Best

Best's Disease

Best's Disease (pronounced: /bɛsts diːziːz/), also known as Vitelliform Macular Dystrophy, is a rare, inherited form of macular degeneration that typically begins in childhood or adolescence.

Etymology

The disease is named after the German ophthalmologist, Friedrich Best, who first described the condition in 1905.

Definition

Best's Disease is characterized by the formation of egg-yolk like deposits, known as vitelliform lesions, beneath the macula, the part of the eye responsible for sharp, central vision. Over time, these lesions can damage the macula and lead to progressive vision loss.

Symptoms

The symptoms of Best's Disease can vary widely, even among members of the same family. They may include:

• Blurred vision
• Distorted vision (metamorphopsia)
• Difficulty with detailed tasks such as reading or recognizing faces
• Central vision loss
• Color vision defects

Diagnosis

Diagnosis of Best's Disease is typically made through a comprehensive eye examination, including a test called an electrooculogram (EOG) that measures the electrical activity of the retina. Genetic testing can also be used to confirm the diagnosis.

Treatment

There is currently no cure for Best's Disease. Treatment is aimed at managing the symptoms and may include low vision aids, occupational therapy, and vitamin A supplementation. In some cases, laser photocoagulation or photodynamic therapy may be used to treat abnormal blood vessels that can develop in the later stages of the disease.

Related Terms

• Macular degeneration
• Vitelliform lesion
• Electrooculogram
• Photodynamic therapy
• Laser photocoagulation

External links

• Medical encyclopedia article on Best
• Wikipedia's article - Best

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