Foveal or parafoveal white dots or flecks are commonly associated with a variety of disorders, including Stargardt-fundus flavimaculatus (initially considered in 2 of the patients described in the present study), retinitis punctata albescens, Goldmann-Favre syndrome, Wagner vitreoretinal dystrophy,yellow dot dystrophy, and other forms of macular dystrophy.19 However,none of those disorders typically manifest an electronegative ERG configuration.Fundus albipunctatus often shows a myriad of symmetrical round white flecks,with the greatest concentration in the midperiphery, but those patients always have night blindness, not a feature of the present cohort. The drusenlike multiple white spots described in the present patients were sometimes associated with atrophic changes at the macula, a common finding in older patients with XLRS, but could be the only fundus abnormality. The spots were not typical of those associated with age-related maculopathy. A similar matrix of extensive white flecks has been described in 2 individuals with juvenile retinoschisis20 and more recently in 2 additional patients with confirmed RS1 defects, including a 214G→A defect.21 Those flecks were associated with typical star-shaped macular lesions (foveal schisis)and did not resemble the drusenlike white dots described in the present study,even in a patient carrying an identical mutation (patient 5). Macular white dots have not previously been described in individuals carrying the previously reported RS1 alleles: 305G→A, 214G→A, 579dupC,325G→T, IVS1+2T→C, and exon 1 deletion.4 The 206T→C mutation in patient 2 is a novel mutation.