Retinitis Pigmentosa (RP) is defined as a hereditary, progressive
degeneration of the neuroepithelium of the retina characterized by night
blindness and progressive contraction of the visual field. RP refers to a
group of hereditary disorders that affect the retina's ability to respond
to light. It primarily affects rod cells, or the photoreceptors
responsible for night vision and seeing in dim light. Rod cells also
account for peripheral vision. Cone cells can also be affected as the
disease progresses. Cone cells are responsible for color vision and seeing
RP is a relatively rare condition. It affects an estimated fifty
thousand to one hundred thousand individuals in the United States.
Worldwide, RP is estimated to affect 1.5 million people. The incidence of
Ocular signs start with the breakdown of rod cells. Rods are present
within the outside macula, or center of the retina. The peripheral retina
is predominately composed of rod cells. Symptoms of RP usually manifest
between the ages of 10 and 30. First, night vision decreases along with
the ability to adjust to dim lighting. When the rod cells and outer cone
cells are afflicted, the subsequent loss of peripheral sight leads to
tunnel vision. Rate of progression for RP varies by individual patient and
There is no treatment for RP. With proper care and diet, the
progression of the disease can be slowed or halted. Researchers are
investigating modes of therapy and treatment to possibly reverse the
degeneration and restore patients' sight.
How Retinitis Pigmentosa is diagnosed.
Doctors use a standard array of tests when a patient is displaying
symptoms of Retinitis Pigmentosa.[iii] These tests are used to determine
loss of vision and to track the progression of the condition.
An electroretinagram (ERG) measures the response of the retina to a
light stimulus. Electrodes are placed be...