[Albescent punctate retinopathy with central pigment epithelium atrophy]

A 45-year-old Italian patient suffering from punctate retinopathy associated with a central atrophy of the retinal pigment epithelium is described. He has suffered from nyctalopia since early childhood, and over the last 3 years has become increasingly susceptible to dazzling. Funktion tests revealed pathologic monofunctional dark adaptation of cones, centrocecal scotoma, a pathologic electro-oculogram and residual a-waves at high light stimulus intensities in the electroretinogram. An autosomal-recessive mode of inheritance is assumed, as 4 of the patient's 9 siblings have the same signs.