Retinitis Pigmentosa Flashcards
What is Retinitis Pigmentosa (RP)?
A genetic eye disease that causes progressive degeneration of the retina, leading to vision loss, particularly in low light.
What are the signs of Retinitis Pigmentosa?
Hyperpigmentation in the shape of bony spicules in the periphery, Areas of atrophy, Arteriolar attenuation, Waxy disc pallor, Peripheral vision loss on visual field testing.
What are the symptoms of Retinitis Pigmentosa?
Loss of night vision,
Tunnel vision,
Loss of central vision in some cases,
Problems with color vision.
How does Retinitis Pigmentosa affect night vision?
It causes difficulty with dark adaptation, leading to night blindness.
What is tunnel vision in Retinitis Pigmentosa?
The loss of peripheral vision, making it difficult to see anything outside a narrow field of view.
What is the management plan for Retinitis Pigmentosa?
Routine referral to HES for diagnosis confirmation, including genetic testing.
What role does genetic testing play in managing Retinitis Pigmentosa?
It helps confirm the diagnosis, determine the disease course, and identify whether gene therapy may be helpful.
How is Retinitis Pigmentosa treated?
There is no single treatment, as over 100 genes can cause RP, though some types, like those caused by the RPE65 gene, can be treated.
What is the significance of the RPE65 gene in Retinitis Pigmentosa?
RP caused by mutations in the RPE65 gene can be treated with gene therapy.
What does arteriolar attenuation indicate in Retinitis Pigmentosa?
It is a sign of retinal degeneration where the retinal blood vessels become narrow and thin.
What is waxy disc pallor in Retinitis Pigmentosa?
A pale appearance of the optic disc, which is a sign of retinal degeneration.
How can Retinitis Pigmentosa affect central vision?
Some patients may experience a loss of central vision, though it is more common to lose peripheral vision.
Why is color vision affected in Retinitis Pigmentosa?
Retinal degeneration can impair the ability to distinguish colors.
What factors influence the progression of Retinitis Pigmentosa?
The rate of progression, age of onset, and severity of visual loss are related to the mode of inheritance.
What is the prognosis for Retinitis Pigmentosa?
The progression and severity vary depending on genetic factors, but it typically leads to gradual vision loss.
