Amberstrom Leber: symptoms, causes, treatment

Leber's amaurosis is a rare but serious congenital disease in which complete or partial loss of vision is possible. Pathology begins to manifest itself almost from birth and during the growing up of the child is able to deliver a lot of trouble to his parents. The possibility of treating amaurosis is a complex issue, forcing scientists to develop new methods and techniques.

Content

  • 1Description of the disease
  • 2Causes
  • 3Symptoms
  • 4Possible complications
  • 5Treatment
    • 5.1Medication
    • 5.2Surgically
  • 6Prevention
  • 7Video
  • 8conclusions

Description of the disease

Leber's amaurosis is characterized by an innate lesion of retinal elements and cells, which are responsible for the perception of light.When it occurs, the early period of a child's life goes against the background of a weakened reaction of the pupil of the eye to light. After this, the development of fear of light and farsightedness is possible, in the future this can lead to a complete loss of vision.

The problem arises from a mutation in the eighteen genes of the retina. At the same time, the same frequency of manifestation of pathologies in both women and men is observed throughout the planet. Also, a temporary disease can be diagnosed.

Causes

The pathology of the congenital type appears as a result of improper metabolism in the sticks and cones of the eye and their subsequent destruction.Transmission of the disease by hereditary pathway is possible in the event that both parents have a gene with a defect. In this case, the cells of photosensitive receptors and pigment epithelium lose their separation properties.

Transistor (temporary) amaurosis can be caused by the following factors:

  • Injuries;
  • Disorders of the blood circulation of the brain;
  • Migraine;
  • Infections;
  • Metabolic disease;
  • Diabetes;
  • A brain tumor;
  • The impact of alcohol, drugs on the body.

Symptoms

In children with Leber's amaurosis, unregistered electroretinography, nystagmus, and the absence of central vision are observed.In addition, it is possible to reduce the reaction of the pupils of the eye to light. The disease is also manifested in strabismus and neurological disorders, photophobia, low visual acuity, various external changes. In adolescence, the patient may develop keratoconus.

Determine the presence of pathology can be viewed. In a sick child, he is unfocused and wandering.

Possible complications

Now in almost all cases patients with amaurosis patients completely lose the ability to see already in adolescence.The supportive means and assistance of relatives and close people can alleviate the problem.It is virtually impossible to avoid losing sight.In addition, atrophy of the optic nerve and various pathologies of the retina are possible.

Treatment

Leber does not have a comprehensive treatment that can get rid of Amaurosis.In childhood, before complete loss of vision, patients are prescribed glasses to improve their condition.Temporary amaurosis is treatable.In this case, methods are used to eliminate the cause of its appearance.

Medication

If the cause of the disease is a violation of blood circulation, patients are prescribed diuretics and hypotension.Migraine is removed by antispasmodics, endocrine diseases require, above all, hormonal correction.

With Amberstrom Leber ophthalmologists can prescribe drugs that relieve some of the symptoms. Correction of vision in this case is impossible.

Surgically

In the surgical method, only the patient whose disease was the result of the onset of a brain tumor may be needed.In other cases, the disease is not treated with the help of surgical operations.

Research in the field of genetic engineering in the future will be able to change the prognosis of the development of the disease.However, the methods are currently under development.

Despite the fact that congenital amaurosis is not treatable, a sick child needs to be prepared and adapted to life in the conditions of illness from early childhood.

Prevention

In order to avoid Leber's amaurosis, there are no methods of prevention.Future parents can undergo a medical examination and determine the state of the genes to avoid the possible birth of a sick child.

Diseases of the acquired type can be avoided if you carefully monitor your health and in time pass the diagnosis of the body.Recommended rest, proper nutrition and a healthy lifestyle.

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Video

conclusions

Unfortunately, today patients with Leber's amaurosis are almost doomed to complete loss of vision and methods of getting rid of the disease have not yet been invented. But this forecast does not apply to patients with amavroz of the acquired form. Prevention of pathology is almost impossible, but future parents can be diagnosed with the probability of its occurrence. Proper upbringing and adaptation will also help the sick child cope with the problems that give rise to this ailment.

Also read about such rare diseases as pterygium of eye and chorioretinitis.