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(Investigative Ophthalmology and Visual Science. 2006;47:1703-1709.)
© 2006 by The Association for Research in Vision and Ophthalmology, Inc.
DOI:  10.1167/iovs.05-0659
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Perifoveal Function in Patients with North Carolina Macular Dystrophy: The Importance of Accounting for Fixation Locus

William Seiple,1,2 Janet P. Szlyk,2,3 Jennifer Paliga,2 and Maurice F. Rabb2

1From the Department of Ophthalmology, New York University School of Medicine, New York, New York; the 2Department of Ophthalmology and Visual Sciences, University of Illinois at Chicago, Chicago, Illinois; and the 3Research and Development Service, Jesse Brown Veterans Administration Medical Center, Chicago, Illinois.

PURPOSE. To quantify the extent of visual function losses in patients with North Carolina Macular Dystrophy (NCMD) and to demonstrate the importance of accounting for eccentric fixation when making comparisons with normal data.

METHODS. Five patients with NCMD who were from a single family were examined. Multifocal electroretinograms (mfERGs) and psychophysical assessments of acuity and luminance visual field sensitivities were measured throughout the central retina. Comparisons of responses from equivalent retinal areas were accomplished by shifting normal templates to be centered at the locus of fixation for each patient.

RESULTS. Losses of psychophysically measured visual function in patients with NCMD extend to areas adjacent to the locations of visible lesions. The multifocal ERG amplitude was reduced only within the area of visible lesion. Multifocal ERG implicit times were delayed throughout the entire central retinal area assessed.

CONCLUSIONS. ERG timing is a sensitive assay of retinal function, and our results indicate that NCMD has a widespread effect at the level of the mid and outer retina. The findings also demonstrated that it is necessary to account for fixation locus and to ensure that equivalent retinal areas are compared when testing patients with macular disease who have eccentric fixation.






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