Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Characterization of Rcbtb1 Knockout Mice and Evaluation of AAV2-RCBTB1 Gene Replacement Therapy.

Translational vision science & technology·2026
Same author

Inpatient versus outpatient management of bilateral optic nerve head elevation presentation to a tertiary emergency department.

BMJ neurology open·2026
Same author

Radial outer retina reflectivity (RORR) sign in LAMP2-associated retinopathy.

Acta ophthalmologica·2026
Same author

The longitudinal relationship between carotid arterial disease and retinopathy complicating type 2 diabetes: The Fremantle diabetes study phase II.

Journal of diabetes and its complications·2026
Same author

Corrigendum to Peripapillary Retinoschisis: The Expanded Spectrum and New Insights From Multimodal Imaging. Am J Ophthalmol. 2026;282:26-40.

American journal of ophthalmology·2026
Same author

Gain of function in BEST1: photoreceptor changes and myopia in autosomal dominant vitreoretinochoroidopathy.

Ophthalmic genetics·2026

Related Experiment Video

Updated: Sep 5, 2025

Regenerative Therapy by Suprachoroidal Cell Autograft in Dry Age-related Macular Degeneration: Preliminary In Vivo Report
10:24

Regenerative Therapy by Suprachoroidal Cell Autograft in Dry Age-related Macular Degeneration: Preliminary In Vivo Report

Published on: February 12, 2018

10.3K

Longitudinal Analysis of Functional and Structural Outcome Measures in PRPH2-Associated Retinal Dystrophy.

Rachael C Heath Jeffery1, Jennifer A Thompson2, Tina M Lamey3

  • 1Centre for Ophthalmology and Visual Science (incorporating Lions Eye Institute), The University of Western Australia, Australia; Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, East Melbourne, Victoria, Australia.

Ophthalmology. Retina
|July 6, 2022
PubMed
Summary

This study tracked disease progression in PRPH2-associated retinal dystrophy using total lesion size (TLS) and autofluorescence. Total macular volume (TMV) appears more sensitive than macular sensitivity (MMS) for monitoring disease advancement.

Keywords:
AutofluorescenceClinical trial endpointInherited retinal diseaseMacular dystrophyOCTPRPH2-associated retinal dystrophyPattern dystrophyRetinal dystrophyRetinal imaging

More Related Videos

Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research
10:10

Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research

Published on: November 2, 2018

9.4K
Author Spotlight: Understanding Age-Related Macular Degeneration Pathophysiology with QAF Workflow
08:54

Author Spotlight: Understanding Age-Related Macular Degeneration Pathophysiology with QAF Workflow

Published on: May 26, 2023

1.6K

Related Experiment Videos

Last Updated: Sep 5, 2025

Regenerative Therapy by Suprachoroidal Cell Autograft in Dry Age-related Macular Degeneration: Preliminary In Vivo Report
10:24

Regenerative Therapy by Suprachoroidal Cell Autograft in Dry Age-related Macular Degeneration: Preliminary In Vivo Report

Published on: February 12, 2018

10.3K
Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research
10:10

Ultrahigh Resolution Mouse Optical Coherence Tomography to Aid Intraocular Injection in Retinal Gene Therapy Research

Published on: November 2, 2018

9.4K
Author Spotlight: Understanding Age-Related Macular Degeneration Pathophysiology with QAF Workflow
08:54

Author Spotlight: Understanding Age-Related Macular Degeneration Pathophysiology with QAF Workflow

Published on: May 26, 2023

1.6K

Area of Science:

  • Ophthalmology
  • Genetics
  • Retinal Diseases

Background:

  • PRPH2-associated retinal dystrophy is a rare inherited condition affecting vision.
  • Understanding disease progression is crucial for patient management and therapeutic development.

Purpose of the Study:

  • To quantify the rates of disease progression in PRPH2-associated retinal dystrophy.
  • To evaluate changes in total lesion size (TLS), decreased autofluorescence (DAF) area, total macular volume (TMV), and mean macular sensitivity (MMS).

Main Methods:

  • A retrospective chart review was conducted on patients with PRPH2 variants.
  • Serial ultrawide-field (UWF) fundus autofluorescence (FAF), OCT, and microperimetry data were analyzed over at least one year.
  • Linear correlation was used to determine the rate of change for key outcome measures.

Main Results:

  • Over a mean follow-up of 4.7 years, TLS and DAF radius showed slow expansion.
  • Total macular volume (TMV) decreased by -0.071 mm³/year, with strong interocular correlation.
  • Mean macular sensitivity (MMS) showed variable changes, with a mean decline of -0.10 dB/year, and was often subnormal despite normal TMV.

Conclusions:

  • Ultrawide-field fundus autofluorescence (UWF-FAF) derived measurements like TLS and DAF demonstrate slow progression.
  • Total macular volume (TMV) may be a more sensitive indicator of disease progression in PRPH2-associated retinal dystrophy compared to mean macular sensitivity (MMS).