Graysons Syndrome (Grayson Wilbrandt corneal dystrophy)
- Graysons Syndrome is an extremely rare disease.
- This syndrome also known as Grayson Wilbrandt corneal dystrophy (GWCD) is one of the rarest form of an eye disease that is corneal dystrophy.
- This GWCD is a mitochondrial condition
- The disease results from deficiency of a substance in the body called nadph.
- This deficiency is due to reduced activity of NADPH.
- This condition is characterized by varied patterns of opacification in the structure of cornea known as the Bowman layer of the cornea
- This layer of the cornea extends anteriorly into the epithelium with decreased to normal visual acuity.
- This disease results from a mutation usually a homozygous one.
- This mutation is in the NADK2 gene on chromosome 5p13.
- Grayson Wilbrandt corneal dystrophy
The prevalence of Graysons Syndrome is less than 1 in 1000000
The existence of Grayson Wilbrandt corneal dystrophy as a distinct entity is questionable
Prevalence of this form of corneal dystrophy is mostly unknown
As of now there is an evidence of only one report of an incompletely studied family
Age of Onset
The typical age of onset of this condition varies from adolescents to adults
This condition is Autosomal Dominant
What are the Symptoms of Graysons Syndrome
- The first symptom of Graysons Syndrome is the Erosions in the eye
- These erosions are painful
- The usual onset of these erosions in the first and second decade of life.
- The erosions are less severe than other forms of corneal dystrophies like Reis Bucklers corneal dystrophy and Thiel Behnke corneal dystrophy.
- Slight decrease in vision is the next symptom
- The course of this disease is most commonly progressive.
What causes this condition
What causes Graysons Syndrome is mostly unknown
The etiology of this condition might be genetic
What are the risk factors of Graysons Syndrome
Here are the risk factors of Graysons Syndrome
There is an approximate 40 to 50 percent risk of this condition in the child below the age group of 20 years if one of the parents has this condition.
The risk of developing this disorder is purely genetic as Graysons Syndrome is an inherited disease.
How is this disease diagnosed?
- The diagnosis of this Graysons Syndrome is by slit-lamp examination
- Slit-lamp examination reveals variable patterns of opacification in the Bowman layer
- This opacification varies from diffuse mottling to diffuse gray-white opacities.
- The opacities extend anteriorly into the epithelium
- The cornea between these deposits is usually clear.
How is Graysons Syndrome treated?
Surgery is the preferred option of treatment for this Grayson Wilbrandt corneal dystrophy
The vision can be restored by the surgical procedures such as penetrating keratoplasty or superficial keratectomy
What is the Prognosis
Favorable prognosis with prompt treatment