Long-term visual prognoses in patients with retinitis pigmentosa: The Ludwig von Sallmann lecture

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Abstract

Retinitis pigmentosa can be followed over almost its entire course with narrow bandpassed, computer averaged cone electroretinograms (ERGs). The long-term rate of decline of these responses can be described by an exponential function. A cone ERG actuarial table based on 1039 patients and 6553 visits is presented to show the estimated number of years for an average patient with a given 30-Hz cone ERG amplitude to decline to 0.05 μV (i.e. virtual blindness). The table is based on a projected rate of loss of 10% of remaining cone ERG amplitude per year for those not on treatment and 8.3% per year for those on treatment with vitamin A palmitate 15,000 IU/day. The table can be used to provide an estimate of the average long-term visual prognosis from a single visit; more precise estimates for a specific patient require several additional visits over 2- to 3-year intervals. Evidence is presented to support the idea that patients with a projected cone amplitude of 3.5 μV or greater at age 40 (about 25% of our patient population with typical retinitis pigmentosa) would be expected, on average, to retain some useful vision for their entire lives without treatment. Knowledge of the amount of remaining cone function in the ERG often reduces patient anxiety and helps patients plan for their future.

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Acknowledgments

I thank Dr. Peter Gouras and his colleagues on the Awards Committee and the officers of the International Congress of Eye Research for giving me this opportunity to speak. I also thank my collaborators Dr. Bernard Rosner, Dr. Michael Sandberg, and Carol Weigel-DiFranco for their invaluable contributions to this research and our clinical and administrative staff for their assistance, namely Dr. Robert Brockhurst, Dr. Alexander Gaudio, Bethany Biron, Emorfily Potsidis, Yuji Che, Rorn Seng,

References (18)

  • J. Choi et al.

    Natural course of ocular function in pigmented paravenous retinochoroidal atrophy

    Am. J. Ophthalmol.

    (2006)
  • D.T. Hartong et al.

    Retinitis pigmentosa

    Lancet, Seminar Series

    (2006)
  • S.O.L. Andréasson et al.

    Narrowband filtering for monitoring low-amplitude cone electroretinograms in retinitis pigmentosa

    Am. J. Ophthalmol.

    (1988)
  • E.L. Berson

    Retinitis pigmentosa and allied diseases: electrophysiologic findings

    Trans. Am. Acad. Ophthalmol. Otolaryngol.

    (1976)
  • E.L. Berson

    Retinitis pigmentosa. The Friedenwald Lecture

    Investig. Ophthalmol. Vis. Sci.

    (1993)
  • E.L. Berson et al.

    Progressive cone–rod degeneration

    Arch. Ophthalmol.

    (1968)
  • E.L. Berson et al.

    A randomized trial of vitamin A and vitamin E supplementation for retinitis pigmentosa

    Arch. Ophthalmol.

    (1993)
  • E.L. Berson et al.

    Further evaluation of docosahexaenoic acid in patients with retinitis pigmentosa receiving vitamin A treatment: subgroup analyses

    Arch. Ophthalmol.

    (2004)
  • E.L. Berson et al.

    Disease progression in patients with dominant retinitis pigmentosa and rhodopsin mutations

    Investig. Ophthalmol. Vis. Sci.

    (2002)
There are more references available in the full text version of this article.

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From the Berman-Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, MA 02114, USA. Presented on October 30, 2006 at the XVIIth Meeting of the International Congress of Eye Research, Buenos Aires, Argentina.

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