Literature DB >> 16565412

Perifoveal function in patients with North Carolina macular dystrophy: the importance of accounting for fixation locus.

William Seiple1, Janet P Szlyk, Jennifer Paliga, Maurice F Rabb.   

Abstract

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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Year:  2006        PMID: 16565412     DOI: 10.1167/iovs.05-0659

Source DB:  PubMed          Journal:  Invest Ophthalmol Vis Sci        ISSN: 0146-0404            Impact factor:   4.799


  3 in total

Review 1.  Allelic and phenotypic heterogeneity in ABCA4 mutations.

Authors:  Tomas R Burke; Stephen H Tsang
Journal:  Ophthalmic Genet       Date:  2011-04-21       Impact factor: 1.803

2.  Preferred retinal locus in macular disease: characteristics and clinical implications.

Authors:  Vivienne C Greenstein; Rodrigo A V Santos; Stephen H Tsang; R Theodore Smith; Gaetano R Barile; William Seiple
Journal:  Retina       Date:  2008-10       Impact factor: 4.256

3.  A novel duplication involving PRDM13 in a Turkish family supports its role in North Carolina macular dystrophy (NCMD/MCDR1).

Authors:  Kent W Small; Stijn Van de Sompele; Karen Nuytemans; Andrea Vincent; Ozge Ozalp Yuregir; Emine Ciloglu; Cahfer Sariyildiz; Toon Rosseel; Jessica Avetisjan; Nitin Udar; Jeffery M Vance; Margaret A Pericak-Vance; Elfride De Baere; Fadi S Shaya
Journal:  Mol Vis       Date:  2021-09-01       Impact factor: 2.367

  3 in total

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