title: [Dominant cystoid macular dystrophy (author's transl)].
abstract: 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.
mesh_terms: D003937:Diagnosis, Differential; D005799:Genes, Dominant; D006801:Humans; D008268:Macular Degeneration / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012162:Retinal Degeneration / Q000235:genetics*; D012174:Retinitis Pigmentosa / Q000175:diagnosis
vernacular_title: Dégénérescence maculaire cystoïde dominante (D.M.C.D.)