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Stargardt-Behr disease
Also known as:
Behr's disease
Behr's syndrome II
Associated persons:
Carl Behr
Karl Bruno Stargardt
Description:
Familial heredomacular degeneration occurring in adult and presenile forms. The presenile form has onset at about 20 years of age, the senile form between 40 and 90 years of age. It is marked by central atrophic chorioretinitis with pigmentary degenerative changes and peripheral retinal lesions. The intensity of the pigment epithelial changes and the degree of visual loss vary from family to family. Usually transmitted as an autosdomal recessive trait.
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.
- C. Behr:
Die Heredodegeneration der Makula.
Klinische Monatsblätter für Augenheilkunde, Stuttgart, 1920, 69: 469-505.
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