Neurocytoma

Neurofibromatosis is the most common congenital form of neuroglial dysplasia affecting the central nervous system. Leads to the development of tumors of neuroepithelial origin or “neurocytes”. Such neoplasms manifest themselves in 4 main variants: astrocytoma (95%), glioblastoma (6%), schwannomas (5%) and their mixed variants (2%). Predisposing factors for the formation of tumors are previous childhood injuries to the skull and nose. A neurological defect in the form of seizures and pain manifests itself in 82% of cases as a type of “molecular rebound epilepsy”, as well as in connection with the growth of tumor masses. The majority of patients (73%) have eye damage (together with Kearns-Sayre syndrome - about 50%).

Sensorineural hearing loss is often observed (in 45% of cases), complicating neurosurgical treatment. The formation of a brain tumor in patients is the result of impaired control of neural tube development in the prenatal period due to mutation of the main allele of the NF-1 gene, which leads to an inferior phenotype. Chromosome X contains the NF-2 gene, both alleles of which take part in the full development of brain tissue and blood vessels. Carriage of gene mu