Two forms of autosomal dominant primary retinitis pigmentosa

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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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Retinitis Pigmentosa
Vertebrate Photoreceptor Cells
Natural History
Retinitis Pigmentosa 2
Retinitis pigmentosa 1

Keywords

  • absolute thresholds
  • electroretinogram
  • inherited retinal degenerations
  • retinitis pigmentosa
  • spectral sensitivity
  • static perimetry

ASJC Scopus subject areas

  • Ophthalmology

Cite this

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title = "Two forms of autosomal dominant primary retinitis pigmentosa",
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.",
keywords = "absolute thresholds, electroretinogram, inherited retinal degenerations, retinitis pigmentosa, spectral sensitivity, static perimetry",
author = "Massof, {Robert W} and Daniel Finkelstein",
year = "1981",
month = "11",
doi = "10.1007/BF00143336",
language = "English (US)",
volume = "51",
pages = "289--346",
journal = "Documenta Ophthalmologica",
issn = "0012-4486",
publisher = "Springer Netherlands",
number = "4",

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T1 - Two forms of autosomal dominant primary retinitis pigmentosa

AU - Massof, Robert W

AU - Finkelstein, Daniel

PY - 1981/11

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N2 - 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.

AB - 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.

KW - absolute thresholds

KW - electroretinogram

KW - inherited retinal degenerations

KW - retinitis pigmentosa

KW - spectral sensitivity

KW - static perimetry

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JO - Documenta Ophthalmologica

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