Retinitis pigmentosa (RP): Symptoms, Signs & Diagnosis
Recognize the typical signs of RP, understand its progression, and learn how the diagnosis is made — and what matters most now.
Briefly explained: What is RP – and what does it mean for me?
Retinitis pigmentosa (RP) is a collective term for hereditary retinal diseases. Simply put: First, the rod cells (vision in twilight) usually lose function, followed later often by the cone cells (colors, details). RP is not curable; TES treatment aims to preserve visual functions for as long as possible and to manage daily life well.1 Many affected individuals initially notice night vision problems, which develop into a loss of peripheral vision over time, while the central vision (reading, recognizing details) is relatively spared at first1. For a small percentage (approx. 1–6%) with a specific genetic form (RPE65), there is gene therapy.1
Important:
RP progresses differently for each individual.
Onset, pace, and symptoms vary from person to person. Many find strategies to organize their daily lives well – support from ophthalmologists, rehabilitation, and aids helps.1
First signs:How to recognize RP in everyday life
Do your eyes take a long time to adjust to the dark? Do you feel unsure on stairs or in poorly lit rooms? These are typical early signs (night vision problems, slow dark adaptation).1
Many people notice that their peripheral vision declines—they bump into things more often or miss people and objects “out of the corner of their eye.” Subjectively, a gradual “tunnel vision” develops, while central vision often remains intact for longer.1
Common symptoms include sensitivity to glare, reduced contrast, and changes in color perception. Simple everyday strategies (e.g., edge filters, even lighting) can provide noticeable relief.1
Some see flashes of light (photopsias) or flickering. These symptoms can occur but are not specific.1
- … you feel insecure in twilight or bump into things more often.
- … narrow corridors/shelves cause problems.
- … you react strongly to light/glare.
- … retinal diseases occur in your family.
Diagnosis: How the clarification process works with an ophthalmologist
Consultation & Family History
First, it’s about your history: When did the symptoms begin? Are there any cases in your family? Which everyday situations are difficult (e.g., darkness, stairs, driving)? Comorbidities and medications are also important. (Note: In syndromic forms such as Usher syndrome, hearing may also be affected.)1
Examinations & Imaging (Fundus, OCT, FAF)
- Funduscopy: The back of the eye is assessed (vessels, pigment, optic nerve). Classic pigment changes are not obligatory – a normal finding does not rule out RP.
- OCT (Optical Coherence Tomography): Cross-sectional images show the photoreceptor layers and aid in monitoring progression.
- FAF (Fundus Autofluorescence): makes metabolic changes visible; typical patterns support diagnosis and progression assessment.
These components together provide a clear picture – your doctor will explain what makes sense for you.1
Visual Field Measurement (Perimetry)
Perimetry measures how far you can see peripherally. The result is a key progression parameter because RP primarily affects peripheral vision. In studies, (semi-)kinetic perimetry (e.g., Goldmann targets V4e/III4e) is used for this purpose, among others.2
Electroretinogram (ERG)
The ERG measures the electrical response of the retina to light. In RP, these signals are often attenuated – an objective functional proof that is particularly helpful in children or when test results are uncertain (your doctor will discuss the benefits and procedure).1
Genetic Test: When it is useful
A genetic test can clarify the cause, facilitate prognoses, and open access to studies or targeted therapies. Important: A normal genetic test does not rule out RP – not every change is known today.1
Progression: How does RP develop – and how quickly?
RP progresses, usually over many years. The visual field is particularly affected: The visual field area decreases exponentially; annual reductions in the range of approximately 5% to 17% are described in the literature – depending on the measurement method, initial situation, and genetics.2 Regular, standardized controls are important for reliable statements (same procedure, similar conditions).2
Good News: What can positively influence the course
Transcorneal electrical stimulation (TES) with OkuStim®
With TES, weak electrical impulses are delivered to the eye via a fine electrode. The goal is to activate protective mechanisms in the retina and thus preserve visual function for longer.
What clinical data show: In an interventional, randomized study over one year with weekly TES treatment, the annual loss of visual field area in treated eyes was less than in untreated eyes; a dose-dependent effect was also observed (higher current strength → stronger effect).2
Safety: Adverse events predominantly occurred as mild, transient complaints (primarily “dry eyes”); no serious therapy- or device-related events were reported. A dose-risk relationship for side effects was not observed.2
What does this mean for you? TES does not cure RP. However, it can help preserve existing visual function for longer. Discuss with your ophthalmologist whether TES is suitable for you.2
Path to Therapy
- Check suitability: Consultation and initial examination at the HCP center
- Individual adjustment: determine personal stimulation threshold and current strength
- Home application: in studies, once a week for 12 months; your doctor will coordinate the frequency with you2
- Follow-up checks: regularly (e.g., visual field/OCT) for objective verification2
Visual Aids, Rehabilitation & Daily Life
Many things help immediately:
- Reduce glare: edge filters, cap with brim, uniform lighting
- Increase contrast: large, clear fonts; strong light-dark differences
- Ensure mobility: low-vision training, possibly long cane; apps/screen readers
- Work/School: disability accommodations, magnification and reading software, targeted treatment of co-morbidities (e.g., cataracts, macular edema) – these measures improve independence and quality of life – a central goal of RP care.1
When should I seek medical advice?
- Early warning signs: insecurity in the dark, frequent bumping into things, problems in poorly lit environments.1
- Family history: Are there cases of RP in the family? Get examined and advised early.1
- Known RP: Please remember regular check-ups (visual field, OCT, possibly ERG) – this way, changes are detected in time and therapy is adjusted.1
Frequently Asked Questions (FAQ)
No. RP progresses slowly and very differently. Many manage well in daily life, work, and family for a long time – often even better with aids and training.1
This primarily depends on the visual field. Your ophthalmologist will check this individually. If there is significant narrowing, driving is no longer permitted – safety comes first. (The importance of the visual field for daily life and mobility is well documented.)1.2
Gene therapy is approved only for a few genetic forms – e.g., for RPE65. A genetic test clarifies whether this could be relevant for you. Further studies are ongoing in parallel.1
This depends on your clinical findings, goals, and daily routine. If TES is suitable, you will receive training, and the therapy can be conveniently carried out at home.2
References
1 Cross N et al. Retinitis Pigmentosa: Burden of Disease and Current Unmet Needs. Clin Ophthalmol. 2022;16:1993–2010.
2 Stett A et al. TcES dose-dependently slows visual field loss in RP. TVST. 2023;12(2):29.
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