Literature DB >> 11157882

On-response deficit in the electroretinogram of the cone system in X-linked retinoschisis.

K R Alexander1, G A Fishman, C S Barnes, S Grover.   

Abstract

PURPOSE: The purpose of this study was to evaluate the hypothesis that the reduced b-wave to a-wave ratio of the brief-flash electroretinogram (ERG) of the cone system typically observed in X-linked retinoschisis (XLRS) represents a relatively greater deficit in the ON response (response to light onset) than the OFF response (response to light offset). A second purpose was to investigate the use of sawtooth flicker as a stimulus for eliciting ERG ON and OFF responses.
METHODS: Light-adapted, full-field ERGs were recorded in six patients with XLRS and six age-similar control subjects in response to 8-Hz rapid-on and rapid-off sawtooth flicker to emphasize ON and OFF responses, respectively. ERG responses were analyzed in terms of the amplitudes and implicit times of the a-wave, b-wave, and d-wave components.
RESULTS: There was no significant difference between the patients with XLRS and the control subjects for either the amplitude of the a-wave of the ON response or the amplitude of the d-wave of the OFF response. However, the amplitude of the b-wave of the ON response was reduced significantly in the patients with XLRS, resulting in a significantly reduced b-wave to d-wave ratio. The patients' implicit times were increased significantly for all waveform components.
CONCLUSIONS: The reduced b-wave to d-wave ratio of the ERG of the cone system in these patients with XLRS is consistent with a relative dysfunction of the cone ON bipolar cell pathway in this disorder. The results show further that sawtooth flicker is a promising stimulus for eliciting well-defined ERG waveforms that can provide a quantitative assessment of the properties of ON and OFF responses in retinal disease.

Entities:  

Mesh:

Year:  2001        PMID: 11157882

Source DB:  PubMed          Journal:  Invest Ophthalmol Vis Sci        ISSN: 0146-0404            Impact factor:   4.799


  15 in total

1.  Novel 473-bp deletion in XLRS1 gene in a Japanese family with X-linked juvenile retinoschisis.

Authors:  Kei Shinoda; Hisao Ohde; Susumu Ishida; Makoto Inoue; Yoshihisa Oguchi; Yukihiko Mashima
Journal:  Graefes Arch Clin Exp Ophthalmol       Date:  2004-02-20       Impact factor: 3.117

2.  On- and off-response ERGs elicited by sawtooth stimuli in normal subjects and glaucoma patients.

Authors:  Gobinda Pangeni; Robert Lämmer; Ralf P Tornow; Folkert K Horn; Jan Kremers
Journal:  Doc Ophthalmol       Date:  2012-03-29       Impact factor: 2.379

3.  Spatial distributions of on- and off-responses determined with the multifocal ERG.

Authors:  Anderson R Rodrigues; Manoel da Silva Filho; Luiz Carlos L Silveira; Jan Kremers
Journal:  Doc Ophthalmol       Date:  2010-04       Impact factor: 2.379

4.  Negative electroretinograms in the pediatric and adult population.

Authors:  Johnstone M Kim; John F Payne; Jiong Yan; Claire S Barnes
Journal:  Doc Ophthalmol       Date:  2012-01-13       Impact factor: 2.379

5.  Characteristics of late negative ERG responses elicited by sawtooth flicker.

Authors:  Sowjanya Gowrisankaran; Mohamed A Genead; Anastasios Anastasakis; Kenneth R Alexander
Journal:  Doc Ophthalmol       Date:  2012-10-06       Impact factor: 2.379

6.  ERG variability in X-linked congenital retinoschisis patients with mutations in the RS1 gene and the diagnostic importance of fundus autofluorescence and OCT.

Authors:  Agnes B Renner; Ulrich Kellner; Britta Fiebig; Elke Cropp; Michael H Foerster; Bernhard H F Weber
Journal:  Doc Ophthalmol       Date:  2007-11-07       Impact factor: 2.379

7.  Abnormalities of the scotopic threshold response correlated with gene mutation in X-linked retinoschisis and congenital stationary night blindness.

Authors:  Keith Bradshaw; Douglas Newman; Louise Allen; Anthony Moore
Journal:  Doc Ophthalmol       Date:  2003-09       Impact factor: 2.379

8.  Human flicker electroretinography using different temporal modulations at mesopic and photopic luminance levels.

Authors:  Balázs Vince Nagy; Mirella Telles Salgueiro Barboni; Cristiane Maria Gomes Martins; Marcelo Fernandes da Costa; Jan Kremers; Dora Fix Ventura
Journal:  Doc Ophthalmol       Date:  2014-06-27       Impact factor: 2.379

9.  A comparison of ERG abnormalities in XLRS and XLCSNB.

Authors:  Keith Bradshaw; Louise Allen; Dorothy Trump; Alison Hardcastle; Nicolas George; Anthony Moore
Journal:  Doc Ophthalmol       Date:  2004-03       Impact factor: 2.379

10.  Electroretinographic findings in a patient with congenital stationary night blindness due to a novel NYX mutation.

Authors:  J Jason McAnany; Kenneth R Alexander; Nalin M Kumar; Hongyu Ying; Anastasios Anastasakis; Gerald A Fishman
Journal:  Ophthalmic Genet       Date:  2013-01-04       Impact factor: 1.803

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