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13 July 2009
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Juvenile macular dystrophy (Image: doctor examining a boy's eye)

Juvenile macular dystrophy

Dr Rob Hicks

Juvenile macular dystrophy is similar to macular degeneration, a condition that affects older people.


What is JMD?

The macula is located at the back of the eye at the centre of the retina. It enables us to see what is directly in front of us and also allows us to see finer detail. So it plays a vital role in helping us to read, write and perform other detailed tasks. It also enables us to recognise faces and see colours.

When macular dystrophy occurs, the light-sensitive cells at the centre of the macula become damaged, and they malfunction and die.

This results in central vision becoming blurred while peripheral vision remains clear.

Symptoms

Central vision becomes blurred with symptoms similar to looking at a faded photograph. This loss of central vision makes it difficult for people affected to see what is directly in front of them and makes reading, writing, recognising faces and performing detailed tasks difficult.

Peripheral vision remains clear, so a person with juvenile macular dystrophy does not develop total blindness. There is no pain or redness of the eyes.

Causes and risk factors

The different types of juvenile macular dystrophy are caused by genetic mutations that are inherited in many different ways.

The commonest form is Stargardt's disease, which usually presents with decreased central vision within the first 20 years of life.

The second most common form of juvenile macular dystrophy is Best disease. It's usually identified during childhood or teenage years and doesn't always affect both eyes equally. Sometimes good central vision can remain in one eye.

Treatment and recovery

Juvenile macular dystrophy cannot be cured, but additional lighting and magnifiers can help to alleviate the symptoms. It's important to protect the eyes by eating healthily and avoiding ultraviolet light exposure.

This article was last medically reviewed by Dr Orlena Kerek in February 2009


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