Macular function testing in a German pedigree with North Carolina macular dystrophy

Background: Our purpose was to investigate central visual function in North Carolina macular dystrophy (MCDR1). A German family with genetically proven MCDR1 was followed for more than 20 years. Methods: In addition to routine clinical examinations, static and kinetic fundus perimetry was performed...

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Hauptverfasser: Rohrschneider, Klaus (VerfasserIn) , Blankenagel, Anita (VerfasserIn) , Kruse, Friedrich E. (VerfasserIn) , Fendrich, Thomas (VerfasserIn) , Völcker, Hans Eberhard (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 1998
In: Retina
Year: 1998, Jahrgang: 18, Heft: 5, Pages: 453-459
ISSN:1539-2864
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://journals.lww.com/retinajournal/abstract/1998/05000/macular_function_testing_in_a_german_pedigree_with.13.aspx
Volltext
Verfasserangaben:$hKlaus Rohrschneider, Anita Blankenagel, Friedrich E. Kruse, Thomas Fendrich, Hans E. Volcker

MARC

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520 |a Background: Our purpose was to investigate central visual function in North Carolina macular dystrophy (MCDR1). A German family with genetically proven MCDR1 was followed for more than 20 years. Methods: In addition to routine clinical examinations, static and kinetic fundus perimetry was performed with the scanning laser ophthalmoscope. The center of fixation was determined.Results: Visual acuity was 0.6 or better in all but one eye of seven members of this family and did not markedly change during follow-up. Fundus examination confirmed that the fixation was at the nasal edge of the central scar in five affected eyes with reduced stability of fixation. One eye had a prominent gliotic membrane, and fixation was within this area. Conclusions: Patients had nearly normal visual acuity even though they presented with deep central scotomas and a shift of the center of fixation away from the fovea. Because MCDR1 has good long-term functional prognosis, there might be an early transdifferentiation of the new locus of fixation. 
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