Wagner's disease

Wagner's disease is a rare, hereditary disorder that causes progressive vision loss. It is characterized by the gradual deterioration of the vitreous humor, the clear gel that fills the space between the lens and the retina of the eye. This disease is also associated with abnormalities of the retina, the light-sensitive tissue at the back of the eye, and the development of cataract, which is clouding of the lens.

Symptoms

The symptoms of Wagner's disease usually begin in childhood or adolescence. The most common symptoms include myopia (nearsightedness), night blindness, and progressive loss of peripheral vision, which can lead to tunnel vision. Some affected individuals also develop cataracts, which can further impair vision.

Causes

Wagner's disease is caused by mutations in the Versican gene. This gene provides instructions for making a protein that is found in many tissues and organs, including the eye. The Versican protein plays a critical role in the structure and function of the vitreous and retina. Mutations in the Versican gene disrupt the normal structure and function of these eye tissues, leading to the vision problems characteristic of Wagner's disease.

Diagnosis

The diagnosis of Wagner's disease is based on the clinical findings and family history. Genetic testing can confirm the diagnosis. The testing involves looking for mutations in the Versican gene.

Treatment

There is currently no cure for Wagner's disease. Treatment is focused on managing the symptoms and preventing complications. This may include glasses or contact lenses to correct myopia, and surgery to remove cataracts. Regular eye exams are important to monitor the progression of the disease and adjust treatment as needed.

Prognosis

The prognosis for individuals with Wagner's disease varies. Some people maintain functional vision into adulthood, while others experience significant vision loss at a young age. The disease progresses slowly, and most people with Wagner's disease maintain some vision throughout their lives.

See also

• Vitreous humor
• Retina
• Cataract
• Myopia
• Night blindness
• Tunnel vision
• Versican

Wagner's disease Resources
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