RETİNİTİS PİGMENTOSA (Moon blindness)

Retinitis pigmentosa is a genetic disease

We can study RP in an inherid eye disease called retinal dystrophy

It has two reseptors dystrophy, rod dystrophy and con dystrophy

It is progressive,
-can cause blindness
- can be systemic such as Usher syndrom and Retinitis Pigmentosa
Obesity,
Mental retardation
Polytdactily
Hypogonadism
Kidney problems

There are two types of reseptors dystrophy
If Patient suffers from rod dystroph
Problem will be releated with periferic vision loss , meaning he has tunnel vision

,If patient suffers from con dystrophy
Central vision loss will be unavoidable
Eventually
It paves the way to blindness

Symptoms:

1-nyctalopia,
Loss of night vision

2-adaptation disorders,
Patient can ‘t adjust well to dark and dimly lit environments

-bumping into furniture or doorframes
-diffuculties in playing games,tenis baseball and baskedball

3-Vision is often worse under bright lighting
4-Color discrimination is also a serious problem

5-Myopy and astigmatism shoudn’t be mislead in terms of diagnosis

6-Patient can report small flashes of light or a twinkling shimmering

This abberant impuls shows the degenerating retina in retinityis pigmentosa cases

Causes:
1-Mutation of –Rodopsin and peripherin
2-Mutation of c-GMP phosphodiesteras
c-GMP phosphodiesterase affects visual transduction cascade in general vision process,
c-GMP is destroyed by c-GMP phosphodiesterase
3-Apoptosis of Photoreceptor cells
4-Splycing complex failure
5-Being unable to restore external segments of rod cells

Examination:
-Pigmentation in Retina
-Attenuation in retinal arteries
-Wawy pallor of the optic nevre head

-Diagnosis -vision
-vision fielt
-ERG

-Complication
-Cataract
-cystic macular oedema
-Myope
-Astigmatism
-Keratoconus
-Glaucoma

Treatment

1)Genes Theraphy

AV,
AAV
Parvo viruses)
Lentiviruses are used as vectors

There are 2 major problems in theraphy

1)vectors can’t be placed in the target area
2)virus expression can’t be controlled

So
Immun system deficiency causes cancer and patient deaths

Other problem also is that it triggers antibody production

2)Biologic Eye
were applied by 23 patients in 7 years.
But so far it’s a disappointment.

They declared sucsess is %10 and
color vision also can’t be provided

3)Treatment of Electro-Acupuncture

0.25x0.25 steel needles were used
-20 seans were applied
-it takes 3 months