aid in detection of carriers of X-chromosome-linked retinitis pigmentosa. Am. J. Ophthalmol. 87: 460–468.

    Article  CAS  PubMed  Google Scholar 

  2. Arden, G.B., Carter, R.M., Hogg, C.R., Powell, J., Ernst, W.J., Clover, G.M., Lyness, A.L., and Quinlan, M.R, 1983, A modified ERG technique and the results obtained in X-linked retinitis pigmentosa. Br. J. Ophthalmol. 67:419–430.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  3. Fishman, G.A., Weinberg, A.B., and McMahon, T.T., 1986, X-linked recessive retinitis pigmentosa. Clinical characteristics of carriers. Arch. Ophthalmol. 104: 1329–1335.

    Article  CAS  PubMed  Google Scholar 

  4. Peachey, N.S., Fishman, G.A., Derlacki, J., and Alexander, K.R., 1988, Rod and cone dysfunction in carriers of X-linked retinitis pigmentosa. Ophthalmology 95: 677–685.

    Article  CAS  PubMed  Google Scholar 

  5. Jacobson, S.G., Yagasaki, K., Feuer, W.J., and Roman, A.J., 1989, Interocularasimmetry of visual function in heterozygotes of X-linked retinitis pigmentosa. Exp. Eye Res. 48: 679–691.

    Article  CAS  PubMed  Google Scholar 

  6. Jacobson, S.G. Roman, A.J., Cideciyan, A.V., Robey, M.G., Iwata, T., and Inana, G., 1992, X-linked retinitis pigmentosa: functional phenotype of an RP2 genotype. Invest. Ophthalmol. Vis. Sci. 33: 3481–3492.

    CAS  PubMed  Google Scholar 

  7. Andréasson, S.O.L., and Ehinger, B., 1990, Electroretinographic diagnosis in families with X-linked retinitis pigmentosa. Acta Ophthalmol. 68: 139–144.

    Article  Google Scholar 

  8. Garcia-Sandoval, B. Del Rio, T., Ayuso, C., Serrano, J.M., and Zato, M.A., 1994, 30-Hz flicker ERG in carriers of X-linked retinitis pigmentosa. Proceedings of the 34th AER Meeting, Granada, Spain, p. 76 (Abstract).

    Google Scholar 

  9. Ponte, F. Anastasi, M., and Lauricella, M.R., 1990, Retinitis pigmentosa and inner retina. Functional study by means of oscillatory potentials of the electroretinogram. Doc. Ophthalmol 73: 337–346.

    Article  Google Scholar 

  10. Anastasi, M., Lauricella, M., and Ponte, F., 1992, Photopic ERG components in retinitis pigmentosa. Acta Ophthalmol 70: 187–193.

    Article  CAS  Google Scholar 

  11. Ernst, W.J., Clover, G., and Faulkner, J., 1981, X-linked retinitis pigmentosa: reduced rod flicker sensitivity in heterozygous females. Invest. Ophthalmol. 20: 812–816.

    CAS  Google Scholar 

  12. Wakabayashi, K., Gieser, J., and Sieving, P.A., 1988, Aspartate separation of the scotopic threshold response (STR) from the photoreceptor a-wave of the cat and monkey ERG. Invest. Ophthalmol Vis. Sci. 29: 1615–1622.

    CAS  PubMed  Google Scholar 

  13. Sieving, P.A., Frishman, L.J., and Steinberg, R.H., 1986, Scotopic threshold response of proximal retina in cat. J. Neurophysiol 56: 1049–1061.

    CAS  PubMed  Google Scholar 

  14. Zrenner, E., and Nelson, R., 1988, Spatial characteristics of scotopic threshold responses in the corneally recorded electroretinogram elicited by multispot patterns. Clin. Vis. Sci. 3: 29–44.

    Google Scholar 

  15. Frishman, L.J., Sieving, P.A., and Steinberg, R.H., 1988, Contributions to the electroretinogram of currents originating in proximal retina. Vis. Neurosci. 1: 307–315.

    Article  CAS  PubMed  Google Scholar 

  16. Naarendorp, F., and Sieving, P.A., 1990, Effects of strychnine and GABA-antagonists on the STR of the cat ERG. ARVO Abstracts. Invest. Ophthalmol. Vis. Sci. 31: 390.

    Google Scholar 

  17. Naarendorp, F., and Sieving, P.A., 1991, The scotopic threshold response of the cat ERG is suppressed selectively by GABA and glycine. Vision Res. 31: 1–15.

    Article  CAS  PubMed  Google Scholar 

  18. Sieving, P.A., Nino, C., 1988, Scotopic Threshold Response (STR) of the human electroretinogram. Invest. Ophtahlmol. Vis. Sci. 29: 1608–1614.

    CAS  Google Scholar 

  19. Sieving, P.A., 1991, Retinal Ganglion cell loss does not abolish the scotopic threshold response (STR) of the cat and human ERG. Clin. Vis. Sci. 6: 149–158.

    Google Scholar 

  20. Frishman, L.J. and Steinberg, R.H., 1989, Light-evoked increases in [K+]o in proximal portion of the dark-adapted cat retina. J. Neurophysiol. 61: 1233–1243.

    CAS  PubMed  Google Scholar 

  21. Aylward, G.W., 1989, The Scotopic Threshold Response in Diabetic Retinopathy. Eye 3: 626–637.

    Article  PubMed  Google Scholar 

  22. Murayama, K., Kuo, C.Y., and Sieving, P.A., 1991, Abnormal threshold ERG response in X-linked juvenile retinoschisis: evidence for a proximal retinal origin of the human STR. Clin. Vis. Sci. 6: 317–322.

    Google Scholar 

  23. Graham, S.L., and Vaegan, 1991, High correlation between absolute psychophysical threshold and the scotopic threshold response to the same stimulus. Br. J. Ophthalmol. 75: 603–607.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  24. Iannaccone, A. Vingolo, E.M., Tanzilli, P., and Rispoli, E., 1994, The Scotopic Threshold Response in different forms of Congenital Stationary Night Blindness. ARVO Abstracts. Invest. Ophthalmol. Vis. Sci. 35: 1377.

    Google Scholar 

  25. Miyake, Y., Horiguchi, M., Terasaki, H., and Kondo, M., 1994, Scotopic Threshold Response in complete and incomplete types of Congenital Stationary Night Blindness. Invest. Ophthalmol. Vis. Sci. 35: 3770–3775.

    CAS  PubMed  Google Scholar 

  26. Iannaccone, A., Forte, R., Tanzilli, P., De Bernardo, C., Vingolo, E.M., and Pannarale, M.R., 1994, Clinical and electroretinographic intrafamilial variability in the Goldmann-Favre vitreo-retinal dystrophy. Proceedings of the International Workshop on Inherited Paediatric Retinal Disorders, Regensburg, p. 36 (Abstract). Ophthalmic Genetics (submitted for publication).

    Google Scholar 

  27. Rispoli, E., Tommasini, P., Pannarale, L., Carloni, C., Perdicchi, A., Rispoli, M., and Pannarale, M.R., 1993, The Scotopic Threshold Response in diabetic patients without evidence of retinopathy. Proceedings of the 4th Schepens International Society Meeting, Hong Kong, p. 148 (Abstract).

    Google Scholar 

  28. Carloni, C., Malagola, R., Tommasini, P., and Rispoli, E., 1994, The Scotopic Threshold Response in non-insulin dependent diabetic patients. Proceedings of the 74th Congress of Italian Society of Ophthalmology (SOI), Mondovi (Cuneo), Italy: Arti Grafiche Dial (in press).

    Google Scholar 

  29. Rispoli, E., Tommasini, P., Pannarale, L., Carrozzoni, P., Giusti, C., Rispoli, M., and Pannarale, M.R., 1993, Clinical applications of the Scotopic Threshold Response in myopic subjects. Proceedings of the 4th Schepens International Society Meeting, Hong Kong, p. 126 (Abstract).

    Google Scholar 

  30. Del Porto, G., Vingolo, E.M., David, D., Steindl, K., Wedemann, H., Forte, R., Iannaccone, A., Gal, A., and Pannarale, M.R., 1993, Clinical features of autosomal dominant retinitis pigmentosa associated with the Gly-188-Arg mutation of the rhodopsin gene. In: Hollyfield, J.G., La Vail, M.M., Anderson, R.E., editors, Retinal Degeneration. Clinical and Laboratory Applications. New York: Plenum Press: 91–101.

    Chapter  Google Scholar 

  31. Rispoli, E., Iannaccone, A., and Vingolo, E.M., 1994, Low-noise electroretinogram recording techniques in retinitis pigmentosa. Doc. Ophthalmol. (in press).

    Google Scholar 

  32. Masland, R.H., 1988, Amacrine cells. TINS 11: 405–410.

    CAS  PubMed  Google Scholar 

  33. Sharpe, L.T., Stockman, A., and MacLeod, D.I.A., 1989, Rod flicker perception: scotopic duality, phase lags and destructive interference. Vision Res. 29, 1539–1559.

    Article  CAS  PubMed  Google Scholar 

  34. Sharpe, L.T., Fach, C.C., and Stockman, A., 1993, The spectral properties of the two rod pathways. Vision Res., 33, 2705–2720.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Ocular Electrophysiology, Center for Inherited Degenerative Retinal Disorders, University “La Sapienza”, Institute of Ophthalmology, Viale del Policlinico 1, 00161, Rome, Italy

    A. Iannaccone, E. M. Vingolo, R. Forte, P. Tanzilli, E. Rispoli & M. R. Pannarale

  2. Medical Genetic Section of the Experimental Medicine Department, University “La Sapienza”, c/o Ospedale L. Spallanzani Via Portuense 292, 00149, Rome, Italy

    B. Grammatico, C. De Bernardo & G. Del Porto

Authors
  1. A. Iannaccone
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. E. M. Vingolo
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. R. Forte
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. P. Tanzilli
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. B. Grammatico
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. C. De Bernardo
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. E. Rispoli
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. G. Del Porto
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. M. R. Pannarale
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA

    Robert E. Anderson

  2. University of California, San Francisco, San Francisco, California, USA

    Matthew M. LaVail

  3. The Cleveland Clinic Foundation, Cleveland, Ohio, USA

    Joe G. Hollyfield

Rights and permissions

Reprints and permissions

Copyright information

© 1995 Springer Science+Business Media New York

About this chapter

Cite this chapter

Iannaccone, A. et al. (1995). Scotopic Threshold Responses and Rod Intensity-Response Functions as Sensitive Indicators of the Carrier Status In X-Linked Recessive Retinitis Pigmentosa. In: Anderson, R.E., LaVail, M.M., Hollyfield, J.G. (eds) Degenerative Diseases of the Retina. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-1897-6_41

Download citation

  • DOI: https://doi.org/10.1007/978-1-4615-1897-6_41

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-5774-2

  • Online ISBN: 978-1-4615-1897-6

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation