Retinitis

Progressive retinal degeneration with dispersion and aggregation of pigment around vessels.
May look mottled or spiculated. Associated optic atrophy and narrow vessels.

Aetiology

• Primary - 50% sporadic; dominant, recessive and X-linked forms
  
• Secondary - see below

Clinically

• Night blindness
  
• Progressive loss of peripheral vision
  
• Reduced vision on ERG

Leber's congenital amaurosis

Degenerative disorder of retinal pigment with pleomorphic changes.

Secondary Retinitis Pigmentosa

• Freidrich's ataxia
  
• Mucoplysaccharidoses - Hunter/Hurler, Scheie, Sanfillipo
  
• Muscular and myotonic dystrophies
  
• Gangliosidoses - Batten-Mayou,
  
• Abetalipoproteinaemia
  
• Progressive external opthalmoplegia
• Laurence-Moon-Beidl
  
• Usher (sensori-neural deafness)
  
• Fanconi syndrome
  
• Cystinosis
  
• Refsum
  
• Alstrom
  
• Cockayne
  
• Hallgren
  
• Cystinosis

Pseudo-Retinitis Pigmentosa

• TORCHES - syphillis, CMV, rubella
  
• Obstetric and other trauma
  
• radiotherapy
  
• Drugs - choloquine, phenothiazines
  
• Retinal vascular occlusion