[Dominant cystoid macular dystrophy (author's transl)].

Dominant cystoid macular dystrophy (D.C.M.D.) is characterized by a macular dystrophy but at the same time by a pigmentary dystrophy of the retinal periphery. Ultimately D.C.M.D. resembles an atypical pigmentary dystrophy, in some cases that of a pericentral retinitis pigmentosa. In an early stage the results of the EOG and darkadaptation curve reflect the process at the level of the peripheral retina, while only in a late stage we may expect some diffuse ERG pathology. D.C.M.D. might be classified as a tapetoretinal dystrophy and in particular as a form of retinitis pigmentosa with an atypical visual disturbance and an atypical fundus appearance (Leber, 1871). In doing so attention is paid to the fact that D.C.M.D. is more than a macular dystrophy. The denomination of the disease as D.C.M.D. reflects the most important features at a relatively young age.