![]() More than 20 of these genes are associated with the autosomal dominant form of the disorder. Mutations in more than 60 genes are known to cause nonsyndromic retinitis pigmentosa. Retinitis pigmentosa is also a feature of several other genetic syndromes, including Bardet-Biedl syndrome Refsum disease and neuropathy, ataxia, and retinitis pigmentosa (NARP). The most common form of syndromic retinitis pigmentosa is Usher syndrome, which is characterized by the combination of vision loss and hearing loss beginning early in life. These forms of the disease are described as syndromic. Less commonly, retinitis pigmentosa occurs as part of syndromes that affect other organs and tissues in the body. Researchers have identified several major types of nonsyndromic retinitis pigmentosa, which are usually distinguished by their pattern of inheritance: autosomal dominant, autosomal recessive, or X-linked. When the disorder occurs by itself, it is described as nonsyndromic. The signs and symptoms of retinitis pigmentosa are most often limited to vision loss. In adulthood, many people with retinitis pigmentosa become legally blind. ![]() The disease progresses over years or decades to affect central vision, which is needed for detailed tasks such as reading, driving, and recognizing faces. Over time, these blind spots merge to produce tunnel vision. Later, the disease causes blind spots to develop in the side (peripheral) vision. Problems with night vision can make it difficult to navigate in low light. The first sign of retinitis pigmentosa is usually a loss of night vision, which becomes apparent in childhood. In people with retinitis pigmentosa, vision loss occurs as the light-sensing cells of the retina gradually deteriorate. These disorders affect the retina, which is the layer of light-sensitive tissue at the back of the eye. Retinitis pigmentosa is a group of related eye disorders that cause progressive vision loss.
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