Sclerosis Tuberous

Tuberous sclerosis is a fairly rare genetic disease, but quite often diagnosed. Most cases of the disease are associated with various chromosomal abnormalities that lead to disruption of cell development in the nervous system. The most common form of tubular sclerosis is Pringle-Bourneville disease.

Sclerosis, which occurs as a result of the accumulation of rough, fibrous, scar-like tissue in the meninges and mesenchymal structures of the spinal cord, connective tissue next to the bone of the skull (the so-called “celebrella sclerosis”), increases the mass of the skull (“hydrocephalus”, delayed brain growth), liquorodynamic disorders, in particular intracranial hypertension (dropsy, increased intracranial pressure). It is possible to distinguish two main forms of this process based on the place of origin: “pontine” and “pyramidal”. Both types have significant differences in the clinical picture and microscopically differ little from each other. In “pontine” sclerosis, scar tissue is located mainly in the pyramids of the temporal bones, the cerebellum, the corpus callosum, the medulla oblongata (sometimes extending beyond the brain into the spinal cord canal) and in the subcortical nuclei of the frontal lobes of the brain. Due to damage to the cerebellum, the process can lead to cerebellar ataxia on neurological examination, for example, the inability to maintain balance when closing the eyes. At

By its definition, it is not a disease, but a syndrome. That did not prevent it from being recognized as an independent nosological unit.

The disease or syndrome depends on its development and manifestation.

Bougainvillea sclerosis is a hereditary dystrophic disease caused by a disorder of lipid metabolism - dermatoglyperoxidase and a decrease in cell resistance to apoptosis. It is observed in patients of both sexes, but is most common in girls. The disease is most common in blondes and light-colored brunettes. The distribution over the territory is extremely uneven. Bougainvillea sclerosis MPS is most commonly reported in Japan, China, Italy, USA and Spain. The risk of incidence varies depending on the region. In Europe the prevalence is 4/10,000