Retinitis Pigmentosa

The authors review the symptomatic and genetic aspects of the various entities of isolated retinitis pigmentosa (R.P), both in its typical form and in the forms associated with the affection of other ocular tissues. Syndromes in which R. P. is associated with the affection of other organs and systemic disorders are also cconsidered. Origin, diagnosis and the course of the disease are discussed with regard to electrophysiology, histopathology, fluorescein angiography and biochemistry. Animal research has provided new realizations about the ultrastructure and physiological mechanisms of retinal photoreceptors, and better understanding of abnormal changes. The possible pathogenesis of the human disease, based on research findings, is onsidered. Although R.P. is generally thought to be to be an "untreatable" disease, therapy may be effective in several pathological entities. Methods and results of therapy with vitamins, light deprivation and vision aids are discussed.