Retinitis
Pigmentosa
This is a disease which was first identified, diagnosed and named after a
scientist named Dr. Donders in 1857. Retinitis Pigmentosa may be defined as a
collection of some interrelated conditions that are genetic, medically unique
and progressive. They also share a common feature of damaged photoreceptors of
the retina (or dystrophy) and also of the pigment epithelium beneath the
photoreceptors.
Retinitis Pigmentosa and Normal Retina
Retina is a membrane
or layer that is placed at the back of the eye. The layer is made up of
photosensitive layer which contains millions of photo receptors. These
photoreceptors gets stimulated when the rays of the light fall on retina. These
rods or the photo receptors and cones are used to convey the message of light
and its pattern to the brain through nerves that unite to form the optic nerve
which leads to a normal visual perception.
Symptoms and Pathophysiology
The very initial
phase of this disease started with rise of the difficulties in vision usually
in dim or low light. This is marked as the reduction in vision along the edge,
which is also identified as tunnel vision. The central vision does not get
affected till the later stages of the diseases are reached.
The main cause of
Retinitis pigmentosa is that can be acquired through inheritance. The disease
starts with the changes in pigment which affects or destroys the small arteries
as well as the blood vessels which is used to supply the blood to the retina. These
effects of this disease can also harm the optic nerve.And all this harm caused
due to the pigmentation begins within the layers of neural retina.
In the developing
stage of this disease retinal blood vessels start thinning.
This slowly affects
the light sensitive cells of the retina. In this process both the
photoreceptors i.e. the cone and the rod, get influenced. It is type of the
inherited defect that decides which of the cells will be affected more. The
failure of rod photoreceptors is most common in Retinitis Pigmentosa.
Chromosomes effecting Retinitis
pigmentosa:
Till today, about 50
different inherited deficiencies have been diagnosed which can be connected
with Retinitis Pigmentosa. Near about 30 to 40% are autosomal dominant, while
50 to 60% are autosomal recessive and rest 5 to 15% are related to X- chromosome(which is generally passed
from mother to her son).
In the beginning the
autosomal dominant forms show very minor symptoms, which are generally seen
around the late fifties or sixties. The most severe form is the X-linked form.
This form is marked with the loss of central vision which may be noticed in
very early years of life i.e. somewhere around thirties.
Statistics of Retinitis pigmentosa:
The main cause of the
disease Retinis Pigmentosa is heredity retinal degeneration-associated
blindness. The occurrence of this disease is 1 in 4,000 in all age groups and 1
in 3,195 in inhabitants aged 45 to 64. It affects men more as compared to the
women and other types have no ethic or sex preferences.
The treatment of this
disease is generally diagnosed by examining the retina using the slit lamp
biomicrosopy.
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