Retinitis Pigmentosa

Dr. Ajay Mohan (AIIMS)MBBS

February 25, 2020

March 06, 2020

Retinitis Pigmentosa
Retinitis Pigmentosa

The human eye is a ball-like structure with many layers in it. When we look at an object, the light from it strikes the outermost layer of the eye (cornea), crosses the liquid medium (aqueous humour) and reaches the coloured portion of the eyes (iris). The iris helps in regulating the amount of light that enters the eye. The image is then sent to the pupil which adjusts the light which would enter the eye. After that, an upside image forms on the lens of the eye. This image is transferred to the retina which is the light-sensitive region of the eye. The retina then sends the image information to the brain with the help of the optic nerve. After the brain processes the image, the image is sent back to the retina which straightens the image and sends it to the lens. This is how we see objects and people around us.

Any damage to any of these parts of the eye can hamper your vision. Retinitis pigmentosa is one of the eye disorders that can diminish your ability to see.

Retinitis pigmentosa is a rare, inherited condition (which means it runs in the family). This disease slowly and progressively degenerates the light-sensitive area, i.e., the retina of the eye, eventually leading to blindness. A group of disorders, retinitis pigmentosa occurs when the rods and cones in the eye no longer function properly.

Though every patient can experience the symptoms and their progression differently, usually

  • Patients lose night vision in their preteen and teen years.
  • Patients often lose their side vision next, usually as young adults.
  • Patients lose central vision later in life.

There is currently no cure for the disease but therapies like sun protection and vitamin therapy can help in delaying the rate of disease progression.

You might also be interested in: Macular degeneration: symptoms, causes, diagnosis, treatment

Retinitis pigmentosa symptoms

The symptoms of retinitis pigmentosa can be seen right from childhood. Usually, the symptoms progressively get worse as the child grows up. The common symptoms of retinitis pigmentosa are:

  • Tunnel vision: The person loses peripheral (side) vision and can only see things in a small confined space in the middle. This makes them prone to bumping into things as they move around, increasing the chances of injury.
  • Night blindness: In night blindness, the person is unable to see during the night or in a room with dim and dark lighting like movie theatres. 
  • Colour blindness: In colour blindness, the person loses the ability to distinguish between colours.
  • Loss of central vision: Some people may also suffer from the loss of central vision.

In the later stages, the person can completely lose vision as the retinal damage is extensive.

Read more: Vision problems in babies

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Retinitis pigmentosa causes

Retinitis pigmentosa (RT) is a genetic condition - it occurs due to a defect in one of the 50 genes that are responsible for the formation of photoreceptors (rods and the cones) in the eyes. Photoreceptors are the cells present in the retina which absorb the light and convert them into electrical signals so that they can be sent to the brain. RT can be passed on to future generations.

Scientists believe that the defect in the gene either stops the formation of photoreceptors or forms faulty receptors or produces a protein which is toxic for the photoreceptors. In all the cases, the retina deteriorates slowly, leading to vision loss.

Diagnosis of retinitis pigmentosa

There are some tests which can be done in order to diagnose retinitis pigmentosa (RP) in a person: 

  • Ophthalmoscope: The doctor may use a tool called the ophthalmoscope to get a better look at the patient's retina. This can show the dark black or red spots present over the retina.
  • Electroretinogram (ERG): In an ERG, the doctor checks the electrical activity of the photoreceptor cells. Reduced electrical activity is usually a sign of progressing damage of the photoreceptors.
  • Visual field test: This test is done to determine the extent of vision loss and find the blind spots. The patient is instructed to follow a dot of light moving in a 180-degree half-circle in front of their head and to their sides. The person is asked to press a button whenever they see the light. This helps to determine the visual field and also the central vision of the person taking the test.
  • Genetic test: Genetic testing can be helpful in diagnosing the disease and also the rate of progression of the disease.
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Retinitis pigmentosa treatment

There is no cure for retinitis pigmentosa yet. But there are therapies to slow down the process of degeneration. Intensive research for a cure through gene therapy and retinal prosthesis (replacing the damaged retina with a healthy one) is still in the very early stages.

So far, the therapeutic approach can only help in reducing the rate of progression of the disease. These therapies involve:

  • Sunlight protection: People with RP are asked to wear dark glasses whenever they step outdoors.
  • Vitamin therapy: Vitamin A and vitamin E have the potential to protect the photoreceptors and have antioxidant reactions which help in delaying the damage to the retina.
  • Management of coinciding complications: Complications like cataract (clouding of the lens), macular oedema (build-up of fluid in the centre of the retina), refractive errors (myopia or nearsightedness and hypermetropia or farsightedness) and inflammatory reactions should be treated immediately to prevent the worsening of the vision.


References

  1. Dyonne T. Hartong, Prof. Eliot L. Berson, Prof. Thaddeus P. Dryja. Seminar: Retinitis Pigmentosa. The Lancet, 18–24 November 2006; 368(9549): 1795-1809.
  2. Dyonne T. Hartong, Prof. Eliot L. Berson, Prof. Thaddeus P. Dryja. Seminar: Retinitis Pigmentosa. The Lancet, 18–24 November 2006; 368(9549): 1795-1809.
  3. Aier School of Ophthalmology, Central South University, China, via US National Library of Medicine ClinicalTrials.gov [Internet]. Retrobulbar Injection of Autoserum in the Treatment of Retinitis Pigmentosa: A Prospective, Non-randomized Interventional Study, 1 January 2020 - 31 December 2022
  4. Hamel C. Retinitis pigmentosa. Orphanet Journal of Rare Diseases, 11 October 2006; 1 Article no. 40
  5. Jones B.W. et al. Retinal remodeling in human retinitis pigmentosa. Experimental Eye Research, September 2016; 150: 149-165
  6. Campochiaro P.A. and Mir T. A. The mechanism of cone cell death in Retinitis Pigmentosa. Progress in Retinal and Eye Research, January 2018; 62: 24-37.

Medicines for Retinitis Pigmentosa

Medicines listed below are available for Retinitis Pigmentosa. Please note that you should not take any medicines without doctor consultation. Taking any medicine without doctor's consultation can cause serious problems.