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

A Pinckers, J G Notting, F Lion

    Journal Francais D'Ophtalmologie
    |February 1, 1978
    PubMed
    Summary

    Dominant cystoid macular dystrophy (D.C.M.D.) affects both the macula and retinal periphery, resembling atypical retinitis pigmentosa. Early signs appear in peripheral vision tests, progressing to diffuse ERG changes later.

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    Area of Science:

    • Ophthalmology
    • Medical Genetics
    • Retinal Diseases

    Background:

    • Dominant cystoid macular dystrophy (D.C.M.D.) presents a complex clinical picture.
    • It involves both macular and peripheral retinal abnormalities.

    Observation:

    • D.C.M.D. can mimic atypical pigmentary dystrophies, including pericentral retinitis pigmentosa.
    • Early electrophysiological findings (EOG, dark adaptation) indicate peripheral retinal involvement.

    Findings:

    • Late-stage D.C.M.D. may exhibit diffuse electroretinogram (ERG) pathology.
    • The condition is classified as a tapetoretinal dystrophy, a form of retinitis pigmentosa with unique visual and fundus features.

    Implications:

    • D.C.M.D. is more than solely a macular dystrophy.
    • The name D.C.M.D. highlights its key features presenting in younger individuals.
    • Understanding D.C.M.D. aids in diagnosing and managing this rare inherited retinal disorder.

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