Retinitis pigmentosa (RP) is a generic term for a group of disorders characterized by hereditary diffuse usually bilaterally symmetrical progressive dysfunction, cell loss and eventual atrophy of retina. Initially photoreceptors are involved and subsequently inner retina is damaged. Although both rods and cones are involved, damage to the rods is predominant. RP may be seen in isolation (Typica...

Arden gratings were used to measure contrast sensitivity in 40 patients with retinitis pigmentosa whose Snellen visual acuity was 6/12 or better. When compared with a group of 30 normal subjects the patients with retinitis pigmentosa had substantially decreased contrast sensitivity, especially at high frequencies. The Arden grating test appeared to be a sensitive test of abnormal central visual...

PURPOSE The purpose of this paper is to report 14 new cases of unilateral retinitis pigmentosa and three new cases of cone-rod dystrophy and to compare the similarities and dissimilarities to those found in the bilateral forms of these disorders. METHODS A total of 272 cases of retinitis pigmentosa and 167 cases of cone-rod dystrophy were studied by corneal full field electroretinograms and e...

To provide a tool for quantifying the effects of retinitis pigmentosa (RP) seen on spectral domain optical coherence tomography images, an automated layer segmentation algorithm was developed. This algorithm, based on dual-gradient information and a shortest path search strategy, delineates the inner limiting membrane and three outer retinal boundaries in optical coherence tomography images fro...

Recent advances in molecular genetic studies have revealed many of the causative genes of retinitis pigmentosa (RP). These achievements have provided clues to the mechanisms of photoreceptor degeneration in RP. Apoptosis is known to be a final common pathway in RP and, therefore, a possible therapeutic target for photoreceptor rescue. However, apoptosis is not a single molecular cascade, but co...

Royal College of Surgeons (RCS) rats are a well established animal model of inherited retinitis pigmentosa (RP). Using RCS rats we examined the distribution of nerve growth factor (NGF) and brain derived neurotrophic factor (BDNF) in the visual cortex, geniculate nucleus and retina at three different postnatal ages. It was found that the retina of rats with RP expresses low amounts of NGF and B...

Leber congenital amaurosis (LCA) and juvenile retinitis pigmentosa (RP) are the most common hereditary causes of visual impairment in infants and children. Using homozygosity mapping, we narrowed down the critical region of the LCA3 locus to 3.8 Mb between markers D14S1022 and D14S1005. By direct Sanger sequencing of all genes within this region, we found a homozygous nonsense mutation in the S...

Retinitis pigmentosa (RP) is a class of diseases that leads to progressive degeneration of the retina. Experimental approaches to gene therapy for the treatment of inherited retinal dystrophies have advanced in recent years, inclusive of the safe delivery of genes to the human retina. This review is focused on the development of gene therapy for RP using recombinant adenoassociated viral vector...