Two forms of autosomal dominant primary retinitis pigmentosa (original) (raw)

Abstract

Two types of autosomal dominant retinitis pigmentosa (RP) are identified on the basis of perimetric measures of rod sensitivity relative to cone sensitivity. Type 1 dominant RP patients are characterized by an early diffuse loss of rod sensitivity with a later loss of cone sensitivity and by childhood onset of nightblindness. Type 2 dominant RP patients are characterized by a regionalized and combined loss of rod and cone sensitivity with adulthood onset of nightblindness. Comparisons of losses in the photopic and scotopic electroretinogram amplitudes corroborate the psychophysical results. Clinical findings are similar for the two dominant RP subtypes, however, there are differences in natural history.

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Authors and Affiliations

1. Wilmer Ophthalmological Institute, The Johns Hopkins University School of Medicine, 21205, Baltimore, MD, USA
   R. W. Massof Ph.D. & D. Finkelstein

Authors

1. R. W. Massof Ph.D.
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2. D. Finkelstein
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Additional information

Supported by research grants from the U.S. Public Health Service, National Institutes of Health (EY-01791) and the National Retinitis Pigmentosa Foundation.

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Massof, R.W., Finkelstein, D. Two forms of autosomal dominant primary retinitis pigmentosa.Doc Ophthalmol 51, 289–346 (1981). https://doi.org/10.1007/BF00143336

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• Issue Date: November 1981
• DOI: https://doi.org/10.1007/BF00143336

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