A Cross-Sectional and Longitudinal Study of Retinal Sensitivity in RPE65-Associated Leber Congenital Amaurosis.

Investigative Ophthalmology & Visual Science
Neruban KumaranMichel Michaelides

Abstract

RPE65-associated Leber congenital amaurosis (RPE65-LCA) is an early-onset severe retinal dystrophy associated with progressive visual field loss. Phase I/II and III gene therapy trials have identified improved retinal sensitivity but little is known about the natural history of retinal sensitivity in RPE65-LCA. A total of 19 subjects (aged 9 to 23 years) undertook monocular full-field static perimetry of which 13 subjects were monitored longitudinally. Retinal sensitivity was measured as mean sensitivity (MS) and volumetrically quantified (in decibel-steradian) using visual field modeling and analysis software for the total (VTOT), central 30° (V30) and central 15° (V15) visual field. Correlation was evaluated between retinal sensitivity and age, best-corrected visual acuity (BCVA), contrast sensitivity, vision-related quality of life, and genotype. Test-retest reliability was also investigated. V30 was identified to have a strong, weak, and moderate correlation with age, BCVA and contrast sensitivity respectively. Furthermore, V30 was identified as having a weak linear relationship with the mobility and independence domains of the vision-related quality of life questionnaire. Longitudinal analysis demonstrated a slow loss of r...Continue Reading

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Citations

Apr 30, 2020·Investigative Ophthalmology & Visual Science·Neruban KumaranMichel Michaelides
Sep 23, 2020·Investigative Ophthalmology & Visual Science·Michalis GeorgiouMichel Michaelides
Sep 22, 2020·Translational Vision Science & Technology·Neruban KumaranGary S Rubin
Dec 6, 2020·Molecular Therapy : the Journal of the American Society of Gene Therapy·Albert M MaguireTomas S Aleman
Dec 15, 2020·Orphanet Journal of Rare Diseases·Anna Skorczyk-WernerMaciej Robert Krawczyński
Mar 11, 2021·Clinical & Experimental Optometry : Journal of the Australian Optometrical Association·Fleur O'HareLauren N Ayton
Jun 6, 2021·Orphanet Journal of Rare Diseases·Andrea SodiUNKNOWN Italian IRD Working Group
Jul 29, 2021·American Journal of Ophthalmology·Xuan-Thanh-An NguyenCamiel J F Boon

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Software Mentioned

Mutalyzer
Adaptive Threshold Estimation ( GATE )
VFMA
FST
JMP
visual field modeling and analysis ( VFMA )
German
EyeSuite
Stata
StataCorp

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