RETINAL DYSTROPHIES AND SYNDROMES
Retinitis pigmentosa (RP) is a group of hereditary retinal diseases, which are characterized by progressive loss of peripheral vision, and poor night vision (nyctalopia) or poor vision in excessive light, and can later lead to loss of central vision.
There are several types of RP caused by numerous genetic mutations, whose pattern of inheritance can be dominant or recessive. RP is typically a dystrophy of photoreceptor cells in which the genetic defect causes cell death mainly in the rods and, more rarely, in the cones and the retinal pigment epithelium (RPE).
Usually the symptoms start to appear between 10 and 30 and disease progression is variable, presenting tunnel vision in the later stages.
Fundoscopy reveals RPE atrophy and hyper-pigmentation in the periphery of the retina and an average optic...