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Retinitis Pigmentosa definition
Retinitis Pigmentosa is the collective term for a group of hereditary disorders that result in progressive loss of vision. Retinitis pigmentosa generally begins with diminished night vision, as the degeneration affects primarily the rods (photoreceptors responsible for vision in dim light and for peripheral vision). Eventually the condition progresses to rods, and then cones, throughout the RETINA. In most people symptoms begin between the ages of 10 and 30, with complete loss of vision by around age 40.
When viewed through the ophthalmoscope, the areas of degeneration appear darker than the surrounding areas of retina. The diagnostic path may also include a DARK ADAPTATION TEST and ELECTRORETINOGRAPHY. There is no known treatment. Several INHERITANCE PATTERNS are responsible for retinitis pigmentosa; doctors recommend GENETIC TESTING and GENETIC COUNSELING for family members when there is a diagnosis of this condition. Retinitis pigmentosa also may accompany a number of other hereditary syndromes.