Stargardt disease is a severe form of inherited juvenile macular degeneration and characterized by discrete yellowish round flecks around the macula at the level of the retinal pigment epithelium (RPE). The disease usually begins in late childhood with a progressive loss of central vision, although in some cases the earliest manifestations begin to appear only in to early adulthood. In its earliest stages, visual acuity may appear to be normal, with patients experiencing only dark adaptation problems. Progressively, patients begin to experience central vision loss, and in advanced cases, macular atrophy, color vision impairment, and severe loss of visual acuity classified as legal blindness. During this progression, fatty deposits, called lipofuscin, accumulate around the RPE in the macula. In the early stages, this accumulation consists of only a few yellowish flecks, and resembles a snail's slime. However, in the later stages, the macula has a bull's eye pattern, accompanied with atrophy of the RPE.
The relatively mild manner in which Stargardt disease first manifests itself renders it very difficult to diagnose in its early stages. Patients typically show a normal ERG, with a normal or slightly affected EOG.
Visualization of a dark choroid using fluorescein angiography is used in the diagnosis of Stargardt disease. In any case, there is no treatment currently available. Low-vision aids may be prescribed to some patients. Some studies show that wearing UV-protective glasses and avoiding exposure to sunlight might slow disease progression.
Al-Merjan et al. (2005) presented the causes and incidence rates of disorders leading to blindness and low vision in Kuwait, based on the data collected by the Visual Disability Committee in a 5-year period from 2000 to 2004. Of the 826,083 people (407,871 males) registered with blindness and low vision, 39% were below the age of 20-years, 32% were between the ages of 21 and 40-years, while only about 10% were over 60-years of age. Stargardt disease was found to occur with an overall incidence rate of 0.70 per 100,000 individuals. The incidence varied between males (0.88) and females (0.52).