Retinitis Pigmentosa

Retinitis pigmentosa (RP) is the name for a group of eye diseases. RP causes the thin layer of tissue in the back of the eye, which is called the retina, to deteriorate. RP diseases are genetic and are passed down from one or both parents.

Retinitis pigmentosa is a genetically transmitted, progressive destruction of the retinal rods that results in atrophy of the retinal pigment epithelium and eventual blindness. As degeneration progresses from the rods to the cones, central vision loss occurs.

Retinitis pigmentosa commonly accompanies other hereditary disorders in several distinct syndromes, most commonly the Laurence-Moon-Biedl syndrome, typified by visual destruction (from retinitis pigmentosa), obesity, mental retardation, polydactyly, and hypogonadism.

Causes

Not much is known about the cause of retinitis pigmentosa, except that it is inherited. If neither of your parents have retinitis pigmentosa, at least one of them must be a carrier of the gene.

Rods - the light-sensing retinal cells that are responsible for vision in dim light - gradually deteriorate so seeing at night becomes more difficult.

Signs and Symptoms

The following are the most common symptoms of retinitis pigmentosa. However, each individual may experience symptoms differently, especially with severity and progression as the most obvious variables.

• Difficulty seeing dim lighting
• Tendency to trip easily or bump into objects when in poor lighting
• Gradual loss of peripheral vision
• Glare
• Loss of contrast sensitivity
• Eye fatigue (from straining to see)

Diagnostic tests

Electroretinography shows an absent or slower than normal retinal response time. Visual field testing (using a tangent screen) is used to detect ring scotoma.

Ophthalmoscopy may initially disclose a normal fundus and later show characteristic black pigmentary disturbances. Electrophysiologic examination (electroretinogram) is used to test the function of the rods and cones and is helpful in evaluating the extent of the disease.

Treatment

Although extensive research continues, no cure exists for retinitis pigmentosa. The use of sunglasses to protect the retina from ultraviolet light may have a vision-preserving effect.

Though controversial, recent studies have indicated that treatment with antioxidant agents (such as Vitamin A palmitate) may delay the progression of this disease.

Prevention

Genetic counseling may determine the risk of this disease occurring in a person's offspring.

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