Takamatsu

TakamatsuTAKAMATSU group of hereditary diseases in which damage to the nervous system combined with skin or chorioretinal by angiomatosis. Neurofibromatosis Recklinghausen. Typical multiple neurofibromas of the peripheral nerves; often localized neurofibromas intracranial (usually kohleovestibulyarnye nerve) and intraspinal, squeezing the brain. On the skin - age spots (red-cyanotic or color "coffee with milk"), in the skin and subcutaneous tissue - multiple tumors. In some cases there is a scoliosis, bone cysts. A significant portion of patients found increased blood levels of factor stimulating the growth of the elements of the nervous system. The prognosis of the disease depends on the availability of removal of tumors of the brain and spinal cord. Disease Hippel - Lindau (cerebroretinal angiomatosis) is a combination of angioplasty brain (usually the cerebellum) angiomatosis of the retina. This may be accompanied by cysts of the pancreas, cyst or tumor of the kidney, and polycythemia. With timely removal of angioplasty the forecast favorable. Tuberose sclerosis disease (Bourneville) - tumor-like proliferation of glia found in childhood. The characteristic triad: progressive dementia, seizures and pigmented adenoma sebaceous glands of the face. Frequent tumor of the heart, kidneys, lungs, the periventricular calcifications. The prognosis is poor. Disease Sterga-Weber (encephalotrigeminal angiomatosis). Typical angiomatous spots, especially in the frontal region, epileptiform convulsions, in most cases, mental retardation, hemiparesis, contralateral pigmented spot on the face, hemianopsia. Craniocervical-calcifications in the form of convolutions with two circuits, which are based on angiomatosis parieto-occipital brain regions. The prognosis depends on the fact that you can eliminate seizures. In this case, the psyche may not suffer and the children develop normally..



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