Introduction
The neurocutaneous syndrome summarizes various hereditary diseases that manifest themselves both on the skin and in the central nervous system.
Definition
The diseases that comprise the neurocutaneous syndrome are characterized by certain malformations of the cotyledons that develop during the embryonic period. This means that these malformations occur during the development of the unborn child. The human organism develops from 3 cotyledons – an outer, a middle and an inner one.
In the neurocutaneous syndrome there are malformations in the neuroectodermal and mesenchymal cotyledons. The neuroectodermal cotyledon is a part of the outer cotyledon from which the nervous system develops during embryonic development. The mesenchymal cotyledon develops into the “embryonic connective tissue“, from which the connective, cartilage, muscle and fat tissue, as well as bones, tendons and blood, emerge. The result of the neurocutaneous syndrome is a variety of diseases, which are classified differently. The classic neurocutaneous syndromes include neurofibromatosis, Sturge-Weber-Krabbe syndrome, Von-Hippel-Lindau-Czermak syndrome and Bourneville-Pringle syndrome.
Causes for the neurocutaneous syndrome
In the neurocutaneous syndrome there are changes in the genes that can cause various malformations in the embryonic period. The neuroectodermal malformations explain the neurological disorders. The mesenchymal malformations can lead to tumors. These can be either benign or malignant. The cause of this gene alteration has not yet been fully clarified.
Diagnosis of neurocutaneous syndrome
The diagnosis depends on the particular syndrome and cannot be generalized. As a rule, various imaging procedures are among the examination methods. The course of the disease can also vary and requires regular medical check-ups. Depending on the clinical picture, different specialist medical check-ups are required.
Associated symptoms
Since the neurocutaneous syndrome summarizes a large number of very different diseases, the symptoms and accompanying complaints are manifold. They can manifest themselves in different ways as skin disorders and disorders of neurological abilities. Depending on which areas of the brain are affected, a variety of failures can occur.
