Short notes retinitis pigmentosa

Retinitis pigmentosa

Degeneration of rod

Symptom: night blindness and difficulty in dark adaptation
5 in 1000 in population

Sign: initially symptomatic only. Then will have bony spicules pigmentation innretina, initially at equator, then will extend to anterior and posterior. Retinal vessel will become attenuated, then optic disc becoming paler till become waxy appearance optic atrophy.
Late complication, pt can also have tubulur vision.

Association with other disease:

Ocular
- high myopia
- POAG
- microphthalmus
- conical cornea
- posterior subcapsular cataract.

Systemic association:
1. Laurence moon biedl syndrome
- retinitis pigmentosa, obesity, hypogenitalism, polydactyly and mental deficiencies.
2. Cockcaney syndrome - retinitis pigmentosa,infantile deafness, mental retardation, dwarfism nystagmus, ataxia.
3. Refsum syndrome - retinitis pigmentosa, cerebellar ataxia, peripheral neuropathy,
4. Usher syndrome - retinitis pigmentosa, labyrinthine deafness.
5. Hallgren,s syndrome- retinitis pigmentosa, vestibulocerebellar ataxia, congenital deafness, mental retardation.

Atypical retinitis pigmentosa better than typical retinitis pigmentosa.


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