June 2017
Volume 58, Issue 8
Open Access
ARVO Annual Meeting Abstract  |   June 2017
Humphrey visual field and flicker electroretinogram changes over two-year follow-up in patients with RLBP1 retinitis pigmentosa enrolled in a prospective Natural History Study
Author Affiliations & Notes
  • Karen Holopigian
    NIBR, East Hanover, New Jersey, United States
  • Jane Green
    Memorial University of Newfoundland , St. John's, Newfoundland, Canada
  • James Whelan
    Memorial University of Newfoundland, St. John’s, Newfoundland, Canada
  • Marie Burstedt
    Umea University, Umea, 901 85, Sweden
  • Zhenzhong Su
    NIBR, East Hanover, New Jersey, United States
  • Jean-Yves Deslandes
    NIBR, East Hanover, New Jersey, United States
  • Xiao Ni
    NIBR, East Hanover, New Jersey, United States
  • Michael Wald
    NIBR, East Hanover, New Jersey, United States
  • Cynthia L Grosskreutz
    NIBR, East Hanover, New Jersey, United States
  • Kalliopi Stasi
    NIBR, East Hanover, New Jersey, United States
  • Footnotes
    Commercial Relationships   Karen Holopigian, Novartis Pharmaceuticals (E); Jane Green, Novartis Pharmaceuticals (C); James Whelan, Novartis Pharmaceuticals (C); Marie Burstedt, Novartis Pharmaceuticals (C); Zhenzhong Su, Novartis Pharmaceuticals (E); Jean-Yves Deslandes, Novartis Pharmaceuticals (E); Xiao Ni, Novartis Pharmaceuticals (E); Michael Wald, Novartis Pharmaceuticals (E); Cynthia Grosskreutz, Novartis Pharmaceuticals (E); Kalliopi Stasi, Novartis Pharmaceuticals (E)
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2017, Vol.58, 3246. doi:
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      Karen Holopigian, Jane Green, James Whelan, Marie Burstedt, Zhenzhong Su, Jean-Yves Deslandes, Xiao Ni, Michael Wald, Cynthia L Grosskreutz, Kalliopi Stasi; Humphrey visual field and flicker electroretinogram changes over two-year follow-up in patients with RLBP1 retinitis pigmentosa enrolled in a prospective Natural History Study. Invest. Ophthalmol. Vis. Sci. 2017;58(8):3246.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Visual field defects and abnormal full-field electroretinograms (ERGs) occur early in retinitis pigmentosa due to RLBP1 mutations (RLBP1 RP) and can be used to follow disease progression. Visual fields and ERGs together with other functional and structural endpoints were monitored in a prospective Natural History Study in RLBP1 RP patients. Here, we report preliminary 2-year results on test-retest variability and progression of Humphrey visual fields and flicker ERGs (results from other endpoints are presented in additional abstracts).

Methods : This non-interventional, two-center (Sweden and Canada), prospective study included evaluations of 45 RLBP1 RP patients every 6 months. All participants had Humphrey visual fields (HVFs) using the SITA standard 30-2 program. A subset of patients (n=15) had full-field light-adapted 30 Hz flicker ERGs with Ganzfeld stimulation (after pupil dilation). Annual progression was estimated as the slope of a random effect linear growth model and test-retest variability was assessed using the intra-class correlation coefficient (ICC). Between-eye correlations were performed for each measurement and at each visit.

Results : HVF mean deviation (MD) and ERG amplitude and implicit time preliminary results (mean values), as well as annual progression slope and test-retest variability (ICCs) for right (OD) and left (OS) eyes are displayed in the table. Statistically significant Pearson’s correlations between the two eyes at baseline and at each follow-up visit were noted for HVF MD ranging from 0.908 to 0.984 (p <0.001); for ERG amplitude ranging from 0.941 to 0.983 (p <0.001) and for ERG implicit time ranging from 0.923 to 0.964 (p <0.001).

Conclusions : RLBP1 RP patients showed HVF and flicker ERG deficits consistent with photoreceptor degeneration. Both eyes were similarly affected with respect to HVF and ERG measurements. There was no statistically significant progression over the 2-year follow-up.

This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.

 

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