Renander-Muller Disease

Renander-Muller disease

**Synonyms:** cataracts, glaucoma, corneal dystrophy, degeneration of the anterior lens capsule, posterior anterior subcapsular cataract, Barraquer-Lens syndrome. The essence of the disease Deposition of breakdown products (primarily lipids) and secondary products of inflammation in all tissues is one of the main signs of this disease. This is an irreversible process, its manifestations lead to their degeneration, and in severe cases – to destruction.

Pathogenesis The pathogenesis of the disease is a deficiency of enzymes: proteases, lipoproteases, nucleases. Because of this, a metabolic “block” is formed, disturbances at the level of cellular respiration. There is also damage to the integumentary tissues of the cell membrane. This is how the incorrect course of any reactions is formed - lipid and protein peroxidation. This is manifested by the accumulation of decay products and waste (lipids, proteins), dystrophy, respectively. With a deficiency of proteolytic enzymes and increased synthesis of connective tissues, irreversible changes are observed.

Bioenergy is not disturbed in the cortex, but at the border of the cortical and gelatinous zone it is significantly reduced. In addition, mitochondrial activity decreases and glycolysis is disrupted. Features and clinical manifestations Posterior subcapsular cataracts are distinguished by the following features:

1. It is two-sided. Cataracts progress quickly: in the first stage, the opacities occupy the entire cortical layer, and in later stages they spread to the posterior subcapsule. However, this happens over several months or years. In addition to lens opacities, almost all tissues of the eye are affected. Opacification of the peripheral part of the lens often affects the lens epithelium and peripheral processes of the crystal fibers. Dysfunctional changes in the fibrous capsule contribute to its destruction and saturation with blood plasma. These changes can lead to the death of the optic nerve, as well as optic purpura. While patients develop acute angle-closure glaucomatous type syndrome. In addition, in patients of the posterior type, the choroid of the eyeball is included in the pathological process, which leads to the formation of hyphema (hemosculum). 2. Appearance of the cataract. The following signs are typical for posterior subcapsular cataracts: The opacity of the lens covers up to 2/3 of its volume, while it penetrates almost its entire volume. Intense transient opacification is noted (there is a possibility of the presence of cavities in the lens body, which are formed by pseudo-erosion of the lenses). Violation of the transparency of the lens in most cases is diffuse. In this case, the thickness of the central zone of the lens is more than 50 mm, wrinkling appears, and the zone of the subcapsular space is clearly defined. Its boundaries have a clear outline and are located under the anterior fibrous flap. Thus, the anterior opacified membrane is characteristic of

Renander-Müller disease is a fairly common disease, mainly occurring in adolescents. In most cases, it affects boys aged 14-16 years. In girls, this disease is much less common.

However, recently the incidence of this disease has increased sharply. Doctors attribute this increase to the deterioration of the environmental situation and an increase in the number of stressful situations in both children and adults. Despite its name, this disease has nothing to do with infectious diseases.

Symptoms of the disease include facial deformation, headaches and decreased visual acuity.