Malformations of the Nervous System [Volume 87] Handbook of Clinical Neurology Series

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.)
Reviews

The editors of this volume deserve special praise and appreciation for producing a book dedicated to malformations of the nervous system that is elaborate and comprehensive.
The role of exponential growth in the field of molecular genetics is reflected throughout, and the authors should be congratulated for integrating the new genetic information with the available scientific data. The book is highly recommended for neonatologists, paediatric neurologists, epileptologists and neuroradiologists who care for these unfortunate children.
Journal of Neurology, Neurosurgery and Psychiatry - June 2008

Table of Contents

SECTION I. Specific malformations using the new integrative classification

Revised classification

Axes and gradients of the neural tube and gradients for a morphological and molecular genetic classification of nervous system malformations.

Midline hypoplasias

Holoprosencephaly. Septo-optic-pituitary dysplasia. Rhombencephalosynapsis. Embryology and malformations of the forebrain commissures.

Disorders of segmentation of the neural tube

Disorders of segmentation of the neural tube: Chiari malformations. Segmentation of the neural tube: agenesis of selective neuromeres. Cerebellar hypoplasias.

Hamartomatous disorders of cellular lineage

Tuberous sclerosis. Hemimegalencephaly syndrome. Disorders of radial neuroblast migration and cerebral cortical architecture. Periventricular nodular heterotopia. Gio12. Subcortical laminar (band) heterotopia. Lissencephaly type I
Lissencephaly type II. Schizencephaly.

Disorders of neural crest induction of non-neural tissues

Neural tube programming and the pathogenesis of craniofacial clefts: the neuromeric. organization of the head and neck: Part I. Neural tube programming and the pathogenesis of craniofacial clefts: the neuromeric
organization of the head and neck: Part II. The oral-facial-digital syndromes.

Other dysgeneses

Congential vascular malformations in childhood. Acquired, induced and secondary malformations of the developing central nervous system.

SECTION II. Comparative manifestations of central nervous system malformations

Epilepsy in patients with cerebral malformations. Neuromuscular disorders associated with cerebral malformations. Neuroendocrine complications of central nervous system malformations. Cerebral dysgeneses associated with chromosomal disorders. Cerebral dysgeneses secondary to metabolic diseases in fetal life.

SECTION III. Diagnostic methods

Imaging malformations of cortical development. Clinical neurophysiology of cortical malformations: magnetoencephalography and electroencephalography. Molecular genetic testing and genetic counselling. Embryology and neuropathological examination of central nervous system malformations.

SECTION IV. Management of central nervous system malformations

Medical treatment in children with central nervous system malformations. Surgical treatment of central nervous system malformations. Neurorehabilitation of children with cerebral palsy. Educational, cognitive, behavioral and language development issues.