This document discusses retinitis pigmentosa, a hereditary retinal disease characterized by the progressive degeneration of photoreceptors in the retina. It predominantly affects rods first and then cones. Key features include night blindness, tunnel vision, retinal bone-spicule pigmentary changes, and arteriolar attenuation. Diagnosis involves electroretinography, electrooculography, and perimetry testing. There is no cure and management focuses on low vision aids and rehabilitation to stop progression.

2. • HOW YOUR EYE WORKS?

4. • Heterogeneous group of retinal diseases
• Most common hereditary fundus dystrophy
• Bilaterally symmetrical ,progressive degeneration
of retinal photoreceptors
• Predominantly affects rods followed by cones

5. • Prevalence – 1 in 5000 of world population
• Age – seen childhood and progresses
• Race
• Sex – males >females 3:2
• Lateratily – both eyes equally affected

6. :-
• Sporadic –Isolate i.e mutation of multiple
gene(>50%), rhodopsin gene (40%)
• Autosomal Dominant:-(25%),least severe
• Autosomal Recessive:-(25%), intermediate
severity
• X-Linked Recessive:-(10%),most severe

9. • Night blindness
• Delayed dark adaptation
• Tunnel vision

10. • Retinal bone-spicule ligament
• Arteriolar attenuation
• Pale and waxy optic disc
• Retinal pigmentary changes

13. • Bilateral mid peripheral intraretinal perivascular
bony-spiculepigmentary changes with arteriolar
narrowing
• Gradual increase in density of the pigment
• Anterior and posterior spread
• Peripheral pigmentation may become severe,with
marked arteriolar narrowing and disc pallor

15. • ERG
• EOG
• PERIMETRY
• OCT
• GENETIC ANALYSIS

16. • Laurence-Moon-biedal syndrome
• Cockayne’s syndrome
• Refsum’s syndrome
• Usher’s syndrome
• Hallgren’s syndrome

17. Atypical Forms of RP:-
• Cone-Rod Dystrophy
• Retinitis Pigmentosa Sine Pigmento
• Sectorial retinitis pigmentosa
• Pericentric retinitis pigmentosa
• Retinitis Pigmenta Albiscens

18. • Measures to stop progression
• Correct any refractive error-
• Low vision aids
• Rehabilitation
• Prophylaxis