What is Best disease?

Best disease, also known as vitelliform macular dystrophy, is a condition that causes macular degeneration. 

It can lead to loss of central vision and trouble with the ability to recognise colours and faces. 

Best disease usually develops in the first decade of life, but may not cause symptoms until later.  

Causes of Best disease

Best disease is a genetic condition caused by a fault in a gene known as BEST1 (also known as VMD2).

Symptoms of Best disease

Best disease usually affects central vision, causing blurring or distortion in this part of the visual field, but does not affect peripheral vision. The condition can affect each eye to different degrees, and can also cause difficulty recognising and distinguishing between colours. 

Treatment for Best disease

Currently, there are no treatments for Best disease. 

Anti-vascular endothelial growth factor medications (anti-VEGFs) are a category of drugs which stop or reduce the growth of new blood vessels. This can slow their leakage and slow down vision loss. Anti-VEGFs are not yet automatically available on the NHS for people with vitelliform macular dystrophy related CNV, but your ophthalmologist would be best placed to decide what treatment is needed in your case.

Gene therapy is currently being researched as a possible treatment for different types of inherited macular dystrophies. Gene therapy aims to replace the faulty gene with a new gene that works properly. Normal genes are injected into the retina using a harmless virus to carry the genetic material. The hope is that that affected retinal cells then begin to work properly and the damage is either stopped or reversed. A lot of this research is in early stages and may take many years before it could possibly become an established treatment, although this kind of research is a positive step forward.

There is no specific research as of yet to show that diet can help to slow down the progression of Best disease. However, a good diet full of fresh fruit and vegetables can help with eye health in general. Smoking is known to accelerate other forms of macular disease so it would be sensible to stop smoking as this possibly could help delay progression of Best disease too.

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