A Simple Guide to Retinitis pigmentosa

A Simple Guide to Retinitis pigmentosa
----------------------------------------------

What is Retinitis pigmentosa?
---------------------------------------

Retinitis pigmentosa is a slow degenerative disease of the retina.


Who is affected by Retinitis pigmentosa?
------------------------------------------------

Retinitis pigmentosa is a genetically determined disease in which abnormal photoreceptors (rods and cones) or the Retinitis pigment epithelium of the retina cause progressive loss of vision.

Initially there is night blindness due to the abnormal photoreceptors.

This is followed by the loss of peripheral visual field known as tunnel
vision which may persist for some years.

Finally the macular region is affected with loss of central vision in the later stages.

In rare cases blindness may occur in childhood.


What is the cause of Retinitis pigmentosa?
------------------------------------------------

The cause is usually in the genes and of the recessive trait.

There is a mild form of dominant trait.

Rarely it is sex linked and can be severe.


What are symptoms and signs of Retinitis pigmentosa?
---------------------------------------------------------

Symptoms:
--------------

1.loss of night vision even in childhood

2.tunnel vision occurs at age of 40-50 for several years or decades

3.loss of central vision usually at 50-60 years of age


Signs:
-----------------

1. In early stage, direct opthalmoscopy show small spidery black spots

2. vessels are sheathed with pigments in some areas

3. Retinal vessels become attenuated (thinned)

4. Optic atrophy(cellophane maculopathy) sets in

5. posterior subcapsular cataracts form at late stage.


How is Retinitis pigmentosa diagnosed?
---------------------------------------------

1. electroretinography (ERG) show progressive loss of photoreceptor function

2.Visual field testing show loss of peripheral vision

3.Flourescin angiography may show dark pigments to establish the presence of Retinitis pigmentosa.


What are the complications of Retinitis pigmentosa?
-------------------------------------------------------

Partial to complete loss of vision.


What is the treatment for Retinitis pigmentosa?
-------------------------------------------------

Retinitis pigmentosa has no cure.

Several methods of treatment aimed at slowing down the progression of loss of vision have been tried:


1.daily intake of 15000 IU of vitamin A palmitate.

2.Retinitis transplants,

3 artificial Retinitis implants,

4.gene therapy,

5.stem cells,


How is Retinitis pigmentosa monitored?
-----------------------------------------

1.regular follow up with the eye doctor.

2.examining the retina for further damage

3.analyzing the visual fields.


What is the prognosis of Retinitis pigmentosa?
----------------------------------------------------

The prognosis is very poor as progression to blindness is the rule.

How can Retinitis pigmentosa be prevented?
-----------------------------------------------

There is no prevention for Retinitis pigmentosa.

The following may help:

1.Genetic counselling

2.Examination of family members for signs of loss of vision