Dominantly inherited macular dystrophy with flecks (Stargardt)
G. W. Cibis, M. Morey and D. J. Harris
A family had dominantly inherited macular dystrophy with flecks and without
evidence of major cone dysfunction. This family shows that the clinical
picture of Stargardt's disease can result from at least two different
genes: one with dominant and another with recessive heredity. Some patients
had fundus flavimaculatus flecks of Stargardt's disease; others did not. In
our opinion progressive atrophic macular (foveal) dystrophy as Stargardt's
disease without flecks is not a separate clinical entity but part of the
same genetic defect. Furthermore, a dominant hereditary pattern in patients
without flecks or major cone dysfunction is insufficient evidence for
classification of such patients as having cone dystrophy.
