Retinitis Pigmentosa, Decreased Vision in Neuro-ophthalmology

Synonyms

Primary pigmentary retinal degeneration; Progressive pigmentary retinopathy; Rod-cone dystrophy

Definition

Retinitis pigmentosa (RP) is a class of genetic degenerative retinal disorders that affect the photoreceptor cells (rods and cones) as well as the retinal pigment epithelium. Over 35 different genes have been identified, and modes of inheritance for RP include autosomal recessive, autosomal dominant, and X-linked transmission. This family of disorders typically results in painless, bilateral, and progressive vision loss due to dysfunction and eventual atrophy of rods, cones, and retinal tissue. Disease onset and manifestation of symptoms can be highly variable as a wide number of hereditary abnormalities can result in RP. In most cases appreciable deficits develop over the course of 1–3 years. The severity and patterning of visual dysfunction is dependent on the distribution of cell and tissue damage, but loss of vision is irreversible.

Nyctalopia

Loss of night vision or...