Retinitis Pigmentosa

Retinitis Pigmentosa

Retinitis Pigmentosa is the name given to a group of inherited eye disorders that affect the retina. The retina has rod shaped cells responsible for night vision and seeing in the dim light. The cone-shaped cells are responsible for color vision and brightness Retinitis Pigmentosa has been proven to be progressive, destructive, hereditary disease that reduces the function of rod-shaped cells. Retinitis Pigmentosa can eventually lead to severe vision loss and blindness.

Retinitis Pigmentosa is a term created by Donders in 1855. It is not just one disease but a group of retinal diseases with common attributes.

There are three different methods by which Retinitis Pigmentosa can be inherited. The most common form of inheritance is called

recessive. In this type both parents carry the gene. They are unaffected; however, their children have a twenty-five percent chance of inheriting this devastating disease. The second most common cause is X-linked. In the X-linked the females carry the gene. When she marries an unaffected male the chances of either male of female children having the disease will have fifty-fifty chance. There is also a dominant form which can strike every generation of the family.

The most common feature of all forms of Retinitis Pigmentosa is a gradual degeneration of the rods and cones. Night blindness, low and dark light, adaptation, poor contrast, sensitivity, and tunnel vision, as though they see the world through a straw, are all symptoms. Hearing impairment occurs frequently in Retinitis Pigmentosa patients.

Some tests to diagnose Retinitis Pigmentosa are venereal disease research laboratory test, fluorescent treponemal antibody absorption tests, antibodies, and electroretinogram or EPG. This is the most critical diagnostic test because it provides an objective measure of rod and cone function across the retina. Some treatments for Retinitis Pigmentosa are Vitamin A, beta-carotene, Acetazolamide, Diltiazem, and Lutein. Surgeries include cataract extraction, transplantation,

Retinal prosthesis, and intravitreal or sub retinal gene therapy.

The causes of retinal degeneration are so numerous that it is unlikely that a single treatment