Malformations of the Nervous System

This is a further volume in the Handbook of Clinical Neurology (Series Editors:: Michael J. Aminoff, Department of Neurology, University of California, San Francisco, USA; François Boller, Bethesda, USA; Dick F. Swaab, Netherlands Institute for Neuroscience, Amsterdam, the Netherlands).

The period of intrauterine and neonatal brain development is crucial for everything we will become and can accomplish in the rest of our life. In that short period of time the outline of the human brain develops into a tremendously complex organ consisting of 100 billion neurons, each making between 1000 and 100 000 contacts with other particular groups of neurons by means of some 100 000 km of nerve fibers. Each group of neurons should be born at the right moment, migrate to the site where they differentiate and make their specific contacts in a limited critical period of brain development in order to function later in a normal way. Building such a complex structure as the brain in such a brief period is certainly the most demanding task for nature. It is in fact a wonder that it does not often end with catastrophic failures in one of the numerous exactly timed and extremely complex processes; instead it mostly results in a healthy baby with good potential for the rest of its existence. This volume deals with those children in whom brain development has resulted in a malformation of the central nervous system. This field has recently gained exciting new insights, for instance from molecular genetics, which are integrated in this volume. Section I of this volume follows the new integrative classification and deals with midline hypoplasias, disorders of segmentation of the neural tube, hamartomatous disorders of cellular lineage, disorders of radial neuroblast migration and cerebral cortical architecture and other dysgeneses. Section II describes the different clinical manifestations of CNS malformation, followed by sections on diagnostic methods, management and treatment. (Taken from the foreword by the series editors.)