Central nervous system (CNS) malformations constitute an important aspect of neurologic diseases; nearly 2,000 such entities are recognized at present. They occur in almost one percent of all live births (80–100/10,000 live births) and represent at least ten percent of all systemic malformations. Approximately 75 percent of fetal deaths, 40 percent of infant deaths and.05 percent of deaths in children under one year of age are caused by CNS disorders. However, clinically encountered CNS malformations are relatively mild; the more severe forms are incompatible with life and are discarded by nature as abortions. As a whole, these malformations occur in about two percent of all conceptions. Concurrent with the successful control of many diseases (e.g., infections) of infancy and childhood is an increase in the relative incidence of morbidity and mortality due to CNS malformations. Only about half of the CNS malformations are recognizable at birth or in the newborn; others become manifest as the child develops. It is well-recognized that many disorders remain silent for years to decades or until the morbid process converts the malformation into a disease. Incidence of major congenital disorders of the brain are shown in Table I.
KeywordsDown Syndrome Spina Bifida Fetal Alcohol Syndrome Central Nervous System Disorder Septum Pellucidum
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