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Retinitis Pigmentosa. Restoring possible.


Therapeutic electrical stimulation is a new alternative
avenue 
for patients with Retinitis pigmentosa


What is the science behind the electrotherapy

Therapeutic electrical stimulation is a non invasive and non surgical way of natural partial restoration of  visual functions done by applying weak currents on retina. This new alternative technology is not aimed at the replacement of damaged cells, but focuses on an increase in the functionality preserved neurons on retina and conductivity along optic nerve fires. The important role of alternating currents, influences brain electrophysiology on a network level and this, in turn, might affect the sensitization of deafferented regions or synchronization (entrainment) of neuronal network firing with long-lasting (plasticity) changes. Such a therapeutic approach is able to elicit a partial visual field enlargement and provide increased visual acuity that positively affects daily life skills.

Additionally the reorganization of the cortical receptive field, connected with damaged neuron, could be activated electrically, which is what the stimulations do. In a practical way the dynamic of receptive fields are associated with the damaged area of the retina acquiring new fields in adjacent areas

Applied therapeutic electrical stimulation is aimed to prevent further deterioration of neurons and the cells on the retina as much as possible. These protections are the result of activated downstream influences (nature signal of stimulation) from the visual cortex to the retina which supports functionality of retina neurons. There is a collected level of evidence that shows that applied electrical therapy induces neuroprotection in retinal degenerations by fascinating releasing of endogenous growth factors (e.g. brain-derived neurotrophic factor, ciliary neurotrophic factor).


Take time and read the RP patient story, who after courses significantly improved vision

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When therapeutic electrical stimulation is recommended?

Our approach is recommended for patients diagnosed with retinitis pigmentosa. Optimal entrance for stimulations are early and intermediate-stages of retinitis pigmentosa, when positive response to treatment would be mostly expected. But if the stage of retinitis pigmentosa is advanced, there is evidence, that functional improvement to some extent is possible. Independence from the disease degree in therapeutic electrical stimulation may help preserve vision that otherwise would be lost.


What to expect during the treatment?

- due to concentric contractions of visual field is pathognomonic for RP - an enlargement of VF size, opening of central“ vision" is the main effects of what the therapy does
- more than half of treated patients reported about improvement of vision in the dark, having positive impact on quality of life
- increasing visual acuity also can be expected to take place when it was disturbed


How long effects of therapeutic electrical stimulation lasting?

- Retinitis pigmentosa is accounted to be a disease with slow deterioration of vision. Therapeutic electrical stimulation slows down this progression and even leaves vision at the condition where therapy started. 
- Improvement of the visual field, generally lasts for 12-24 months depending on severity of  the disease. To support vision, additional therapies (repeated course of stimulation) are needed. 

Dr. Anton Fedorov Neurologist Berlin Center for Vision RestorationDr. Anton Fedorov


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Dr. Julia Chibisova Neurologist Berlin Center for Vision RestorationDr. Julia Chibisova


Functional improvement by retinitis pigmentosa
can be elicit by therapeutic electrical stimulation

 

We would like to illustrate with two clinical cases, how enlargement of visual fields were possible after repeated courses of therapeutic electrical stimulation performed with 6-8 months free intervals.

Clinical Case I

"Retinitis Pigmentosa Visual Field Improvement_Clinical Case"

Retinitis Pigmentosa Visual Field Improvement_Clinical Case I

Grey -  blind areas, White ares within grey - seeing areas of visual fields

 

Clinical Case II

"Retinitis Pigmentosa Visual Field Improvement_Clinical Case"

Retinitis Pigmentosa Visual Field Improvement_Clinical Case II

Description of clinical cases and results

Clinical Case I

Patient - 15 years old girl, was diagnosed with Retinitis Pigmentosa 2 years ago, when the she complained about difficulties when adaptating to darkness. Visual filed examinations revealed tunnel vision on both eyes with minimal deficit of visual acuity. Conducted Electroretinogram (ERG) confirmed reduced rod responses. Ophthalmoscopic examination revealed typical development of Retinitis Pigmentosa - narrowed retinal blood vessels, especially arteries with pigment accumulation in the form of so-called bone cells. Optic nerve head was unaffected. Patient participated in 2 courses of therapeutic electrical stimulation and reported significant increasing of seeing area as a results of visual fields borders enlargement, which helped her a lot in her daily life.

Clinical Case II

Patient - 38 years old man, was diagnosed with Retinitis Pigmentosa 17 years ago with typical complains as the moment he started his first course -  profound  difficulties when adaptating to darkness with severe restrictions of visual filed on both eyes.  Visual acuity was also deteriorated on both eye 20/100 - 30/100. Pre treatement ophthalmoscopic examination revealed profound pigment accumulation in the form of so-called bone cells along blood vessels. Optic nerve head was moderate pallor, more temporal. Patient participated in 3 courses of therapeutic electrical stimulation during 2 years and reported remarkable enlargement of visual fields together with increasing of visual acuity on both eyes. 

Retinitis pigmentosa

 

Retinitis pigmentosa (RP) is comprised of a group of inherited progressive retinal dystrophies, characterized by rod and cone photoreceptor degeneration and progressive loss of vision. As retinitis pigmentosa progresses and more photoreceptors degenerate, patients experience an extended VF deficit (tunnel vision) leading to blindness legal and functional disability. Concentric contractions of VF are associated with many deficits resulting in reading with an increased number of pauses (indicating poor reading ability), prolonged reading time, appearance of optokinetic nystagmus, and compensatory actions such as hand motions to move the characters being read into the center of the visual field or head motions to extend the reading distance. Developed vision loss adversely affect patients social and personal welfare. VF deterioration creates for the most part an alteration of contrast sensitivity - the ability to discriminate between shades of gray - that affect daily visual functioning, lifestyle, and social development and influence visual-perceptual state.

 

How a diagnosis of Retinitis pigmentosa is established?

Diagnosis of retinitis pigmentosa (RP) is established when the following are present:

Bildschirmfoto 2015-09-23 um 08.53.06Rod dysfunction as measured by dark adaptation (elevated rod final threshold) or with Electroretinogram (ERG) Bildschirmfoto 2015-09-23 um 08.53.17Loss of peripheral vision that often is greater superiorly but can involve other regions as well Bildschirmfoto 2015-09-23 um 08.53.25Bilateral involvement that has a high degree of symmetry, with respect to both the severity and the pattern of visual field loss and retinal changes
 

Slowdown

slow down the progress of retinal degeneration

Retinitis pigmentosa management

 Until now, there are no medical solutions known for this pathology or for other degenerative visual problems.
In a disease such as retinitis pigmentosa, the management options are extremely limited and aimed at two main goals:

Rise up

partial improvement of impaired visual functions

 

A new approaches to treatment retinitis pigmentosa have been recently investigated and focused on:

Nutrition

nutritional and vitamin supplementation

dnk

gene therapy based on mutation approach

stem cells

stem cells
treatment

drug

search for new pharmacologic agents 

electronic

intraocular or retinal electronic implants

Stem Cell Therapy

Cell replacement (stem cell) therapy could be a viable treatment option for retinal dystrophies in the future to make an impact on visual loss too. Cell transplantation has the ability to stop degeneration and avoid further vision loss more so than any other treatment available to the damaged retina. Over the years, diverse transplant approaches have been developed and were used. But given the side effects of photoreceptor transplantation and the additional risks it carries, for iatrogenic malignancy or ectopic growth it would need to be decided upon case by case. stem cell therapy

Gene therapy

gene therapy Gene augmentation therapy appears a feasible, safe treatment strategy for at least some inherited retinal dystrophies. The concept involves transferring genetic material into cells, tissues or organs with the aim of curing a disease or improving the clinical status of a patient. However gene therapy could not prevent retinal degeneration progressing. Unfortunately an improvement in visual function therefore cannot be assumed to imply protection from degeneration, suggesting the need for a combinatorial approach in treating retinal dystrophies.

 

Electronic retinal implants

Electronic retinal implants were developed to replace the photoreceptive function of the human eye by using light-dependent electrical stimulation of the inner retinal layers with an intended purpose to transform blindness into low vision or make vision more useful in day by day activity. The electrical implants demonstrate that inner retinal neurons in blind patients can respond to appropriate stimulation and lead to a useful visual percept allowing simple navigation and object recognition. These electronic designs are under continual development to increase the resolution, improve the surgical implantation procedures, and increase the sophistication of their signal-encoding algorithms.

retinal implants

 

Today these treatments are minimally effective in slowing disease progression or even less apt to partially restore eyesight. New therapies should be created to improve the quality of life for patients with retinitis pigmentosa, by improving visual function, achieving both a quantitative and a qualitative enhancement in social functioning. Many patients with retinitis pigmentosa are motivated to try available therapies to attempt to improve eyesight by applying valuable and proven modern technologies.

All therapeutic approaches mentioned above  are still far away from today’s patients needs and are recently developed technologies and therapies which should be used in an attempt to improve quality of life by making the eyesight better.

What form of Retinitis pigmentosa, except classical form are indicated for therapy

Several disorders caused by pathogenic variants in the same genes implicated in RP clinically are considered to have retinitis pigmentosa complaints and patients could also benefit from our treatment:

Tick Tapetoretinal dystrophies. Tapetoretinal dystrophies are hereditary eye diseases, the most common of them being dystrophy of retina pigmentosa. They are inherited as autosomal recessive, autosomal dominant, or X-chromosome linked (X-linked or sex linked) traits, or may occasionally occur as the result of fresh mutation 
 Tick Usher syndrome different types. Usher syndrome is a congenital autosomal recessive syndrome with profound, bilateral sensorineural hearing loss, abnormalities of vestibular nerve function with non-progressive ataxia which causes slowly progressive development of vision deterioration.
 Tick Cone or cone-rod dystrophy, sometimes called inverse or central RP, refers to a group of disorders characterized by bilateral and symmetric loss of cone function in the presence of reduced rod function. Loss of central visual acuity, photoaversion, and color vision defects appear before peripheral visual loss and defective dark adaptation. 
 Tick Unilateral RP refers to unilateral functional and ophthalmoscopic changes; the underlying etiology and mechanism remain unknown. Many non-genetic causes of retinopathy may masquerade as unilateral RP and should be excluded

 

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