The disease of Stargardt was described in 1909 by Karl Bruno Stargardt. This pathology associates a fall of bilateral vision with particular retinal lesions. It is about most frequent of the macular Dystrophies hereditary.

Private clinic

This disease starts at young people between 6 and 15 years, which note a fall of progressive bilateral vision in connection with an atrophy of the Macula. This vision will decrease gradually until 1/10ème or 1/20ème.La disease atteind the central vision what strongly obstructs the reading (vision of précison), on the other hand the patients can move with foot normally or to even make bicycle; (control of car: impossible). With the examination one notes a macular lesion often associated with yellowish spots distributed on the bottom with eye, it is the Fundus flavimaculatus . These yellow spots “pisciform” (in the fish shape) correspond to deposits of Lipofuchsine in the cells of the pigmentary epithelium of the Rétine.

Treatment

No treatment is available

Evolution

The evolution is done towards the reduction progressive and inescapable of the vision, to reach low values, but the patients do not become blind. The conservation of the peripheral vision enables them to move and be autonomous and independent.

Genetics

• the transmission is generally autosomic recessive: the responsible Chromosome is 1
• the transmission is more rarely dominant: the responsible Chromosome is 6 and 13
• the genetic Conseil is sometimes difficult.

External bonds

• http://ophtasurf.free.fr/maladies/maladies_degenerescence.htm
• http://www.stargardt.de.vu/
• http://www.pro-retina.de
• http://www.syndromedestargardt.be.cx

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