Macular function in macular degenerations: repeatability of microperimetry as a potential outcome measure for ABCA4-associated retinopathy trials

Artur V Cideciyan; Malgorzata Swider; Tomas S Aleman; Willam J Feuer; Sharon B Schwartz; Robert C Russell; Janet D Steinberg; Edwin M Stone; Samuel G Jacobson

doi:10.1167/iovs.11-8415

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Macular function in macular degenerations: repeatability of microperimetry as a potential outcome measure for ABCA4-associated retinopathy trials

Journal article

Open access

Peer reviewed

Macular function in macular degenerations: repeatability of microperimetry as a potential outcome measure for ABCA4-associated retinopathy trials

Artur V Cideciyan, Malgorzata Swider, Tomas S Aleman, Willam J Feuer, Sharon B Schwartz, Robert C Russell, Janet D Steinberg, Edwin M Stone and Samuel G Jacobson

Investigative ophthalmology & visual science, Vol.53(2), pp.841-852

02/2012

DOI: 10.1167/iovs.11-8415

PMCID: PMC3317423

PMID: 22247458

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Abstract

To measure macular visual function in patients with unstable fixation, to define the photoreceptor source of this function, and to estimate its test-retest repeatability as a prerequisite to clinical trials. Patients (n = 38) with ABCA4-associated retinal degeneration (RD) or with retinitis pigmentosa (RP) were studied with retina-tracking microperimetry along the foveo-papillary profile between the fovea and the optic nerve head, and point-by-point test-retest repeatability was estimated. A subset with foveal fixation was also studied with dark-adapted projection perimetry using monochromatic blue and red stimuli along the horizontal meridian. Macular function in ABCA4-RD patients transitioned from lower sensitivity at the parafovea to higher sensitivity in the perifovea. RP patients had the inverse pattern. Red-on-red microperimetric sensitivities successfully avoided ceiling effects and were highly correlated with absolute sensitivities. Point-by-point test-retest limits (95% confidence intervals) were ±4.2 dB; repeatability was not related to mean sensitivity, eccentricity from the fovea, age, fixation location, or instability. Repeatability was also not related to the local slope of sensitivity and was unchanged in the parapapillary retina. Microperimetry allows reliable testing of macular function in RD patients without foveal fixation in longitudinal studies evaluating natural disease progression or efficacy of therapeutic trials. A single estimate of test-retest repeatability can be used to determine significant changes in visual function at individual retinal loci within diseased regions that are homogeneous and those that are heterogeneous and also in transition zones at high risk for disease progression.

Visual Fields - physiology

Retinal Degeneration - diagnosis

Follow-Up Studies

Humans

Middle Aged

Male

Macular Degeneration - diagnosis

Dark Adaptation

Young Adult

ATP-Binding Cassette Transporters - genetics

ATP-Binding Cassette Transporters - metabolism

Adult

Female

Child

Macular Degeneration - physiopathology

Retinal Degeneration - genetics

Tomography, Optical Coherence

Clinical Trials as Topic

Retinal Degeneration - physiopathology

Visual Acuity

DNA - genetics

Macular Degeneration - genetics

Adolescent

Visual Field Tests

Mutation

Details

Title: Subtitle: Macular function in macular degenerations: repeatability of microperimetry as a potential outcome measure for ABCA4-associated retinopathy trials
Creators: Artur V Cideciyan - Scheie Eye Institute, Department of Ophthalmology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA. cideciya@mail.med.upenn.edu
Malgorzata Swider
Tomas S Aleman
Willam J Feuer
Sharon B Schwartz
Robert C Russell
Janet D Steinberg
Edwin M Stone
Samuel G Jacobson
Resource Type: Journal article
Publication Details: Investigative ophthalmology & visual science, Vol.53(2), pp.841-852
DOI: 10.1167/iovs.11-8415
PMID: 22247458
PMCID: PMC3317423
NLM abbreviation: Invest Ophthalmol Vis Sci
ISSN: 0146-0404
eISSN: 1552-5783
Publisher: United States
Grant note: R01 EY013203 / NEI NIH HHS EY 013203 / NEI NIH HHS
Language: English
Date published: 02/2012
Academic Unit: Iowa Neuroscience Institute; Ophthalmology and Visual Sciences
Record Identifier: 9983979981002771

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