Stargardt disease is characterised by progressive vision loss that begins in childhood. It is the most common form of inherited juvenile macular degeneration. As it is inherited, it is also referred to as a genetic disease. In Stargardt disease, photoreceptor cells, in the central portion of the retina that allow us to see colour and sharp images, slowly die. There are several forms of Stargardt Disease that differ by gene affected, age of onset, retina pathology, severity and speed of progression.
Stargardt Disease was first described by a German ophthalmologist, Karl Stargardt, in 1909. He identified a recessively inherited macular dystrophy in seven people that went on to be known as Stargardt Disease. It is sometimes referred to as STGD, Stargardt macular dystrophy, juvenile macular degeneration or fundus flavimaculatus. It is diagnosed by clinical assessments that are sometimes followed up with genetic testing.
Stargardt Disease has an incidence of approximately 1 person affected for every 8-10,000 people. Therefore, it is a rare disease. Signs of Stargardt Disease usually, but not always, start to appear before the age of 20. A loss of visual acuity that is not helped with glasses is the main symptom. This means not being able to see fine details such as when reading, or not being able to see objects in the distance. Side or peripheral vision is usually not as affected as central vision. The range of symptoms experienced and the speed of visual deterioration can vary widely from person to person. Most people with Stargardt disease retain some level of vision and it is rare that someone would become completely blind.