Stargardt's disease

Alternative eponyms

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The most frequently encountered juvenile onset macular degeneration.

Description

Stargardt macular dystrophy is the most frequently encountered juvenile onset macular degeneration. It is a bilateral, symmetric, slowly progressive macular degeneration that begins insidiously with gradual deterioration of central vision, and ends in total blindness. Gradually, the foveal and macular reflexes disappear. On examination, the retina of an affected individual shows a macular lesion surrounded by yellow-white flecks, or spots, with irregular shapes. Onset is between 6 and 20 years of age. Stargardt macular dystrophy and fundus flavimaculatus as described by Adolphe Franceschetti (1896-1968) represent the same genetic entity. Both are transmitted as an autosomal recessive trait.

Stargardt’s report in 1909 was based upon seven persons in two families. In 1917 he documented another affected person.

Bibliography

  • K. B. Stargardt:
    Über familiäre, progressive Degeneration in der Makulagegend des Auges.
    Albrecht von Graefes Archiv für Ophthalmologie, 1909, 71: 534-550.
  • K. B. Stargardt:
    Über familiäre Degeneration in der Makulagegend des Auges, mit und ohne psychische Störungen.
    Archiv für Psychiatrie und Nervenkrankheiten, Berlin, 1917, 58: 852-857.
  • A. Franceschetti:
    Ueber tapeto-retinale Degenerationen in Kindesalter.
    In: Entwicklung und Fortschritt in der Augenheilkunde. Stuttgart, Enke Verlag, 1963: 107-120.

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