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June 1994

Stargardt's Macular Dystrophy

Author Affiliations

Portland, Ore

Arch Ophthalmol. 1994;112(6):752-754. doi:10.1001/archopht.1994.01090180050033

STARGARDT'S MACULAR dystrophy (SMD) was first described in 1909 by the German ophthalmologist Karl Stargardt.1 The original report described seven patients from two families in whom a macular degeneration developed that had its onset present as a reduction in visual acuity, often with a normal fundus appearance, in the first or second decade of life. Male and female patients were equally affected. Their parents were normal and only the siblings were affected, suggesting an autosomal recessive inheritance. Eventually, atrophic macular lesions ensued, surrounded by a varying number of yellowish, deep retinal flecks. Visual fields were normal and none of the patients complained of night blindness. In a subsequent publication, Stargardt2 described mild widening of the Rayleigh equation toward the red end on Nagel anomaloscope testing, even with 20/200 visual acuity, indicating that only mild color vision loss occurs despite severe loss of central vision. Rosehr3 found that

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