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Sorsby's pseudoinflammatory macular dystrophy

A Hoskin, K Sehmi, A C Bird

    The British Journal of Ophthalmology
    |December 1, 1981
    PubMed
    Summary
    This summary is machine-generated.

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    This study refines the understanding of pseudoinflammatory macular dystrophy by examining family members. It details vision loss and characteristic fundus changes, differentiating it from dominant drusen.

    Area of Science:

    • Ophthalmology
    • Medical Genetics

    Background:

    • Pseudoinflammatory macular dystrophy is a rare inherited retinal disorder.
    • Previous descriptions by Sorsby and Mason established initial findings.

    Purpose of the Study:

    • To more accurately typify pseudoinflammatory macular dystrophy.
    • To identify additional affected individuals and characterize disease progression.

    Main Methods:

    • Examination of 43 additional family members.
    • Identification of affected individuals through history and clinical examination.

    Main Results:

    • Six new affected members identified by history; 7 examined.
    • Vision loss typically occurs in the 5th decade due to disciform macular degeneration.

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  • Fundus changes include drusen-like deposits, angioid streaks, and subretinal plaques.

    • Conclusions:

    • The study enhances the characterization of pseudoinflammatory macular dystrophy.
    • Distinctive fundus findings differentiate this condition from dominant drusen.