Retinitis Pigmentosa

RETÄ°NÄ°TÄ°S PÄ°GMENTOSA (Moon Blindness)

1- Retinitis Pigmentosa Disease is;

• 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,
    • Polytdactily
    • Hypogonadism
    • Kidney problems

2- There are two types of reseptors dystrophy:

• If Patient suffers from Rod Dystrophy:
  • Periferik yani çevre görme bozulmuÅŸtur.
  • 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

3- 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

4- 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

5- Examination:

• Pigmentation in Retina
• Attenuation in retinal arteries
• Wawy pallor of the optic nevre head

6- Diagnosis:

• Vision
• Vision field
• ERG

7- Complication:

• Cataract
• Cystic macular oedem
• Myope
• Astigmatism
• Keratoconus
• Glaucoma
• Nistagmus

8- Treatment:

• Genes Theraphy
  Adeno-Virus
  A.A.V (Adeno-Assosiated Virus) and Lentiviruses are used as vectors.
  
  There are 2 major problems in theraphy
  • vectors can’t be placed in the target area
  • virus expression can’t be controlled
  
  So;
  • Immun system deficiency causes cancer and patient deaths
  • Other problem also is that it triggers antibody production


• 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


• Treatment of Electro-Acupuncture:
  • 0.25x0.25 steel needles were used
  • 20 seans were applied
  • it takes 3 months
  
  Lastly;
• Now we are not at the beginning of gene therapies anymore
• I can say that

From now on electro-acupuncture will definetily play a serious role in gene therapies...