Literature DB >> 35711968

Frequency Doubling Technology Visual Field Loss in Fabry Subjects Related to Retinal Ganglion Cell Function as Explored by ERG and OSOME.

Langis Michaud1, Marie-Lou Garon1, Pierre Forcier1, Vasile Diaconu1.   

Abstract

Purpose: This study aims to evaluate potential causes of FDT visual field loss in a selected group of Fabry subjects. Patients and
Methods: This is a pilot observational study. Subjects were assessed during 2 visits. The following tests were performed: visual acuity, tonometry, optical coherence tomography (OCT) optic nerve scan, frequency doubling time (FDT) and threshold (SAP) VF, ERG, and Online Spectro-reflectometry Oxygenation Measurement in the Eye (OSOME). Results are compared across visits and, when indicated, interpreted against those collected on non-Fabry population matched for age and sex.
Results: The study population was composed of 3 males (34.3 ± 8.9 y.o.) and 5 females (46.4 ± 6.5 y.o). For all subjects, BCVA remained 6/6 OU throughout the study and OCT optic nerve scans were normal. FDT showed a defect in at least 1 quadrant for all participants, in contrast with SAP. FDT PSD value was found different vs SAP. For ERG, the i-wave (52.1 + 2.7 ms) and B-waves (31.6 ± 2.1 ms) peak times were significantly longer compared to a non-Fabry population (p < 0.05). Overall blood oxygenation varied from 61.3% ± 4% to 68.1% ± 4% at the second visit, suggesting a loss of capillary perfusion. Blood volume varied based on location (superior/inferior), eye tested (OD/OS) and time (visit 1/2). The range of values exceeds normal subjects findings (p < 0.05). Blood volume was correlated to FDT PSD value for the superior area of the optic nerve.
Conclusion: The results suggest that Fabry subjects present FDT deficits and abnormal ERG patterns that may be explained by a retinal dysfunction affecting retinal ganglion cells (RGCs), second to vascular alterations.
© 2022 Michaud et al.

Entities:  

Keywords:  Fabry disease; OSOME; electroretinography; retinal ganglion cells

Year:  2022        PMID: 35711968      PMCID: PMC9192351          DOI: 10.2147/OPTH.S356245

Source DB:  PubMed          Journal:  Clin Ophthalmol        ISSN: 1177-5467


  43 in total

1.  Variability components of standard automated perimetry and frequency-doubling technology perimetry.

Authors:  P G Spry; C A Johnson; A M McKendrick; A Turpin
Journal:  Invest Ophthalmol Vis Sci       Date:  2001-05       Impact factor: 4.799

2.  The ocular manifestations in Fabry's disease.

Authors:  N A Sher; R D Letson; R J Desnick
Journal:  Arch Ophthalmol       Date:  1979-04

3.  The role and interpretation of pilot studies in clinical research.

Authors:  Andrew C Leon; Lori L Davis; Helena C Kraemer
Journal:  J Psychiatr Res       Date:  2010-10-28       Impact factor: 4.791

4.  Fabry's disease: ocular findings in a female carrier. A light and electron microscopy study.

Authors:  T A Weingeist; F C Blodi
Journal:  Arch Ophthalmol       Date:  1971-02

5.  Ocular pathology of Fabry's disease in a hemizygous male following renal transplantation.

Authors:  E M Riegel; K S Pokorny; A H Friedman; J Suhan; R H Ritch; R J Desnick
Journal:  Surv Ophthalmol       Date:  1982 Mar-Apr       Impact factor: 6.048

6.  Subclinical optic neuropathy in Fabry disease.

Authors:  Susanne Pitz; Kathrin Grube-Einwald; Giulia Renieri; Jörg Reinke
Journal:  Ophthalmic Genet       Date:  2009-12       Impact factor: 1.803

7.  Frequency doubling technology perimetry abnormalities as predictors of glaucomatous visual field loss.

Authors:  Felipe A Medeiros; Pamela A Sample; Robert N Weinreb
Journal:  Am J Ophthalmol       Date:  2004-05       Impact factor: 5.258

8.  Longitudinal study on ocular manifestations in a cohort of patients with Fabry disease.

Authors:  Langis Michaud
Journal:  PLoS One       Date:  2019-06-27       Impact factor: 3.240

Review 9.  Suitability of the Visual Field Index according to Glaucoma Severity.

Authors:  Marina Cc Sousa; Luis G Biteli; Syril Dorairaj; Jessica S Maslin; Mauro T Leite; Tiago S Prata
Journal:  J Curr Glaucoma Pract       Date:  2016-02-02

Review 10.  Nutrition and Vascular Supply of Retinal Ganglion Cells during Human Development.

Authors:  Paul Rutkowski; Christian Albrecht May
Journal:  Front Neurol       Date:  2016-04-07       Impact factor: 4.003

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