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ROHRSCHNEIDER KLAUS MD; BLANKENAGEL, ANITA MD; KRUSE, FRIEDRICH E MD; FENDRICH, THOMAS PhD; VOLCKER, HANS E MD
Retina: May 1998
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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.

From the Department of Ophthalmology. University of Heidelberg. Germany.

Supported in part by a grant from the Deutsche Forschungsgemeinschaft DFG (Vo 437/2-1).

The authors have no proprietary interest in any aspect of this study.

Reprint requests: Dr. Klaus Rohrschneider, Univ.-Augenklinik. Im Neuenheimer Feld 400. D-69120 Heidelberg, Germany.

© The Ophthalmic Communications Society, Inc.