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Stargardt’s Disease

Stargardt’s Disease is one of a number of inherited diseases of the human eye.

Primarily affecting the central region, or macula of the eyes of children and young adults, it is classified as a progressive, bilateral form of juvenile macular degeneration.

Worldwide prevalence of this disease is about one in 10,000, and although it is a common form of juvenile onset hereditary macular dystrophy,it is classified as a relatively rare disease.

As a genetic disorder, it usually follows a pattern of autosomal recessive inheritance.

Where both parents carry the faulty gene, there is a one in four chance of the child developing the disorder, affecting either male or females.

The macula plays a crucial role in visual acuity. It deals with fine detail, reading, driving, and colour perception among many other things. As a result of this disease, the macula deteriorates, leaving just blurry peripheral vision in its wake. These types of effects on the visual field can be very disruptive to the lives of children, teenagers and young adults, often striking at a most crucial time of social and physical development.

If you look at the  macula of someone with Stargardt’s Disease you will see a circular pattern  resembling ‘beaten copper’  – orange, yellow and white flecks that are a result of the dead and dying photoreceptor cells in that part of the eye.

It is striking to think how such a small area of the eye (around 6mm in diameter) can have such a devastating impact on the visual field, and how this in turn, can affect people’s quality of life.

Stargardt’s Disease, like other macular diseases such as Age Related Macular Degeneration, causes a central blind spot (scotoma) to form in the middle of the visual field.

In my research, I have examined this and many other symptoms that affect my field of view, including photophobia (sensitivity to sunlight), a throbbing appearance in the visual field which is often accompanied by a central ring of translucent colours, such as orange , purple or green (depending on what I am looking at), and very often an experience  of cortical completion, or  visual ‘filling in’ of the blind spot caused by brain activity.

There are a myriad of other visual symptoms caused by this disease, and I continue to explore them in my work.

As this post on Stargardt’s Disease is very brief and is meant to support the exploration ofentoptic symptoms on this website, I urge the reader who wants to find out more to search through some of the additional links on Stargardt’s Disease such as those listed below.

Vision Australia:’s-disease

Macular Disease Foundation Australia’sDisease_Factsheet_web.pdf

American Macular Degeneration Foundation

Foundation Fighting Blindness


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