Neurocutaneous Syndromes: Developmental Dysplasias of the Early Period of Gestation. A Clinical and MRI Study

The neurocutaneous syndromes, also called phakomatoses or hamartomatous syndromes, are a highly variable and heterogenic group of dermatologic, somatic and nervous system disorders. Much attention is paid to these syndromes by neurologists because they are usually accompanied by epilepsy and mental retardation. Dermatological symptomatology is relatively variable, and somatic disturbances are very common, but nervous system disorders, both in clinical and in MRI pictures, are regularly observed. The normal process of neural crest formation in the 4th week of gestation is characterized by a period of rapid proliferation and differentiation of the neuroependymal cells and this process of neuroblast migration is often considered to be the most important developmental period of the brain. This process continues between the 10th and 22nd weeks of gestation and ends with the cellular apoptosis of 30–50% of participating neuroblasts. In some cases, the neuroblasts will not reach their predetermined site within t...

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