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Detection Using the Multifocal Electroretinogram of Mosaic Retinal Dysfunction in Carriers of X-linked Retinitis Pigmentosa

Overview
Journal Ophthalmology
Publisher Elsevier
Specialty Ophthalmology
Date 2002 Mar 5
PMID 11874762
Citations 18
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Abstract

Purpose: To examine whether a mosaic pattern of retinal dysfunction in obligate carriers of X-linked retinitis pigmentosa (XLRP) could be observed in local electroretinographic responses obtained with the multifocal electroretinogram (mfERG).

Design: Prospective observational case series.

Participants: Five obligate carriers of XLRP (mean age, 53.2 years) were recruited into the study.

Methods: Examination of each subject included a complete ocular examination, Humphrey visual field, standard full-field electroretinogram (ERG), and mfERG testing. For the mfERG, we used a 103-scaled hexagonal stimulus array that subtended a retinal area of approximately 40 in diameter. The amplitudes and implicit times in each location for the mfERG was compared with the corresponding value determined for a group of normally sighted, age-corrected control subjects.

Main Outcome Measures: Mapping of 103 local electroretinographic response amplitudes and implicit times within the central 40 with the multifocal electroretinogram.

Results: Localized regions of reduced mfERG amplitudes and/or delayed implicit times were found in four of five carriers. In one of these four carriers, a mosaic pattern of mfERG dysfunction was present even in the absence of any clinically apparent retinal changes, retinal sensitivity losses on Humphrey field testing, or abnormal full-field cone ERG responses. However, one carrier with a typical tapetal-like reflex demonstrated no deficit on any functional tests.

Conclusions: The mfERG demonstrated patchy areas of retinal dysfunction in some carriers of XLRP. This mosaic pattern of dysfunction may be observed in some patients with a normal-appearing fundus, normal psychophysical thresholds, and normal amplitude and implicit time full-field ERG cone responses.

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