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Retinitis pigmentosa (RP) is one name for a large group of inherited vision conditions that causes progressive degeneration of the retina of the eyes. Peripheral (side) vision gradually decreases and is eventually lost. Central vision is usually preserved until late in these conditions. Some forms of RP may be associated with deafness, kidney disease, central nervous system conditions, metabolic conditions, and chromosomal abnormalities. Symptoms are generally noticed between the ages of 10 and 40. The rate and extent of progression is widely variable. When other members of a family are affected, the rates of progression are usually similar within that family. Some individuals with retinitis pigmentosa are also born deaf (Usher syndrome type I), or hearing-impaired (Usher syndrome type II). Laurence-Moon/Bardet Biedl syndrome is an inherited condition characterised by lower hormone production by the testes or ovaries, retinitis pigmentosa, mental retardation, and paralysis of the lower part of the body accompanied by involuntary muscle contractions (spastic paraplegia).
Retinitis pigmentosa is a group of hereditary progressive conditions that may be inherited as autosomal recessive, autosomal dominant or X-linked recessive conditions. In about half of the cases of retinitis pigmentosa, there is no family history of the condition. |