Retinitis Pigmentosa
All info about retinitis pigmentosa
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What is retinitis pigmentosa?

Retinitis pigmentosa (RP) is a disease that leads to retinal degeneration. The sensory cells (photoreceptors) of the retina at the back of the eye may die off due to genetic abnormalities. Most often, it is the rods at the far edge of the visual field that are affected first, and as the disease progresses, the damage can slowly and gradually increase in size, reaching the macula. The vision loss can be particularly challenging when the macula, the central visual field region with the sharpest vision, is damaged.

Causes

Retinitis pigmentosa is inherited in the majority of cases. Sometimes the disease is also triggered by certain medications. The resulting retinal degeneration shows symptoms similar to retinitis pigmentosa, in which case it is called pseudoretinitis pigmentosa.

Origin of the name and epidemiology

The name retinitis pigmentosa originates from 1855, when the Dutchman Frans Donders first described the clinical symptoms and signs. The suffix ”-itis” indicates an inflammation, which is not medically correct, because there is no inflammation. Therefore, this disease was renamed retinitis pigmentosa.

In Germany, between 30,000 and 40,000 people are affected by retinitis pigmentosa; worldwide there are an estimated 3 million people affected. It is possible that the number of unreported cases is higher, as the disease progresses insidiously. The probability of contracting retinitis pigmentosa is about 1 in 2,000.

How do patients see with retinitis pigmentosa?

Retinitis pigmentosa manifests in adolescence or a little later, with night blindness as the first symptom. Then the vision slowly diminishes: The eyes are less and less able to adapt to changing light conditions and are subsequently sensitive to glare. Contrasts are no longer recognized, and finally colors can no longer be seen properly. While the central visual field remains intact for a long time, peripheral vision is impaired, which is also called “tunnel vision” or “tube vision.”

Retinitis-pigmentosa-Tunnelblick
Example of tunnel vision in retinitis pigmentosa (RP)

How does retinitis pigmentosa develop?

Even in early stages, affected individuals may be dependent on orientation aids, such as a long cane, due to night blindness and tunnel vision. Often, retinitis pigmentosa leads to blindness. Nearly half of all sufferers also develop cataracts in adulthood. From the first symptom to full blindness can be a very long process, extending over several decades. Therefore, the diagnosis of retinitis pigmentosa is psychologically very stressful.

Associated retinitis pigmentosa syndrome

About a quarter of all RP patients suffer from associated retinitis pigmentosa syndrome. This means that not only the eye but other organs of the patient show symptoms of the disease as well. These can include hearing disorders as well as cardiac arrhythmias, paralysis, gait disorders, or muscle weakness, among many other symptoms. The best known of these are Usher, Bardet-Biedl, Refsum, NBIA, Alport, and Saldino-Mainzer syndromes.

Retinitis pigmentosa diagnosis

If symptoms of retinitis pigmentosa appear in childhood, an ophthalmologist should be consulted. RP can be diagnosed at a young age with the help of an electroretinogram, which can detect reduced nerve signaling potentials. An ophthalmologic examination may also reveal narrowed retinal vessels, waxy yellow atrophy of the optic nerve papilla, and bony pigmentation. Macular changes are found only in later stages. Possible hearing disorders or certain blood values also can show whether retinitis pigmentosa is possibly present.

Is there a cure for retinitis pigmentosa?

Unfortunately, there is currently no conventional medical treatment that can cure retinitis pigmentosa or prevent the progression of  the disease. Up to half of all people affected go blind before they reach the age of 40. However, there are a few rare special forms of retinitis pigmentosa in which the process can be brought to a halt with targeted measures.

Clinical observations suggest that vision loss from retinitis pigmentosa is partially reversible with SAVIR therapy. This therapy uses small microcurrent pulses to improve blood flow in the eye and brain, so that “paralyzed” nerve cells and photoreceptors can be revived, and the remaining optical stimuli can be better processed. This microcurrent treatment can significantly improve the vision of patients suffering from retinitis pigmentosa.

SAVIR therapy for retinitis pigmentosa

Is microcurrent treatment effective?

In a clinical trial, 82 patients whose vision was impaired by optic nerve damage were treated for 10 days with either microcurrent (SAVIR therapy) or a noncurrent placebo therapy. For the microcurrent treatment, electrodes were attached above and next to the eyes, and the patients were administered very mild current pulses via the electrodes for 40 minutes daily. After only 10 days of microcurrent therapy, visual performance improved significantly in two-thirds of the study participants, as the neuron network in their brains had recovered. Blood flow to the brain also improved. In one example, the visual cortex in the back of the head was once again able to process visual signals with the frontal cortex in the forehead area in order to generate meaningful images from the visual impulses of the eyes. As a result, patients were able to see better, even though the damage to the optic nerve was considered “irreparable”. The therapy had simply reactivated “dormant” (silent) neurons. Similar observations were also reported in patients with retinitis pigmentosa in the scientific literature, which was confirmed by our observations at SAVIR.

Risks and side effects of SAVIR therapy

SAVIR therapy has hardly any risks or side effects. In more than 2,000 patient treatments, not a single serious adverse event was reported. The current pulses used are so weak that they are hardly felt on the skin. Patients may notice brief flashes of light when their eyes are closed during treatment. By the way, the current pulses are much lower than those of a pacemaker. There is more light at the end of the tunnel for retinitis pigmentosa.
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