Retinitis Pigmentosa is a genetic disorder that causes progressive vision loss. The light sensing cells of the retina gradually deteriorate. Patients at first lose night vision as a result of the death of rod photoreceptors which starts in childhood. Even low light causes difficulties. Blind spots develop in peripheral vision and later the disease affects central vision caused by degeneration of rod cones. Patients are limited in their reading, driving, using a computer and visual recognition of people.
The gene mutations that occur most frequently with retinitis pigmentosa are RHO,the most common autosomal dominant; RP2, RPGR for most cases of X-linked form and USH2A, the most common of autosomal recessive form.
The condition usually is limited to vision loss but can be associated in Usher syndrome that has vision and hearing loss. It may also be associated with other syndromes; some of which cause neuropathy and ataxia.